Blood Disorders PDF

19 Nursing care of conditions related to haematological disorders Mairead Ni Chonghaile1 and Laura O’Regan2 1 St James’ Hospital, Dublin, Ireland St George’s University of London and Kingston University, London, UK 2 Contents Introduction Overview of blood Blood transfusion Investigations Anaemi...

19 Nursing care of conditions related to haematological disorders Mairead Ni Chonghaile1 and Laura O’Regan2 1 St James’ Hospital, Dublin, Ireland St George’s University of London and Kingston University, London, UK 2 Contents Introduction Overview of blood Blood transfusion Investigations Anaemia Polycythaemia Haemophilia Thrombocytopenia 387 387 395 397 402 408 409 409 Disseminated intravascular coagulation Multiple myeloma Leukaemia Lymphoma Haematopoietic stem cell transplant Conclusion References Learning outcomes Having read this chapter, you will be able to: • • • Outline the normal and altered pathophysiology of the haematology system Describe the medical and nursing management of patients with haematological disorders Describe nursing care principles governing safe practices in the transfusion of blood and blood products Fundamentals of Medical-Surgical Nursing: A Systems Approach, First Edition. Edited by Anne-Marie Brady, Catherine McCabe, and Margaret McCann. © 2014 John Wiley & Sons, Ltd. Published 2014 by John Wiley & Sons, Ltd. 410 410 413 415 417 420 421 Nursing care of conditions related to haematological disorders Chapter 19 Introduction Nursing knowledge and skills related to the haematological system are also applicable to all areas of medical and surgical nursing. A sound knowledge and understanding of haematology, and how to interpret it, underpins assessment and care planning interventions. This chapter will address normal and abnormal pathophysiology, including the nursing care and treatment of both malignant and nonmalignant haematological conditions. Overview of blood The haematological system comprises blood, bone marrow and lymph nodes. Blood is considered to be an organ, although it exists in fluid connective tissue and develops from the mesenchyme, a connective tissue in which the blood cells and smaller components are suspended in and surrounded by plasma fluid. The pH of the blood is between 7.35 and 7.45. The total blood volume is estimated at 4–5 L in females and 5–6 L in males, accounting for approximately 8% of the body weight. The composition and function of blood are outlined in Boxes 19.1 and 19.2. Cellular components There are three main types of blood cell; erythrocytes (red blood cells [RBCs]), leucocytes (white blood cells [WBCs]) and thrombocytes (platelets). The blood cells originate from a pluripotent stem cell type Box 19.1 Components of the blood (adapted from Nair & Peate 2009) Body weight and volume Whole blood (8%): • Plasma (55%) • Blood cells (45%) Other fluids and tissues (92%) Box 19.2 • • • • • • • Formed cells Plasma: • Proteins (7%) • Water (91.5%) • Other solutes (1.5%) Blood cells: • Platelets (150,000–400,000/μL) • White blood cells (>5000–10,000/μL): • Neutrophils (60–70%) • Lymphocytes (20–25%) • Monocytes (3–8%) • Eosinophils (2–4%) • Basophils • Red blood cells (4.8–5.4 million/μL) Main functions of blood Acts as a transport system, carrying oxygen, blood cells, hormones, enzymes, chemical and minerals Obtains nutrients from the alimentary canal, distributing them throughout the body Removes waste products, e.g. carbon dioxide from the lungs and excretory waste from the kidneys Regulates body heat by distributing the temperature Haemostasis (control of bleeding) Regulates water and electrolyte balance Circulates antibodies, providing a protective immune function 387 Part 2 Adult Medical and Surgical Nursing Key: Key: Progenitor cells CFU-E Precursor cells or ‘blasts’ Colony-forming unit – erythrocyte CFU-Meg Colony-forming unit – megakaryocyte Formed elements of circulating blood CFU-GM Pluripotent stem cell Colony-forming unit – granulocyte macrophage Tissue cells Myeloid stem cell Lymphoid stem cell CFU–E CFU–Meg Proerythroblast Megakaryoblast CFU–GM Eosinophilic myeloblast Basophilic myeloblast Myeloblast Monoblast T lymphoblast B lymphoblast Eosinophil Basophil Neutrophil Monocyte T lymphocyte (T cell) B lymphocyte (B cell) Nucleus ejected Reticulocyte Megakaryocyte Red blood cell (erythrocyte) Platelets (thrombocytes) 388 Granular leucocytes Agranular leucocytes Macrophage Plasma cell Figure 19.1 The formation of blood cells. Reproduced from Nair, M. & Peate, I. (2009) Fundamentals of Applied Pathophysiology, with kind permission from Wiley Blackwell. synthesised in the bone marrow and found in the iliac crest of the pelvis, the long bones of the humerus and the vertebrae, skull, ribs and sternum. This pluripotent stem cell differentiates into specific blood cell lines. Haemopoiesis (or haematopoiesis) is the term that refers to the formation of specific cell types (Figure 19.1). Many disorders of the haematological system arise from a deviation from normal haemopoiesis, resulting in malformation of blood cells along the developmental line. Erythrocytes Normal RBCs are biconcave discs, approximately 8 μm in diameter, whose shape and pliability gives them the flexibility to pass through capillary walls, facilitating gaseous exchange and the carriage of essential oxygen around the body. Mature RBCs contain no nucleus. Reference values for RBCs are outlined in Table 19.1. Nursing care of conditions related to haematological disorders Table 19.1 Chapter 19 Erythrocyte reference values (adapted from Waugh & Grant 2010) Measure Normal values Erythrocyte count • Number of erythrocytes per litre, or per cubic millilitre (mm3), of blood Males: 4.5–6.5 × 1012/L (4.5–6.5 million/mm3) Females: 3.8–5.8 × 1012/L (3.8–5.8 million/mm3) Packed cell volume (PCV, haematocrit) • The proportion of red cells per litre, or per mm3, of blood 0.40–0.55 Mean cell volume (MCV) an average cell, measured in femtolitres • The volume of (1 fL = 1 × 10−15 L) 80–96 fL Haemoglobin • The weight of haemoglobin in whole blood, measured in g/100 mL blood Males: 13–18 g/100 mL Females: 11.5–16.5 g/100 mL Mean corpuscular haemoglobin (MCH) • The average amount−12of haemoglobin per cell, measured in picograms (1 pg = 10 g) 27–32 pg/cell Mean corpuscular haemoglobin concentration (MCHC) • The weight of haemoglobin in 100 mL of red cells 30–35 g/100 mL of RBCs Haemoglobin Haemoglobin consists of a protein called globin and haem, a pigment containing iron. The haemoglobin molecule, composed of four polypeptide chains, consists of two alpha and two beta chains, and has four atoms of iron, each carrying one molecule of oxygen. Each RBC contains about 280 million haemoglobin molecules. Haemoglobin accounts for 33% of the weight of the RBC by virtue of its iron content and is responsible for giving blood its characteristic red colour, oxygenated blood being a brighter red colour than deoxygenated blood. When haemoglobin binds with oxygen, it produces oxyhaemoglobin, which makes up the oxygen-carrying capacity of blood. RBCs do not contain any mitochondria and use anaerobic respiration to produce energy, thus conserving the oxygen they are carrying. In addition to iron, vitamin B12, folic acid, pyridoxine (vitamin B6) and other factors are required for the healthy synthesis of erythrocytes. Where there is a deficiency during erythropoiesis, a decreased number of erythrocytes are produced and anaemia occurs. Erythropoiesis Hypoxia increases erythropoiesis by stimulating the production of erythropoietin in the kidneys, which increases the production of proerythroblasts to enter the blood and the rate of reticulocyte maturation. As a result, the number of erythrocytes and the amount of oxygen uptake increase, reducing the hypoxic state. The average life span of erythrocytes is 120 days, after which they are destroyed in a process known as haemolysis and are removed by the reticuloendothelial system, particularly in the liver and the spleen. The reticuloendothelial cells produce bilirubin from haemoglobin that is released from the RBCs, and this is excreted in the bile. Iron released from haemoglobin during bilirubin formation is carried in the plasma to the bone marrow, bound to a protein called transferrin and used for the production of new haemoglobin. 389 Part 2 Adult Medical and Surgical Nursing Leucocytes Leucocytes (WBCs) are divided into two groups: granulocytes (60%) and agranulocytes (mononuclear cells; 40%). They are easily differentiated from RBCs as they contain a nucleus, are larger and have the ability to move out of blood vessels, a process called diapedesis. Granulocytes Granulocytes are defined by the presence of granules in the cytoplasm. Their diameter is 2–3 times that of RBCs, and their nuclear matter is arranged in lobes, between two and four in number, giving rise to the name polymorphonuclear leucocytes. The number of circulating granulocytes remains constant except in the presence of infection, during which numbers increase (leucocytosis). A decrease from normal values is called leucopenia. Granulocytes are further divided into three groups: • • • 390 basophils eosinophils neutrophils. Basophils account for approximately 1% of granulocytes, contain lobed nuclei and have a specific immune function as they bring about a reaction to harmful substances. Their cytoplasmic granules contain heparin, histamine and other substances, which cause inflammation. Allergens – antigens that cause an allergy – stimulate the basophils to release the contents of their granules. Eosinophils account for 2% of granulocytes, contain a B-shaped nuclei and have a specific phagocytic mode of action. They contain enzymes and peroxidase in their granules, which function against parasites. Eosinophil numbers increase during allergic reactions. Neutrophils account for 60–70% of total circulating WBCs, and their primary function is phagocytosis. Their nuclei are multilobed and their cytoplasm contains lysosomes. They are capable of moving out of the blood vessel in response to foreign material and are present at the site of inflammation within an hour. The number of neutrophils increases in the presence of: • • • • • • • infection leukaemia inflammation metabolic disorders injury to the body pregnancy myocardial infarction. Agranulocytes (mononuclear leucocytes) There are two types of agranulocyte: lymphocytes, accounting for 25%, and monocytes, accounting for 5% of the total number of leucocytes. Lymphocytes are suspended in lymphatic fluid, which is found in the lymphatic tissues and spleen. Their lifespan is between a few hours and years, and they differ from other WBCs in their mode of action as they are not phagocytic. There are two types of lymphocyte: T and B lymphocytes. T cells are formed in the thymus gland, while B cells originate in the bone marrow (see Figure 19.1). T lymphocytes are involved in graft reactions and in fighting cancer and viruses by direct cell-kill, while B lymphocytes make antibodies in response to antigens in the body. Once this occurs, and assisted by T lymphocytes, B lymphocytes enlarge and divide, producing two types of cell: • • plasma cells memory B cells. Plasma cells release antibodies, or immunoglobulins. Once antibodies have bound to antigens, they are prime targets for helper T lymphocytes, cytotoxic T lymphocytes and macrophages. Antibodies bind to the toxin and activate the complement system, which in turn accelerates the phagocytic response. This process of the combination of B and T lymphocyte production leads to the release of cytokines and is more specifically referred to below in relation to macrophage activity. The cytokines produced Nursing care of conditions related to haematological disorders Chapter 19 are chemical messengers released by specific cells of the immune system that are used by immune cells to communicate with one another. Examples of cytokines include interleukins and interferons. The memory B lymphocytes hold within them the memory of the interaction that has occurred for a variably long period of time afterwards, providing immunity against the same antigens (see Chapter 18). Monocytes are the largest of the leucocytes and have two modes of action; one is phagocytosis, where as the other mode of action involves their entering the tissues and developing into macrophages. Interleukin-1, which is produced by both types of cell, has a specific function in the inflammatory response and in producing immunity. Interleukin-1 acts on the hypothalamus, increasing the body temperature, stimulates the development of some globulins in the liver and enhances the activation of T lymphocytes. The monocyte and macrophage system is also referred to as the reticuloendothelial system. A number of macrophages are fixed in particular body locations – as synovial cells, Kupffer cells (in the liver), Langerhans cells (in the skin), alveolar macrophages (in the lungs), sinus (reticular) cells (in the spleen), cells in the lymph nodes and thymus gland, mesangial cells (in the kidney) and osteoblasts (in bone). Macrophages release cytokines, for example interleukin-1, and link the specific with the nonspecific immune reaction. Thrombocytes (platelets) Platelets lack a nucleus, are disc-shaped particles measuring 2–4 μm in diameter, arise from megakaryocytes in the bone marrow and are regulated by thrombopoietin. Platelets usually circulate for approximately 1 week before being destroyed and have a key role in haemostasis (the control of bleeding). Blood clotting There are three phases of haemostasis: • • • Phase 1 is vasoconstriction of the injured blood vessel. Phase 2 sees the formation of a platelet plug. Phase 3 involves the formation of a fibrinogen clot. The final stage is called fibrinolysis and involves the breakdown of the clot. Vasoconstriction The first phase of clotting begins in response to an injury to the blood vessel, causing the vessel to go into spasm and constrict. Thrombocytes produce serotonin, causing the muscles of the vessel wall to constrict and thus decrease the blood flow. Constriction is also brought about by other factors, including the stimulation of pain receptors and the release of thromboxanes by the damaged cell wall. Platelet plug formation Platelets adhere to one another, releasing thromboxanes and ADP (adenosine diphosphate), which further promote platelet aggregation. The result is the formation of a primary platelet plug, from which the intrinsic coagulation pathway arises due to breakdown of parts of the platelets and the release of substances into the bloodstream. The extrinsic pathway arises from the release of tissue factors a result of vessel injury. There is an overlap between the two pathways, involving the interaction of a complex series of clotting factors (Figure 19.2). Fibrinogen clot formation There are two pathways by which coagulation occurs – the extrinsic and the intrinsic pathway – in collaboration with the various clotting factors listed in Box 19.3. The extrinsic pathway is activated when the vessel is damaged, causing the release of certain clotting factors to work in collaboration with 391 Part 2 Adult Medical and Surgical Nursing Extrinsic pathway Intrinsic pathway Platelets, thromboplastin precursors, factors VII, IX, XI, XII Tissue damage, factors III and VII Calcium ions Thromboplastin 392 Prothrombin, factor II Thrombin Fibrinogen, factor I Fibrin Red blood cells Platelets Clot formation White blood cells Factor XIII Fibrinolysin Calcium ions Figure 19.2 The clotting cascade. calcium to release thromboplastin. As a result of a series of reactions, prothrombin is converted to thrombin, which in turn converts fibrinogen to fibrin. The intrinsic pathway is activated when the collagen lining of the blood vessel is exposed, producing thromboplastin. The fibrin forms a mesh across the damaged vessel, trapping blood cells and allowing the cut to heal. Clots that form in the body are broken down by the fibrinolytic system, which consists of plasmin and other proteolytic enzymes. As the tissue is repaired, the clots dissolve. Nursing care of conditions related to haematological disorders Chapter 19 Box 19.3 Clotting factors. Reproduced from Nair, M. & Peate, I. (2009) Fundamentals of Applied Pathophysiology, with kind permission from Wiley Blackwell I II III IV V VII VIII IX X XI XII XIII Fibrinogen Prothrombin Thromboplastin Calcium Proaccelerin, labile factor Serum prothrombin conversion accelerator Antihaemophilic factor Christmas factor, plasma thrombin component Stuart–Power factor Plasma thromboplastin antecedent Hageman factor Fibrin-stabilising factor Blood plasma Plasma is the straw-coloured, fluid part of blood in which all the cellular components are suspended (Box 19.4); it is made up of 92% water and accounts for 55% of the blood volume. Blood groups Antigens are located on the surface of RBCs, while antibodies are found in the circulating plasma. Antibodies are antigen-specific, combining to a certain antigen and causing agglutination, i.e. clumping together, of the cells. The combination of antigens and antibodies can also cause haemolysis, rupturing the blood cells. Blood grouping is derived from the antigens on the surface of RBCs, which make up the ABO and rhesus systems (Table 19.2). The use of the term ‘universal donor’ for individuals with group O blood can be misleading as there are two mechanisms by which group O blood can cause a reaction: • • There may be mismatching of groups other than ABO, emphasising that all blood groups should be correctly matched. Donors’ antibodies can react with recipients’ antigens. Type O blood contains anti-A and anti-B antibodies. Therefore, if, for example, type O is transfused to a type A patient, the A antibodies will react against the A antigens on the surface of the type O blood. This is, however, not usually cause for concern due to the dilution of the donor’s antibodies in the recipient’s blood. The distribution of blood groups often correlates with different ethnicities, as suggested in Table 19.2. Rhesus factor The rhesus factor is an important antigen found on the surface of some RBCs. In the UK, it is estimated that 85% of the population are rhesus-positive (as they have factor D) and the remaining 15% are rhesus-negative (as they do not have factor D). If rhesus-positive blood is given to a rhesus-negative person, agglutination will occur, and there are important implications here for cross-matching. Antibodies against the rhesus antigen occur when there is exposure through a transfusion of blood or by cross-placental transfer of blood that is positive to a rhesus-negative mother. 393 Part 2 Adult Medical and Surgical Nursing Box 19.4 Components of plasma Albumin • This accounts for 60% of plasma proteins • It helps to regulate the osmotic pressure of the blood • Low levels (hypoalbuminaemia) cause movement of fluid into the cellular spaces, resulting in oedema • Albumin has carrying capacity for fatty acids, drugs and hormones • Along with fibrinogen, it is held to be responsible for plasma viscosity Fibrinogen • Fibrinogen accounts for 4% of plasma proteins Globulins • Globulins account for 36% of plasma proteins • They can be divided into three types: • Alpha • Beta • Gamma • Alpha/beta globulins: • Are produced in the liver • Are transport globulins and carry substances such as thyroxin on thyroid-binding globulin, iron on transferrin, and fat-soluble vitamins • Gamma globulins are produced by B lymphocytes and are known as antibodies or immunoglobulins 394 Electrolytes • These are inorganic molecules that separate into ions when dissolved in water • They have an important function in regulating osmosis, pH balance, muscle contraction and nerve impulses • The main electrolytes are sodium, potassium, calcium, magnesium, chloride and bicarbonate Cellular waste • Plasma forms a transport medium for urea, creatinine and uric acid Nutrients • Plasma transports amino acids, glucose, glycerol and fatty acids to the alimentary tract Hormones • Hormones are transported in plasma to their target organs, where they exert their function Gases • The main gases transported are oxygen and carbon dioxide • Oxygen combines with haemoglobin to form oxyhaemoglobin • Carbon dioxide is present as bicarbonate ions in the plasma Rhesus incompatibility can pose a major problem in pregnancy when fetal blood crosses the placenta; the mother becomes sensitised to the rhesus antigen and produces antibodies, which cross the placenta causing agglutination or a condition called haemolytic disease of the newborn, which can be fatal. There is usually no problem in the first pregnancy as the cross-placental passage of antigens is minimal and occurs in late pregnancy or at birth due to a tear in the placenta; there is little time for the mother to produce antibodies against the antigens. In subsequent pregnancies where the fetus is rhesus-positive, the reaction to a leak of blood across the placenta is more pronounced as the mother is already sensitised to the antigen. In severe cases of haemolytic disease of the newborn, a transfusion through the umbilical cord may be necessary and premature birth may be induced. Nursing care of conditions related to haematological disorders Chapter 19 Mothers are monitored for antibody levels and given an injection of anti-D antibody after the first birth, miscarriage or abortion, or prior to birth. Its purpose is to inactivate the fetal antigens and prevent sensitisation of the mother and production of antibodies, or further antibody production by the mother. Blood transfusion Donated blood is routinely screened for HIV, hepatitis B and C and syphilis. Some countries do not allow blood donation from prospective donors who have lived in the UK between 1980 and 1996, in order to remove the risk of transmission of Creutzfeldt–Jakob disease (CJD). Blood is currently leucodepleted as this reduces the risk of transmission of CJD and other infectious reactions. Box 19.5 provides details on the nursing management of a blood transfusion, while Table 19.3 outlines different blood products. Table 19.2 Blood grouping (adapted from Nair & Peate 2009) Blood Group Frequency in the UK (%) Antigens Antibodies Can donate to Can receive from A 42 Antigen A Anti-B A, AB A, O B 8 Antigen B Anti-A B, AB B, O AB 3 Antigen A Antigen B None AB A, B, AB, O O 47 None Anti-A Anti-B A, B, AB, O Universal donor, if rhesus negative O Box 19.5 Nursing management of a blood transfusion – core principles Ascertaining the rationale for blood transfusion • The rationale needs to outweigh the risks • The reasons for a transfusion are: • Life-saving situations (massive blood loss due to trauma) • Replacing blood loss due to surgery or a procedure • Treatment of conditions that result from bone marrow failure, e.g. anaemia, haemophilia or sickle cell disease • Check the hospital thresholds for ordering blood (which are set out to minimise blood waste and give optimal treatment to a specific patient group); e.g. the haemoglobin level needs to be below 8 g/L in some haematology day care units • The clinical abnormality determines the type of transfusion • In massive blood loss, defined as >1.5 L, an emergency blood alert is sounded immediately by contacting the blood bank • Each emergency is classified and coded, according to the clinical indication; each has appropriate specific guidelines and blood issuing requirements (Continued) 395 Part 2 Adult Medical and Surgical Nursing Blood sampling • Safety measures are paramount to good-quality care • This is the responsibility of registered nurses and phlebotomists • Ensure the correct labelling of blood samples: • Complete all information fields, and check off the patient’s name band • The sample bottle must be signed by the professional • The sample blood bottle label is completed at the patient’s bedside by the staff member who takes the blood • Serious ABO mismatch errors occur at the time of sampling or when the blood is given to the patient due to incorrect identification (Provan et al. 2009) Cross-matching/requesting • Check local policy guidelines to ensure that the appropriate professional with the correct training and competency completes the cross-match form and requests the blood • The patient’s details, type of blood, amount and time to transfuse (urgency) as well as any specific requirements must be clearly written on the request form • Special requirements include whether the blood should be negative for cytomegalovirus or have been irradiated prior to issue; this is particularly important for stem cell transplant patients Prescription 396 • • • • In the UK and Ireland, blood can be prescribed by suitably trained nurse practitioners or doctors It is prescribed on the drug chart under the ‘IV medicines’ section or on a transfusion prescription record The unit of blood or any blood product is checked by two registered nurses or one registered nurse and a doctor Yearly competency training in the handling and administering of blood products is a professional requirement Consent/checking procedure • Patients must provide consent prior to administration; this must be documented by the prescriber. A separate consent form is not necessary • Educate patients on: • The rationale for transfusion • The potential side effects • Any possible alternatives • Provide an information leaflet to patients who regularly receive blood transfusions • For patients who lack the capacity or are unconscious, the ‘best interests’ principle is followed in the absence of an advance directive • Patients need to be cross matched and consented prior to major surgery • Ascertain whether patients have any religious beliefs that might prelude them from consenting to a blood transfusion, e.g. they are Jehovah Witnesses: • Clearly document what, if any, blood product is acceptable for that patient • It is recommended that suitable emergency contingency plans be documented in care plans prior to major elective surgery or prior to giving birth. Such plans must be agreed well in advance (at least 3 weeks) by the physician or surgeon with the inclusion of the patients, and if necessary religious ministers and legal representatives Nursing care of conditions related to haematological disorders Chapter 19 Record-keeping • Manage the traceability of blood by means of a paper trail • Blood is not dispatched from the blood blank until it has been checked and registered by trained professionals • Accompanying paperwork must match the identification and requirements of patients and their prescription • Regular traceability audits and blood transfusion error audits are required • Observations must be monitored and reactions reported Administration • The care and management of blood transfusions needs to comply with local policy • Prior to administration: • At the bedside, two registered nurses must check the patient’s identification against the name band • Check the prescription against the paperwork accompanying the blood • Check for damage to the packaging and for suspended components or clotting • Monitor vital signs prior to commencement, every 15 minutes during the first hour and then hourly • Observe for: • Acute and delayed haemolytic reactions • Anaphylaxis • Acute lung injury • Post-transfusion purpura • Pyrexia • Transfusion-associated graft versus host disease • Transfusion-transmitted infection • Fluid overload • Report signs of abnormality to the medical team; the blood will be stopped and the appropriate treatment administered • Report adverse events or ‘near misses’ to the: • Transfusion manager • Risk management team, via the internal incident reporting system • In the UK and Ireland, problems associated with blood transfusion are reported to: • Serious Hazards of Transfusion (SHOT) schemes • The risk management incident reporting system • Further investigation and reporting to SHOT is the responsibility of the blood bank manager and the hospital transfusion committee • SHOT receives yearly reports of numerous avoidable errors (the most common being administration of the wrong blood to patients) Transfusion reactions A number of blood reactions can occur when transfusing blood products (Box 19.6). General signs and symptoms include pyrexia, tachycardia, rigors, urticaria, bone and muscle pain, dyspnoea and gastrointestinal upset. Investigations Since the collection of specimens requires an invasive procedure, consent must be sought from patients, who are informed about: 397 Part 2 Adult Medical and Surgical Nursing Table 19.3 Blood products (adapted from Dougherty & Lamb 2009) Transfusion type ABO/rhesus cross-matching Administration Shelf-life storage RBC transfusions Yes ABO compatible A blood-giving set with an integral filter Change 12-hourly Change if giving another fluid type Shelf life: 35 days at 2–6°C Once out of the fridge, use within 30 minutes Maximum time to complete infusion is 5 hours Platelets Rhesus-negative patients under the age of 40 should be given rhesus D-negative platelets Preferably ABO compatible Platelet-giving set Change for each unit Shelf-life: 4–5 days at 20–24°C once on the platelet agitator Start 30 minutes after the platelets have left the agitator Give within 30 minutes Fresh frozen plasma Yes ABO compatible Blood-giving set with an integral filter Should be changed every 12 hours Change if giving another fluid type Storage: up to a year at –30°C Administer as soon as thawed Complete infusion within 4 hours Anaphylaxis is a risk with rapid infusion Cryoprecipitate Yes ABO compatible Blood-giving set with an integral filter Should be changed every 12 hours Change if giving another fluid type Storage: up to a year at –30°C Administer as soon as thawed Complete infusion within 4 hours Anaphylaxis is a risk with rapid infusion 398 • • • the purpose of the test; the time frame in which they will know the result; how the result will be communicated. Care of the venepuncture site and specimen collection should adhere to good clinical practice guidance. Blood tests Blood tests (Box 19.7) are routinely collected to: • • • confirm disease; monitor disease; regulate therapy or treatment. Nursing care of conditions related to haematological disorders Box 19.6 • • • • • • • • • Chapter 19 Management of transfusion reactions Stop the infusion and contact the medical team Check the vital signs Check the patient’s identity against the paperwork Mild fever: • Paracetamol • Infuse at a slower rate Mild allergic reactions: • Stop the infusion • Give chlorphenamine prior to a slower recommencement of the transfusion Suspected ABO incompatibility: • Send the unit to the blood bank • Set up a saline infusion and initiate early warning scoring • Monitor and support urinary output Severe allergic reactions (anaphylaxis): • Chlorphenamine • Bronchodilators • Intravenous fluids • If necessary, adrenaline, hydrocortisone and dopamine • Send the unit to the blood bank Suspected bacterial infection: • Blood cultures • Intravenous antibiotics and oxygen therapy • The unit of blood must be investigated Fluid overload: • Furosemide and oxygen therapy • Monitor the patient closely Bone marrow aspirate/biopsy Bone marrow aspiration and trephine biopsy are used to diagnose haematological conditions and monitor the success of treatment. A sample is taken by inserting an aspiration needle into the bone marrow. The routine site is the iliac crest, although rarely the sternum can be used, but only for aspiration. The sample is then sent for specific cytogenesis (chromosomal testing), immunophenotyping and molecular analysis. The procedure is performed by suitably trained healthcare professionals. Nurses are routinely involved with patient care before, during and after the procedure (Box 19.8). Patients who require a bone marrow aspirate or biopsy experience high anxiety. The procedure is painful, and the discomfort can last for 3 days. 399 Part 2 Box 19.7 Adult Medical and Surgical Nursing Blood tests Blood film • This requires a small sample of blood in EDTA (ethylenediaminetetraacetic acid) • It can discern various haematological conditions, such as leukaemia Full blood count (FBC) • The FBC gives information about RBC, haemoglobin, WBC and platelet levels, including the differential breakdown or percentage of each nucleated cell per cubic millilitre of blood • It fulfils a role in detecting and monitoring a disease • It is essential for the surveillance of ongoing treatment • The FBC also gives the value of the reticulocyte count, the haematocrit or PCV (packed cell volume), and the mean cell volume (MCV) Haemoglobin • Haemoglobin is measured during the FBC and expressed in grams per litre Erythrocyte sedimentation rate • The erythrocyte sedimentation rate (ESR) measures the rate at which the RBCs fall in columns in a capillary tube • A raised ESR is indicative of an infection, inflammatory disorder and/or malignancy 400 Neutrophil count • Neutrophils are a type of WBC • They are documented on a differential blood count9 • Neutropenia is characterised as a count <2.0 × 10 /L • The risk of developing infective complications greatly increases as the count reduces • Their parameters are as follows: • A count of 1.0–1.5 × 1099/L carries no significant risk of infection • A count of 0.5–1.0 × 10 /L carries a moderate risk of infection, and the patient can usually be treated as an outpatient • A count <0.5 × 109/L carries a major risk of infection and necessitates admission and intravenous antibiotic treatment Coagulation screen • The screen measures various bleeding times by measuring the activity of certain components of the clotting mechanism: • Prothrombin time (PT) – assesses the extrinsic clotting pathway. It is prolonged in patients who are on warfarin therapy, and in patients with liver disorders and disseminated intravascular coagulation (DIC) • Activated partial thromboplastin time (APTT) – assesses the intrinsic clotting pathway. It is prolonged in patients receiving heparin and those who have liver disease and DIC • International normalised ratio (INR) – measures the PT of patients compared with an international average range Sickling test • This is the diagnostic test for sickle cell anaemia Coomb’s test • A positive Coomb’s test or direct antiglobulin test (DAT) indicates the presence of RBC antibodies • This is the diagnostic tool to detect red cell haemolysis Reticulocyte count • This is the percentage of young (1–2-day-old), non-nucleated erythrocytes in the peripheral blood • Raised or lowered reticulocyte counts are indicative of altered physiological states Nursing care of conditions related to haematological disorders Chapter 19 Haemoglobin electrophoresis This is performed to detect abnormal haemoglobin production It is used to diagnose haemoglobinopathies or thalassaemias • • Human leucocyte antigen (HLA) typing • HLA typing is a histocompatibility test to discern gene complexes encoding the cell surface membranes of the immune system • It ascertains an HLA match for patients being considered for stem cell or bone marrow transplants from a donor • The degree of likeness of the complexes between the recipient and perspective donor will determine the likelihood of the selection and potential side effects after transplantation Box 19.8 Nursing management of patients undergoing bone marrow aspiration • • • • • • • • • • • • • Ensure that: • Consent is obtained and information provided on: — the rationale for the test — what the test entails — potential side effects • Patients are: — offered short-acting sedation (lorazepam or midazolam) — given local anaesthetic — offered oral analgesia during and following the procedure Position patients in the left or right lateral position Take observations before and immediately after the procedure and at 15-minute intervals thereafter Observe patients throughout, and give further sedation as required Observe oxygen saturation levels after the procedure, as the sedative effect can cause hypoxia Assist practitioners (specialist nurse or doctor) with collecting and labelling the samples Apply a pressure dressing to the site Observe the site for haemorrhage, haematoma and infection Observe patients for 1–4 hours after the procedure, depending on the amount of drug used and in line with local policy Document in the notes the procedure, the samples taken and the patient’s condition Educate patients on observing the wound site for infection and haemorrhage Give the patient an information leaflet, which will also indicate whom to contact should any complication occur Inform patients when the results will be available and who will provide this information 401 Part 2 Adult Medical and Surgical Nursing Anaemia Anaemia is derived from the Greek word meaning ‘without blood’ and refers to a reduction in the number of RBCs and/or the haemoglobin level. Anaemia is not a disease but a laboratory term used to describe this altered physiological state, and is defined as occurring when the haemoglobin level is less than 13.5 g/dL in males or 11.5 g/dL in females. Various terms are used to classify anaemia. Groupings can be associated with causative factors or the appearance of the RBCs. The causative factors include: • • • • • a decrease in RBC production due to: • external deficiencies such as iron deficiency; • bone marrow failure; excessive haemorrhage; an increased demand for RBCs in pregnancy; haemolysis; malaria. When anaemia is classified according to the appearance of the cells, the terms ‘microcytic’, ‘macrocytic’ and ‘normocytic’ are used. Chronic anaemia can be associated with other conditions such as lymphoma, leukaemia, HIV and metastatic cancers. Management 402 When planning the care of anaemic patients (Table 19.4), it is useful to plan according to the basic needs assessment, putting the first priority on symptoms that can be life-threatening, such as blood loss and hypoxia. Table 19.4 Nursing management of anaemic patients Management Care of life-threatening complications Cardiac failure can occur in acute blood loss or where anaemia is experienced in conjunction with other conditions such as chronic obstructive pulmonary disease, cardiogenic shock or chronic pre-existing blood loss Dyspnoea in extreme cases causes collapse and respiratory failure Shock can occur where the condition is acute or untreated In life-threatening situations, early warning scoring needs to be performed; where necessary, patients’ level of care and dependency is increased to the level of intensive care monitoring Diagnosis and monitoring Recognise signs and symptoms Take routine blood tests and correctly interpret them Assist with investigations Administration of medication Administer prescribed medications when dietary supplementation is insufficient Administer blood transfusion as per local policy Dyspnoea Nurse the patient in the upright position to maximise lung expansion Use an integrated approach that concurrently manages both anxiety and breathlessness Physical demands will be great due to this symptom; care must be taken to assist patients with activities of daily living while promoting independence Nursing care of conditions related to haematological disorders Table 19.4 Chapter 19 (Continued) Management Blood transfusions Caution is required with elderly patients to ensure against overload of the circulatory system; blood may need to be given more slowly or spaced over 2 days Monitor fluid balance Administer erythropoietin if prescribed; this may improve quality of life as hospitalisation for blood transfusions will no longer be necessary Tiredness Patients can experience tiredness with the slightest exertion Educate patients that tiredness should lessen as the treatment takes effect Assistance should be given as required; periods of rest are encouraged Patients may be debilitated and may need to be on bed rest; maintain the Waterloo score and attend to pressure sore prevention and risk of deep vein thrombosis Anxiety and depression Patients may experience light-headedness and confusion due to hypoxia Anaemia may cause altered body image and loss of normal personal body state, which is replaced by a more dependent life-limiting persona This can increase anxiety and bring about feelings of depression Patients need: • Assistance to restore functioning • Reassurance • Monitoring • Understanding of the condition Specific management is required in severe cases Maintaining a safe environment Patients are susceptible to falls due to symptoms of anaemia Skin integrity is impaired, and skin is easily damaged so must be monitored carefully Educate patients on the maintenance of body heat and mobilisation Mental attention span is lessened; time off work and assistance in the home may be required A social services referral may be required following assessment Mouth care Monitor and record any injuries Patients may experience cracks at the corners of the mouth; instruction on mouth care and maintenance of nutrition is necessary Nutritional assessment and guidance Refer to a dietitian Reinforcement of essential foods is required Provide information leaflets Discharge planning Ensure patients are safe to go home Educate patients on self-care, recognition of anaemia and medications Where required, dietetic support is ongoing Arrange an outpatient appointment Alternatively, a follow-up appointment can be made with the GP Elderly people or those who struggle with family responsibilities may need social services support in place prior to discharge 403 Part 2 Adult Medical and Surgical Nursing Iron deficiency anaemia (microcytic anaemia) Iron deficiency anaemia, also referred to as microcytic anaemia, in which RBCs are characteristically small, is the most common anaemia in the world. Low levels of iron in the blood result in decreased haemoglobin synthesis and a decrease in oxygen-carrying capacity. Causes include: • • • • dietary deficiency of iron; loss of iron due to haemorrhage; poor absorption of iron from the gastrointestinal tract after gastrectomy; an increase in demand for iron due to pregnancy and growth. Iron is required daily for the production of haemoglobin and RBCs. After iron has been ingested, it is bound by transferrin, stored in the liver and readily available for use. When iron is deficient, erythropoiesis initially continues as normal; however, when stores are depleted, increased numbers of microcytic RBCs are produced in place of the normal mature RBCs. The normal total body iron content is 3 g; approximately 0.5–1 g of iron is absorbed per day through the walls of the intestinal tract, particularly the stomach and small intestine. Iron is found in foods such as meat, fish, eggs, chicken and pulses. In developing countries and social situations where it is difficult to access such foods, the lack of iron in the diet can predispose to iron deficiency anaemia. The patient’s clinical presentation together with a history of known risk factors can lead to a suspicion of iron deficiency anaemia (Table 19.5). Table 19.5 404 Diagnosis of iron deficiency anaemia Investigations based on clinical history and symptoms Specific signs and symptoms General signs and symptoms Haemoglobin below normal limits Brittle or spoon-shaped nails Tiredness Iron studies (ferritin) below normal level Brittle hair Anxiety Elevated reticulocyte count if bleeding Hypoxia Dyspnoea Faecal occult blood on rectal examination Cracks in the corner of the mouth (cheilosis) Cyanosis Gastroscopy to detect a source of bleeding Atrophy of the papillae on the tongue Collapse due to light-headedness Sigmoidoscopy Dyspnoea or breathlessness Poor peripheral circulation and sensitivity to the cold Colonoscopy Loss of appetite due to mouth sores as above Skin integrity impaired Radiology Craving to eat clay, starch or coal (pica) Hypothermia Ultrasound of the abdomen Dysphagia and glossitis with the formation of a pharyngeal web, called Paterson–Kelly syndrome (also known as Plummer–Vinson syndrome) Depression Nursing care of conditions related to haematological disorders Chapter 19 Management Nursing management is outlined in Table 19.4. Medications for iron deficiency anaemia include the following: • • • • Oral supplementation is given as iron sulphate 200 mg, three times a day with food for up to 6 months as required. As constipation is a side effect, stool softeners may need to be given. Alternative oral preparations may need to be considered if abdominal side effects are too limiting. Intramuscular iron supplements are given in malabsorption disorders or when there is poor compliance; these need to be given in a ‘Z–track’ manner to prevent tracking and discoloration of the skin. Macrocytic anaemia Macrocytic or megaloblastic anaemia is characterised by large stem cells or macrocytes. This occurs due to defective deoxynucleic acid (DNA) synthesis, leading to an ineffective and lower production of RBCs in the bone marrow. The RBCs that are produced are abnormally shaped, large and tend to have a reduced lifespan. Causes of macrocytic anaemia include: • • • • folate deficiency; vitamin B12 deficiency; prolonged exposure to certain therapies; alcohol abuse. Vitamin B12 deficiency This is the most common cause of macrocytic anaemia and results from a deficiency of vitamin B12. Vitamin B12 is found in meat and dairy products, cannot be synthesised in the human gut and is often added to cereals to fortify them. It is essential for the synthesis of DNA, with deficiency causing impaired cellular production of RBCs. Vitamin B12 is absorbed from the intestine in the presence of the intrinsic factor (IF), which is produced by the gastric mucosa. Lack of vitamin B12 and folic acid alters the structure of the IF, disrupts the production of RBCs and affects the functioning of the nervous system. Pernicious anaemia is an autoimmune condition in which the body’s antibodies target the gastric parietal cells and the IF. The exact mechanism of action is not clearly understood. It is speculated that it occurs in association with other autoimmune conditions and can be linked to hereditary factors such as blood group A in middle-aged females. Vitamin B12 deficiency due to low intake can be attributed to: • • • • • total or partial gastrectomy or gastrojejunostomy; gastric lesions; stomach cancer; alcohol abuse; malabsorption due to inflammatory bowel conditions such as Crohn’s disease. Signs and symptoms can take up to 2 years to present in patients with chronic inflammatory conditions or following gastric surgery. Apart from the general symptoms of anaemia, the clinical picture relates to damage to other bodily functions and includes: • • varying degrees of jaundice due to an increased breakdown of abnormal RBCs in the liver; degenerative neurological changes such as peripheral neuropathy and central changes that can be irreversible: • as vitamin B12 is necessary for the maintenance of myelin (the myelin sheath) that surrounds the nerves; • showing varying degrees of neurological disturbances when insufficiencies occur; 405 Part 2 Adult Medical and Surgical Nursing Box 19.9 • • • • • • • Management of patients with vitamin B12 deficiency As per the nursing management of anaemic patients (see Table 19.4) Treat as an outpatient or where necessary as an inpatient Monthly injections of cyanocobalamin are needed for patients who lack IF: • These can cause anaphylactic reactions so patients should be observed for at least 30 minutes after administration As this is not a curable condition, treatment is life-long Administer blood transfusions as necessary Provide care and support for jaundice and neurological dysfunction Arrange follow-up care with the GP or nurse practitioner in the community • • • • • with an initial presentation of poor vision, unsteady gait, ataxia and loss of sympathetic nervous system function affecting elimination and sexual functioning; untreated neurological symptoms, which can be fatal. uncontrolled elimination or diarrhoea; impaired balance and perception of self in space; smooth tongue. The nursing management is outlined in Box 19.9. 406 Normocytic anaemia Normocytic anaemia refers to conditions in which RBCs are relatively normal in size and haemo­­ globin content but there are an insufficient number of cells. Three main conditions fall under this categorisation: • • • Aplastic anaemia Sickle cell anaemia Haemolytic anaemia. Aplastic anaemia If left untreated, aplastic anaemia is a rare yet life-threatening condition. There is a significant decrease in blood cell production in the bone marrow, which leads to a deficiency of RBCs, WBCs and platelets. Fat cells proliferate to replace the stem cells. When all three cells lines are deficient, patients are said to be pancytopenic. Up to 30% of aplastic anaemia is thought to be caused by: • • • • • • chemical compounds such as benzene; cytotoxic medications, for example busulfan; other medications like chloramphenicol and antiepileptic drugs; ionising radiation, either therapeutic or non-therapeutic; viral infections, for example hepatitis; bone marrow infiltration in diseases such as myeloma, or by metastases. The remaining 70% of causes are said to be idiopathic (i.e. as having no detectable cause). Symptoms are insidious; 1–2 months can elapse following exposure to a causative factor prior to its onset. Classical symptoms of anaemia are present in addition to possible infections and bleeding. It may not be obvious on taking the history that there has been an exposure to a causative factor. The diagnosis is usually made from a laboratory analysis and the clinical features. A full blood count and blood film will reveal pancytopenia; however, a bone marrow aspirate is the only way to achieve a definitive diagnosis. Management is outlined in Box 19.10. Nursing care of conditions related to haematological disorders Box 19.10 Chapter 19 Management of aplastic anaemia Medical treatment • Urgent hospital admission • Treatment of the presenting symptoms with: • Blood transfusion • Platelets • Intravenous antibiotics • Stem cell or bone marrow transplant: • Assess for: — human leucocyte antigen matching — medical fitness • Admit to hospital for at least 4 weeks to enable regeneration of the bone marrow • If successful, this can bring about a cure for aplastic anaemia • Regular blood test screening for non-transplanted patients • Medications: • Corticosteroids (methylprednisolone) • Ciclosporin, given alone or with antithymocyte or antilymphocyte globulin; these suppress the immune cells responsible for destroying the bone marrow • Stimulating growth factors for the regeneration of blood components • May be administered on either an inpatient or outpatient basis, as the patient’s condition dictates • Potential side effects are life-threatening due to bone marrow depression • This course of treatment is used for moderate aplastic anaemia or for those not eligible for stem cell or bone marrow transplant Nursing care • Administer blood transfusion and anti-infection therapy • For patients on corticosteroids: • Monitor blood sugars • Daily urinalysis – if glucose is detected, check the blood glucose for secondary diabetes mellitus; insulin may be required if the result is positive • Blood pressure monitoring • Give information on side effects, e.g. mood changes • Administer in the morning to avoid sleep disturbance • Educate patients on: • Treatments, medications and their side effects • Prevention of infection • Dietary support and maintenance of good supplies of essential nutrients for blood cell regeneration • Ongoing management for non-transplanted patients, including education to detect for signs of pancytopenia • Monitoring of transplant patients for up to 2 years, observing for any graft versus host reaction • Regular bone marrow aspiration to ascertain disease status 407 Part 2 Adult Medical and Surgical Nursing Sickle cell anaemia 408 Sickle cell anaemia is a hereditary chronic haemolytic anaemia. Sickle cell trait and sickle cell disease are found in individuals of African-Caribbean, Middle Eastern, East European, Indian and Pakistani origin. It is thought that the sickle cell trait helps to provide protection against contracting malaria in these countries. Sickle cell conditions are characterised by the presence of abnormal haemoglobin molecules (HbS), which result from the replacement of one amino acid molecule (valine) with another amino acid (glutamic acid). In the heterozygous variant, the person inherits the abnormal haemoglobin HbS gene from one parent and the normal HbA gene from the other parent. This person will inherit the HbSA sickle cell trait and may not be aware of this abnormality unless tested for it or exposed to hypoxic conditions, at which time the individual will become symptomatic. The trait will be passed onto the next generation. In a homozygous variant, the abnormal haemoglobin is inherited from both parents and the person will suffer from sickle cell anaemia. When the abnormal haemoglobin molecule is deoxygenated, it causes the RBCs to adopt a sickle-like shape. The significance of the stiff, sickle-like shape, which occurs in stressful or environmentally cold conditions, is that it is fixed, unlike that of normal RBCs, whose concave shape can alter, enabling them to pass into the thin capillaries in all conditions. Sickled cells become trapped and obstruct the blood flow causing ischaemia (lack of blood supply), which in turn results in pain, often of a severe nature. Once the sickled RBCs have been reoxygenated, they regain their normal shape. However, when a person encounters repeated crises, known as sickle cell crises, the ability of RBCs to return to their normal shape diminishes as the elasticity of the cell walls weakens, leading to haemolysis. The mechanism by which sickle cell crises occurs is unclear, but a crisis can last from several hours to several weeks. Certain conditions and trigger factors are known to precipitate a crisis, including: • • • • • • • • hypoxia, such as at high altitude or during strenuous exercise; anaesthesia; infection; pregnancy; dehydration; cold climatic conditions; excessive alcohol; emotional stress. Symptoms include: • • • • • • • pain and swelling of the occluded blood vessels, such as in the abdominal cavity, or stasis in the vessels of the hands and feet; pulmonary hypertension; tachycardia; association with infection, particularly of a bony nature; elevated platelet and WBC counts that contribute to the vessel occlusion; haematuria; persistent erection of the penis. The nursing management of sickle cell anaemia is shown in Table 19.6. Polycythaemia Polycythaemia is a condition characterised by an abnormally higher than normal level of haemoglobin and carries with it a high risk of clotting. The condition can be primary or secondary. Primary polycythaemia is due to a mutation in the stem cells to produce excessive numbers of RBCs; secondary polycythaemia is due to hypoxia at high altitudes or overuse of erythropoietin. Regular venesection (removal of blood from the circulation) is the treatment of choice. Nursing care of conditions related to haematological disorders Table 19.6 Chapter 19 Nursing management of patients with sickle cell anaemia Parameter Nursing management Pain management Patients experience moderate to severe pain Assess pain using a pain assessment tool Medications: • Mild to moderate pain – anti-inflammatory drugs • Severe pain – opiates Bed rest Oxygen therapy Blood transfusion As per local policy Vital signs Record these 4-hourly Treat infection promptly Fluid balance Intravenous therapy may be indicated to promote blood flow Counselling Designated haematology units may have sickle cell specialists who can provide families with genetic counselling Discharge advice Educate the patient on: • What triggers a crisis • What to do • Whom to contact when the situation occurs Pregnancy Specialist pregnancy care for sickle cell patients, in collaboration with the haematology team Haemophilia Haemophilia is an inherited disorder that results from a life-long deficiency of clotting factors (factor VIII; haemophilia A) or Christmas factor (factor IX; haemophilia B). Treatment involves replacement of the clotting factors and specialist haematological monitoring and intervention. Thrombocytopenia Thrombocytopenia is a reduced platelet count resulting from excessive bleeding or an inability of the bone marrow to produce a normal number of platelets. Types of thrombocytopenia • Idiopathic thrombocytopenic purpura (ITP; acute or chronic) is an autoimmune disorder in which • • the body makes antibodies, mediated by B lymphocytes, that destroy platelets. Acute ITP is more common in children, whereas the chronic form is associated with adults. Thrombotic thrombocytopenic purpura occurs when small blood clots form throughout the body; a large number of platelets are used up in this, reducing the count in the peripheral circulation. Haemolytic-uraemic syndrome is characterised by reduction in both platelet and RBC levels. It can be a side effect of ciclosporin therapy or result from Escherichia coli infection. 409 Part 2 Adult Medical and Surgical Nursing Box 19.11 • • • • • • • • • Management of thrombocytopenia Platelet transfusion Steroid treatment Splenectomy may be indicated Treat secondary causes Monitor: • Sites that are bleeding • Vital signs to allow early recognition of a worsening condition Regular mouth care – to maintain oral hygiene due to bleeding tendencies Management of dehydration and fluid balance Educate patients on observing for and preventing bleeding at home Avoid: • Constipation, by encouraging dietary fibre • Aspirin, as it can interfere with platelet function The causes of thrombocytopenia are: 410 • • • • • • • • chemotherapy; radiotherapy; infection; increased body heat; pernicious anaemia; systemic lupus erythematosus; HIV infection; heparin-induced thrombocytopenia. The signs and symptoms include: • • • • • • unexpected bleeding; petechiae; gastrointestinal bleeding; joint pain; epistaxis; heavy menstrual bleeding. Box 19.11 outlines the management of thrombocytopenia Disseminated intravascular coagulation Disseminated intravascular coagulation is a serious life-threatening condition that results from simultaneous overactivation of the coagulation system and fibrinolytic pathways. This leads to a dual effect of widespread clotting with a propensity for bleeding. The causative factors include transfusion reactions, leukaemia and cytotoxic treatment. It requires intensive monitoring and specialist haematological input. A balance needs to be achieved between replacing clotting factors, by means of infusing cryoprecipitate and fresh frozen plasma, and anticoagulating patients with heparin. M