Hematology Lecture Notes PDF

Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN Adult bone marrow produces :  175 b RBCs...

Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN Adult bone marrow produces :  175 b RBCs  70 b neutrophils  175 b platelets Blood Vessels  Veins  Arteries  Capillaries MEDICAL AND SURGICAL NURSING Blood Forming Organs Hematology  Liver  Thymus Lecturer: Mark Fredderick R. Abejo RN, MAN  Spleen  Bone Marrow  Lymph nodes  Lymphoid organ OVERVIEW OF THE STRUCTURE AND FUNCTION OF THE HEMATOLOGIC SYSTEM CHARACTERISTICS:  Color o Arterial Blood HEMATOLOGY – the scientific study of the structure and o Venous Blood functions of blood in health and in disease.  Fraction of body weight 8%  Volume Female: 4 -5 L BLOOD – is the circulatory fluid of the CV system which is  Male: 5 - 6 L circulating constantly through a closed circuit of tubes.  Temperature 38 C ( 100.4 F )  pH 7.35 - 7.45 FUNCTIONS:  Viscosity (relative to water) ► supply oxygen from the lungs and absorbed nutrients Whole blood: 4.5-5.5 from the GIT to the cells Plasma: 2.0 ► remove waste products from tissues to the kidneys,  Specific gravity - 1.048 to 1.066 ( 1.055 – 1.065) skin and lungs for excretion  Composition: ► transport hormones from their origin in the endocrine glands to other parts of the body Liquid phase: PLASMA (55%) ► protect the body form dangerous microorganism ► promote Hemostasis ( to stop bleeding) ► - A light yellow substance which is one of ► regulate body temperature by heat transfer – the major fluids of the body. Major function vasoconstriction and vasodilation is to maintain the blood volume within the vascular compartment Hematopoiesis  Process of blood cell production.  At birth, it is accomplished in the liver, spleen, thymus, lymph nodes and red bone marrow.  After birth, it is confined in the red bone marrow ( but some WBCs are still produced in the lymphatic tissues).  During childhood, all blood cells are essentially produced in marrow sites of the flatbones of the skull, clavicle, sternum, ribs, vertebrae, and pelvis  After puberty, hematopoiesis becomes localized within the flatbones of the sternum, ilium, ribs, and vertebrae, sometimes occurring in the proximal ends of long bones (humerus and femur)  All formed elements come from one stem cell or the ► 92% Water HEMOCYTOBLAST. Cell differentiation gives rise ► Serum to the cell lines with the help of growth factors. ► Plasma Proteins – all produced in the LIVER  Albumin – most abundant, maintains osmotic pressure  Globulin  Alpha – transports bilirubin, steroids and hormones  Beta – transports iron and copper  Gamma – transports immunoglobulins  Prothrombin – clotting factor  Fibrinogen – clotting factor ► Electrolytes ( Na+,,Ca 2+,, HCO3ˉ, CIˉ), ► Miscellaneous (less than 1%): sugars, fats, vitamins, hormones 1 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN Solid phase: FORMED ELEMENTS /  Non-granulocytes (agranulocytes) CELLELAR COMPONENTS (45%) 1. Monocytes – largest WBC (macrophage)  Upon release in the bone marrow and travel RBC (Erythrocytes) – only component which is to the different tissues, it is just a hypoactive anucleated phagocytic cell, become a Macrophage N = 4-6 million/mm3 when it attaches to the endothelium of  Are biconcave discs (AKA discocytes) which are less organs and performs its full phagocytic than 7.5 micrometers in diameter. function.   Long term phagocytosis (months)  ERYTHROPOIESIS process of formation of RBC  KUPFFER – kidneys  ERYTHROPOETIN hormone produced primarily by  HISTOCYTES – skin and subcutaneous the kidney; necessary for erythropoiesis  ALVEOLAR macrophage – lungs  HEMOGLOBIN iron-containing protein of RBC,  MICROGLIA – CSF delivers oxygen to tissue  MACROPHAGE - blood  Carries about 200-300 million molecules of 2. Lymphocytes hemoglobin(heme-globin-iron) that attach oxygen within each RBC, responsible for 97% of O2 transport  B cells (bone marrow)  Molecules of Hgb (carries oxygen) differentiated in the bone marrow, antibody- ( Ave. 12 - 18 g/dL) mediated immune response (Humoral) Female: 12-16 g/dL  For immunity Male: 13-18 g/dL  T cells (Thymus)  HEMATOCRIT – red cell percentage in whole blood  For immunity (three times of normal Hemoglobin)  Differentiated in the Thymus and lives FEMALES: 36-42% long cell-mediated response MALES: 42-48%  Target site of HIV  AIDS incubation period: 6 mos –  Substances needed for maturation of RBC 5 years; window period 6 mos 1. FOLIC ACID – prevents neural tube deficit; needed in the FIRST trimester of pregnancy  AZT  ZIDOVUDINE or 2. IRON – needed in the THIRD trimester RETROVIR : drug of choice for 3. VIT B12 (Cyanocobalamin) aids 4. VIT C (Ascorbic Acid)  WESTERN BLOT – confirmatory 5. VIT B6 (Pyridoxine) test for aids 6. INTRINSIC FACTOR (released in stomach’s  Kaposi’s SARCOMA parietal cells)  NK cells  Normal lifespan – 80-120 days  Natural killer cells  Spleen – kills RBCs in the red pulp  Anti-tumor and anti-viral properties WBCs (Leukocytes) Humoral (Antibody-Mediated) Immune Response N = 5,000-10,000/mm3 B Cells – Matures into Plasma Cells responsible for Antibody  Granulocytes production 1. Neutrophils – most abundant, 60-70% of total WBCs 5 Classes of Immunoglobulins (MADGE) :  First line of defense, most common type of – Immunoglobulin M (IgM) leukocyte but a short lifespan of only 10-12 1st immunoglobulin produced in an immune hours making them ineffective in destroying responsepresent in plasma, too big to cross infectious agents membrane barriers  Helpful in localizing the infection and in – Immunoglobulin A (IgA) immobilizing the pathogens until other Sound in body secretions like saliva, tears, mucus, WBCs arrive bile, milk & colostrum  for acute inflammation – Immunoglobulin D (IgD) Present only in the plasma & is always attached to the 2. Eosinophils– allergic reactions B Cell  Weak phagocytic action, elevated during – Immunoglobilin G (IgG) asthma attacks. 80% of circulating antibodies  Usually activated during parasitic invasion Can cross the placenta and provide passive immunity (Schistosomes / blood flukes) Present in all body fluids  Lifespan= hours to 3 days – Immunoglobulin E (IgE)  Modulates or reduce IgE mediated allergic Responsible for Allergic & hypersensitivity reactions reactions Stimulates Mast cells & Basophils to release Histamine which mediates inflammation & the 3. Basophils – not phagocytic in nature, they are allergic response mediators in inflammatory process.  Involved in the release of chemical mediators PLATELETS (Thrombocytes)  Prostaglandin  Serotonin N = 150-450 thousand mm3  Histamine  Bradykinin  Promotes hemostasis  prevention of blood loss   For inflammation promote clotting mechanisms  MEGAKARYOCYTES – immature/baby platelets; target site of DHF  Normal lifespan: 9-12 days 2 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN b. Sex  women have lower hemoglobin and hematocrit levels, more prevalence of agranulocytosis  hemophilia, bleeding expressed among males  because some laboratory results are sex-specific c. Race  Blacks have lower hemoglobin levels than whites (more prevalent sickle cell anemia) d. Family history  Because some hematologic disorders are inherited: – Anemia – Thrombocytopenia – Bleeding disorders (hemophilia and Von Willebrand’s Dse), – Congenital Blood Disorder (Sickle Cell anemia) – Jaundice, infections, delayed healing, – Cancer – Autoimmune dse (aplastic anemia, pernicious anemia) Hemostasis (Blood Clotting) e. Congenital lack of the intrinsic factor Three Major Phases Modifiable 1. Platelet Plug Formation – Platelets adhere and stick to vessel lining that are a. Exposure to certain chemical and drugs damaged forming a Platelet Plug or White  Radiation overexposure Thrombus  Anti-neoplastic drugs/ chemotherapy – Platelets release chemicals to attract more platelets  Chemical Oxidants (e.g benzene, nitrites, lead, arsenic, to the injured site etc.) 2. Vascular Spasms  Drugs (chloramphenicol, sulfonamide, anti-convulsant, – Platelets release Serotonin causing spasms of the streptomycin, hair dyes blood vessel, constricting it & decreasing blood flow 2. History – Chief complaint 3. Coagulation or Blood Clotting  Disorders of the hematologic system often affects all – Thromboplastin is released by damaged cells organs and tissues – plasma Clotting Factors form an activator that  Determine: triggers the Clotting Cascade – a Blood Clot is formed o Onset – Serum is squeezed out within the hour pulling the  abrupt or gradual? ruptured edges together  since childhood or recent o Allergen triggered response? Seasonal? Plasma Clotting Factors o How long do the allergic manifestations last? Relieved I Fibrinogen or persist once the allergen is removed? II Prothrombin III Tissue Thromboplastin o Quality and quantity IV Calcium  How severe? Massive bleeding? How long does V Proacelerin it last? VII Proconvertin  How long do the bleeding episodes last and how VIII Antihemophilic Factor severe they are? IX Christmas Factor  Does blood ooze from a site or does sudden X Stuart – Prower Factor massive bleeding occur? XI Plasma Thromboplastin  How often do bleeding episodes occur and how Antecedent long do they last? XII Hageman Factor  What does the client do to stop them? XIII Fibrin Stabilizing Factor  Is there any break in skin integrity? Compatible Blood Types  Swelling? Edema? Fever? Pain? Tenderness? Pruritus? Redness? Or drainage? Assessment of the Hematologic System  Note allergic manifestations such as rhinitis, sneezing, nasal stuffiness, postnasal drip, sore 1. Risk Factor Analysis throat, voice changes, hoarseness, wheezing, persistent cough, dyspnea, malaise, fatigue, Non-Modifiable tearing or altered hearing acuity. a. Age o Severity and location  immune response is diminished in both very young and  Rest can alleviate fatigue? Bleeding of joints? very old  Can rest alleviate fatigue? (s/sx of anemia)  anemia prevalence increases with age  Ask how activities and activity tolerance changed  folic acid deficiency in growth spurt (infants and over time adolescents)  Does the client bruise easily?  because some laboratory results are age- specific  Has bleeding in the joints?  Number and saturation of sanitary pads  Anaphylactic reactions? Or simple allergic response? 3 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN o Precipitating factors 6. LUNGS  Anticoagulant? – bleeding – dyspnea, orthopnea (anemia)  Bone Marrow suppression – anemia, leukemia, and thrombocytopenia 7. CARDIOVASCULAR SYSTEM  Antineoplastic drugs? Antibiotics? Radiation? – tachycardia, palpitation, murmurs, angina (anemia)  Infectious agents? Corticosteroids or immunosuppressive drugs? 8. GIT  Allergic triggers (inhalants such as pollens and – dysphagia (mucous membrane atrophy due to iron def. dust, contact agents such as dyes and cosmetics, anemia) ingested agents such as foods and drugs, – abdominal pain( bleeding) injectable agents such as drugs, vaccine and – hepatomegaly, splenomegaly (hemolytic anemia) insect venom) – hematemesis, melena (thrombocytopenia and bleeding disorders o Aggravating and relieving factors  salicylates containing OTC may aggravate 9. GUT bleeding – hematuria (bleeding disorders)  what relieves allergic manifestations – amenorrhea and menorrhagia – (iron def. and bleeding do) 3. Past Medical History 10. MUSCULOSKELETAL a. Major illnesses and hospitalization – joint pain (hemophilia)  Previous hematologic problems – back pain  Surgical procedures that may affect the hematologic – sternal tenderness and bone pain system – (sickle cell crisis)  Liver problems –  Any bleeding disorders 11. NERVOUS SYSTEM – headache, confusion o How long was the bleeding problem? Do any – (anemia, polycythemia) members of the family have a history of – brain hemorrhage bleeding? – (thrombocytopenia and bleeding disorders) o Is bleeding linked with any specific event or – peripheral neuropathy, procedure? Does it occur with menses or – paresthesis, loss of balance (pernicious anemia) following minor trauma? Any frequency of nose bleeding? Does he bruise easily? Any petechiae? o How severe are any of the bleeding episodes? DIAGNOSTIC PROCEDURES What is the durations? o Any history of hepatic/ splenic or renal disease? Recently taken medications? 1. COMPLETE BLOOD COUNT b. Medications- aspirin, chloramphenicol, antineoplastic drugs a. RBC count- # of RBCs/ mm3 of blood, to diagnose anemia c. Allergies- Hx of allergies, BT and/reactions and ploycythemia d. Family History b. Hemoglobin- # of grams of hgb/ 100ml of blood; to measure the oxygen-carrying capacity of the blood 4. Psychosocial Hx and Lifestyle c. Hematocrit – expressed in %; measures the volume of RBCs a. Occupation- exposure to chemicals and radiation in proportion to plasma; used also to diagnose anemia and b. Habits- nutritional, substance abuse, alcohol abuse ploycythemia and abnormal hydration states d. RBC indices- measure RBC size and hemoglobin content a. MCV (mean corpuscular volume) b. MCH (mean corpuscular hemoglobin) Review of Systems/ Physical Examination c. MCHC (mean corpuscular hemoglobin concentrarion) 1. SKIN e. Platelet count- # of Platelet/ mm3; to diagnose – pallor thrombocytopenia and subsequent bleeding tendencies – ruddy skin f. WBC count- of WBCs/ mm3 of blood; to detect infection or – jaundice inflammation – dry skin, brittle, spoon shaped with longitudinal ridges g. WBC Differential count- determines proportion of each WBC in a sample of 100 WBCs; used to classify leukemias 2. EYES Normal Values – visual disturbances (anemia and polycythemia) – blindness (retinal hemorrhage related to RBC: Women – 4.2-5.4 million/mm3 thrombocytopenia and bleeding do) Men – 4.7-6.1 million/mm3 – scleral jaundice (hemolytic anemia) Hgb: Women – 12-16 g/dl Men – 13-18 g/dl 3. EARS Hct : Women – 36-42% – vertigo, tinnitus (severe anemia) Men – 42-48% – bleeding in auditory canal WBC: 5000-10,000/mm3 – (bleeding do) Granulocytes Neutrophils: 55-70% Eosinophils: 1-4% 4. NOSE Basophils: 0.5-1.0% – epistaxis (thrombocytopenia and bleeding disorders) Agranulocytes Lymphocytes: 20-40% 5. MOUTH Monocytes: 2-8% – smooth, glossy, bright red tongue and sore tongue Platelets: 150,000-450,000/mm3 (pernicious and Fe def. anemia) – gingival bleeding (thrombocytopenia and bleeding disorders) 4 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN 2. PERIPHERAL BLOOD SMEAR 7. COAGULATION SCREENING TESTS To determine the variations/ abnormality in RBCs, WBCs and a. Bleeding Time – measures the ability to stop bleeding after Platelets: normal size and shape (normocytes) and normal color small puncture wound (normochromic) b. Partial Thromboplastin Time (PTT) – used to identify deficiencies of coagulation factors, prothrombin and fibrinogen; 3. DIRECT ANTIGLOBULIN EST (Coomb’s Test) monitors heparin therapy. c. Prothrombin Time (Pro-time) – determines activity and Used in cross matching blood when transfusion reaction occurs, interaction of the Prothrombin group: factors V (preacclerin), test umbilical cord for Erythroblastosis fetalis and diagnose VII (proconvertin), X (Stuart-Power factor), prothrombin and acquired hemolytic anemia fibrinogen; used to determine dosages of oral anti-coagulant. 4. INDIRECT ANTIGLOBULIN TEST Normal Values Identifies antibodies to RBC antigens in the serum of clients Reticulocytes: 25-75 x 10 9/L who have greater than normal chance of developing transfusion Bleeding Time: 2.75-8 min reactions. Partial Thromboplastin Time (PTT): 20-35 sec. Prothrombin Time (PT): 12-14 sec. 5. RETICULOCYTE COUNT Used to determine the responsiveness of the bone marrow to the BLOOD DISORDERS depletion of circulating RBCs (probably due to hemolytic anemia or hemorrhage) I. IRON DEFICIENCY ANEMIA (IDA) – chronic microcytic anemia due to inadequate absorption of iron 6. BONE MARROW ASPIRATION and BIOPSY leading to hypoxemic tissue injury Used to determine size and shape of RBCs, WBCs and platelet A. INCIDENT RATE precursors and to examine various maturational abnormalities. 1. Developed countries (d/t high intake of cereals and milk) 2. Accidents (adults) 3. Tropical areas (blood sucking parasites) 4. Women 15-35 (reproductive age) 5. Common among the poor (poor nutrition) B. PREDISPOSING FACTORS 1. Chronic blood loss  Trauma  Menstruation  GIT bleeding  Hematemesis  Melena (UGIB)  Hematochezia (LGIB) (d/t E. histolytica  DOC: metronidazole) 2. Inadequate intake of iron rich food 3. Inadequate absorption of iron due to  Chronic diarrhea  R/t increased cereal intake with decreased animal CHON ingestion, related to subtotal gastrectomy  Malabsorption syndrome 4. Improper cooking of foods C. SIGNS AND SYMPTOMS 1. Usually asymptomatic, first sign: weakness and fatigue 2. Headache, dyspnea, dizziness, palpitations, cold sensitivity, generalized body malaise, pallor 3. Brittleness of hair, spoon shaped nails (koilonychia  180 degrees ang normal)  d/t Nursing Responsibility hypoxia  atrophy of epidermal cells 4. Atrophic glossitis, stomatitis, dysphagia Preprocedure - explain the purpose, obtain consent - inform client of pain or of what to D. DIAGNOSTICS: ALL DECREASED! expect 1. RBC - give sedatives as ordered 2. Hgb 3. Hct Procedure - place patient in lateral position, with 4. Reticulocytes site of aspiration uppermost 5. Iron - clean pt’s skin with antiseptic sol’n - administer local anesthesia to numb E. NURSING MANAGEMENT skin and subcutaneous tissues 1. Monitor for signs of bleeding of all hema test - apply ice on the contralateral side to including urine, stool and GIT relieve pain 2. Enforce CBR so as not to overtire patient 3. Encourage increased iron diet (Damo!  green Postprocedure - apply pressure until bleeding stops leafy vegetables, California raisins, organ meat, - check site frequently for bleeding legumes, yolk, dried foods - give pain relievers to relieve pain 5 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN 4. Avoid tannates in tea and coffee because it C. DIAGNOSTICS impairs iron absorption 1. SCHILLING’S TEST – indicates decreased 5. Administer medications as ordered reabsorption of vitamin B12; confirms presence  Oral iron preparations (300mg OD) of pernicious anemia  FeSO4, Fe Fumarate, Fe Gluconate  NURSING MANAGEMENT D. NURSING MANAGEMENT 1. Administer with meals to lessen 1. Enforce complete bed rest (consistent to all types GIT irritation of anemia) 2. Use straw for liquid form 2. Administer Vit B12 injections at MONTHLY 3. Administer with orange juice or intervals for lifetime as ordered; common site: vitamin C to facilitate absorption 4. Inform client of SE/monitor for dorso and ventrogluteal, no drug toxicity because a. Anorexia it is water soluble and is easily excretable; oral b. Nausea and vomiting forms might develop tolerance. c. Abdominal pain 3. Increase caloric intake, CHON, CHO, Fe, Vit C d. Diarrhea/constipation 4. Encourage client to use soft bristled toothbrush e. Melena and avoid irritating mouthwashes (remember  Parenteral Iron Preparations there are mouthsores!)  Iron Dextran IM or IV 5. Avoid heat application (there is numbness  Sorbitex IM remember?)  may lead to burns  NURSING MANAGEMENT 1. Administer using z-tract method III. APLASTIC ANEMIA – stem cell disorder leading to bone to prevent discomfort, marrow depression  pancytopenia (all blood cells discoloration and leakage 2. Avoid massaging of injection site decreased)  anemia, leucopenia, thrombocytopenia instead encourage pt. to ambulate to facilitate absorption A. PREDISPOSING FACTORS 3. Monitor SE 1. Chemicals a. Pain at injection site  Benzene and its derivatives b. Localized abscess 2. Irradiation c. Lymphadenopathy 3. Immunologic injury d. Fever and chills 4. Drugs e. Pruritus and urticaria Hypotension   Broad spectrum antibiotics anaphylactic shock   Chloramphenicol epinephrine  Sulfonamides (Bactrim…)  Chemotherapeutic Agents RBC (80-120 days)  destroyed in Spleen  Hgb  Nitrogen Mustard (Anti-metabolite) Hemoglobin breaks into:  Vincristine (plant alkaloid)  Globin  Methotrexate (alkylating agent)  Heme  Phenylbutazones A. Ferrous 1. Bilirubin 2. Biliverdin B. SIGNS AND SYMPTOMS B. Ferritin 1. Headache, dizziness, dyspnea, palpitations, Early sign of anaphylactic shock: dyspnea pallor, cold sensitivity, generalized body malaise 2. Leukopenia (increased susceptibility to infections) II. PERNICIOUS ANEMIA – chronic anemia resulting from 3. Thrombocytopenia deficiency of intrinsic factor leading to hypochlorhydria  Petechiae  Ecchymoses (decreased HCl secretion); IDIOPATHIC  Oozing of blood from venipuncture sites A. PREDISPOSING FACTORS C. DIAGNOSTICS 1. Subtotal gastrectomy 1. CBC – pancytopenia 2. Hereditary factors 2. Bone Marrow Biopsy or Aspirate 3. Inflammatory disorders of the ileum  Posterior iliac crest 4. Autoimmune  Would reveal fat necrosis in the bone 5. Strictly vegetarian diet marrow  Stomach (widest area of alimentary canal)  Argentaffin/oxyntic/parietal cells in stomach D. NURSING MANAGEMENT produces IF  promotes reabsorption of vit B12 (Cyanocobalamin)  maturation of 1. Removal of underlying cause RBC 2. BT as ordered  If absent IF dyspepsia  weight loss  3. Enforce complete BR so increase calories in diet 4. Administer O2 inhalation  Secretes HCl acid  it aids in digestion 5. Reverse isolation  Immature RBCs  sequestered in spleen  6. Monitor for signs of infection bilirubinemia  jaundice 7. Avoid IM, SQ or any venipuncture sites 8. instruct: use electric razor when shaving B. SIGNS AND SYMPTOMS 9. Medications as ordered 1. Headache, dizziness, dyspnea, palpitation, cold  Immunosuppressants via central venous sensitivity, pallor and generalized body malaise catheter 2. GIT changes  Anti-lymphocyte globulin (ALG) –  Mouth sores, Red beefy tongue, Dyspepsia given within 6 days – 3 weeks to or indigestion, Weight loss, Jaundice achieve maximum therapeutic effect 3. CNS changes – PA is the most dangerous form of anemia  Tingling sensation, Paresthesia, Ataxia, Psychosis 6 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN IV. SICKLED CELL ANEMIA - is a life-long blood disorder 2. Patients will take a 1 mg dose of folic acid daily characterized by red blood cells that assume an abnormal, for life. rigid, sickle shape 3. From birth to five years of age, they will also have to take penicillin daily due to the immature immune system that makes them more prone to early childhood illnesses. 4. Painful crises are treated symptomatically with analgesics; pain management requires opioid administration at regular intervals until the crisis has settled 5. The first approved drug for the causative treatment of sickle-cell anaemia, hydroxyurea, was shown to decrease the number and severity of attacks 6. Bone marrow transplants have proven to be effective in children F. NURSING MANAGEMENT 1. Administer O2 & Blood Transfusion as Rx 2. Maintain adequate hydration 3. Avoid tight clothing that could impair circulation. 4. Keep wounds clean and dry. 5. Provide bed rest to decrease energy expenditure and oxygen use. 6. Encourage patient to eat foods high in calories, CHON, with folic acid supplementation. A. PREDISPOSING FACTORS 7. Analgesics: 1. Hereditary factors o Acetaminophen 2. African ,South/Central American people and o Morphine Mediterranean countries o avoid aspirin as it enhances acidosis,which promotes sickling B. SIGNS AND SYMPTOMS 8. Avoid anticoagulants( sludging is not due to Related to anemia: clotting ). 1. Shortness of breath 9. Antibiotics. 2. Dizziness 10. Avoid activities that require so much energy. 3. Headache 11. Keep arms and legs from extreme cold. 4. Coldness in the hands and feet 12. Decrease emotional stress. 5. Pale skin 13. Provide good skin care 6. Chest pain Related to pain: V. THALASSEMIA MAJOR (Cooley’s anemia) 1. Sickle Cell Crisis - Sickle cell crises often affect  B - thalassemia refers to an inherited hemolytic the bones, lungs, abdomen, and joints. anemia, characterized by reduction or absence of the - A sickle cell crisis occurs when sickled red B-globulin chain in Hgb synthesis blood cells form clumps in the bloodstream.  Fragile RBC & short life span These clumps of cells block blood flow through  Autosomal recessive pattern of inheritance the small blood vessels in the limbs and organs.  Insufficient B-globulin chain synthesis allows large This can cause pain and organ damage amounts of unstable chains to accumulate  Precipitates of alpha chains that form cause RBC’s to Complications of Sickle Cell Crisis be rigid & easily destroyed, leading to severe  Hand-Foot Syndrome hemolytic anemia = chronic hypoxia  Splenic Crisis  Skeletal deformities: pathologic fractures  Infections  Hemosiderosis – excess iron supply, which leads to  Acute Chest Syndrome iron deposits in the organ tissues leading to decreased  Pulmonary Arterial Hypertension function  Delayed growth and puberty  Stroke A. CLINICAL MANIFESTATIONS  Eye problem 1. onset is usually insidious  Priapism 2. Sx are primarily related to progressive anemia,  Gallstone expansion of marrow cavities of the bone &  Ulcers of the leg developmemnt of hemosiderosis  Multiple organ failure 3. Early Sx often include progressive pallor, poor feeding & lethargy C. DIAGNOSIS 4. Further signs: hemorrhage, bone pain, exercise 1. CBC reveals hgb of 6-8g/dl , increase intolerance, jaundice, & protuberant abdomen reticulocyte count, low hct 5. hemosiderosis of the eye and lungs 2. HemoglobinElectrophoresis, confirmatory diagnosis for sickled cell anemia B. DIAGNOSTIC EVALUATION 3. Urinalysis – UTI Decrease hemoglobin 4. Chest Xray and CT scan – pulmonary RBC= increase in number complication Hgb elctrophoresis – elevated levels of HgF ( doesn’t hold O2 E. MEDICAL MANAGEMENT well ) 1. Children born with sickle-cell disease will – limited amount of HgA undergo close observation. 7 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN 2. Assess for early S/Sx of thromboembolic C. COMPLICATIONS complications : swelling of limbs, increased 1. Splenomegaly warmth, pain 2. Growth retardation in the second decade 3. Monitor CBC & assist with phlebotomy as 3. Endocrine abnormalities : ordered – delayed development of secondary sex Patient Education characteristics – most boys fail to undergo  Educate about risk of thrombosis; encourage puberty, girls – menstruation problems patient to maintain normal activity pattern & – DM – due to iron deposits in the pancreas avoid long periods of rest – Hypermetabolic rates  Avoid hot showers 4. Skeletal complications  Report @ regular intervals for follow up – Frontal & parietal bossing (Enlargement) blood – Maxillary hypertrophy – leading to occlusion VII. HEMOPHILIA – Premature closure of epiphyses of long  Hereditary coagulation defect, usually bones transmitted to affected male by female carrier – Osteoporosis & pathologic fractures through sex – linked recessive gene, resulting in 5. Cardiac problems: pericarditis & CHF – usual prolonged clotting time. cause of death  Most common type is Hemophilia A or Classic Hemophilia - factor VIII deficiency (called D. MANAGEMENT Antihemophilic Factor / AHF) 1. Frequent and regular transfusion of packed  Hemophilia B or Christmas Disease – factor IX RBC’s to maintain Hgb levels above 10 g/dL deficiency (called the Christmas Factor) 2. Iron chelation therapy with deferoxamine  Male inherits hemophilia from their mothers, and (Desferal) – reduces toxic effects of excess iron females inherit the carrier status from their & increases iron excretion thru urine & feces fathers. 3. Splenectomy – Found predominantly, but not exclusive, in 4. Supportive management of symptoms male offsprings 5. Bone marrow transplant  Bleeding occurs due to impaired ability to form 6. Prognosis and Survival rate is poor because of no fibrin clot known cure 7. Often fatal in late adolescence or early adulthood VI. POLYCYTHEMIA VERA  Underlying cause is unknown  Hyperplasia of all bone marrow elements > increase RBC mass > increase blood volume viscosity > decrease marrow iron reserve > Splenomegaly A. ASSESSMENT 1. Reddish purple hue of skin & mucosa, pruritus 2. Splenomegaly, hepatomegaly 3. Epigastric discomfort, abdominal discomfort 4. Painful fingers & toes from paresthesias 5. Altered mentation 6. Weakness, fatigue, night sweats, bleeding tendency 7. Hyperuricemia – from increased RBD formation and destruction B. DX TESTS 1. CBC 2. BONE MARROW ASPIRATION & Biopsy C. MANAGEMENT 1. HYPERVISCOSITY = phlebotomy @ intervals determined by CBC A. ASSESSMENT results to decrease RBC mass 1. Abnormal bleeding in response to trauma or =generally 250-500ml removal @ a time surgery. (muscles/joints) 2. HYPERPLASIA 2. Joint bleeding causing pain, tenderness, = myelosuppressive therapy, swelling, and limited range of motion. = generally using hydroxyurea or IV radioactive 3. Tendency to bruise easily. phosphorus (32P), biologic response 4. Epistaxis modifier, ie alpha interferon 5. Hemarthrosis (bleeding in joints causing pain, 3. HYPERURICEMIA= allupurinol swelling and limited movement) (Zyloprim) 4. PRURITUS = antihistamines (cimitidine), B. IMPLEMENTATION low dose acetyl salicylic acid; certain anti- 1. Administer factor VIII concentrate. depressants (paroxetin), phototherapy, 2. Monitor for bleeding and maintain bleeding cholestyramine precautions. 3. Monitor for joint pain; IMMOBILIZE the affected extremity if joint pains occur. D. NURSING INTERVENTION 1. Encourage/assist ambulation 4. Monitor urine for hematuria. 8 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN 5. Instruct the parents regarding activities for the B. Dx Tests: child, emphasizing the avoidance of contact 1. marked decrease of blood platelets sports. 2. low levels of fibrinogen & other clotting factors 6. Instruct the parents on how to control bleeding 3. prolonged prothrombin & partial thromboplastin (direct/indirect pressure) times & abnormal erythrocyte 7. DDVAP (Desmopressin) – promotes the release morphologic characteristics of Factor VIII in hemophilia A 8. Use soft toothbrush and point out need for C. NURSING MANAGEMENT regular dental checkups 1. Monitor for signs of bleeding of all hema tests : 9. Refer to National Hemophilia Association blood, urine, stool, git 10. Emphasize avoidance of Aspirin 2. Administer IV fluid replacement as ordered 11. Provide diet information as excess weight places 3. Administer oxygen inhalation as ordered further stress on joints 4. Administer medications as ordered  Vitamin K R - Rest  Heparin I - Immobilize  Pitressin (Vasopressin) via heplock C - Cold Compress 5. Provide Heplock, avoid IM, SQ and any E - Elevate venipunctures 6. Institute NGT decompression  Iced saline solution VIII. DISSIMENATED INTRAVASCULAR  Cold saline solution COAGULATION  Lavage: 500-1000cc of water; monitor NGT  DIC is a disorder of diffuse activation of the clotting output cascade that results in depletion of clotting factors in 7. Prevent complications the blood.  Hypovolemic shock (first sign: cold clammy  occurs when the blood clotting mechanisms are skin) (+) Anuria activated all over the body instead of being localized to an area of injury. IX. IDIOPATHIC THROMBOCYTOPENIA PURPURA  grave coagulopathy resulting from overstimulation of clotting & anticlotting processess in response to  Increased destruction of platelets with resultant disease & injury platelet count of less that 100,000/mm3 characterized  Small blood clots form throughout the body, and by petechiae and ecchymoses of the skin. eventually the blood clotting factors are used up and  Exact cause unknown; may be autoimmune. not available to form clots at sites of tissue injury.  Spleen is the site for destruction of platelets  Clot - dissolving mechanisms are also increased  often triggered by URTI or Childhood communicable stimulated by many factors including infection in the disease – Measles & chickenpox blood & severe tissue injury – burns and head injury, reactions to blood transfusions, carcinomas and A. ASSESSMENT: obstetrical complications such as retained placenta 1. Petechiae after delivery. 2. Ecchymosis 3. Blood in any body secretions, bleeding form mucous membranes, nosebleeds. 4. Decreased platelet count 5. Anemia 6. easy bruising 7. blood in stool or urine 8. CBC reveals platelet count below 20,000/mm3 9. Bone marrow aspiration done to rule out leukemia B. MEDICAL MANAGEMENT: Drug therapy: 1. Prednisone – decreases anti-platelet antibodies (monitor for infection) 2. IVIG (Intravenous Immune Globulin) – helps to effectively increase platelet count 3. Anti-D Antibody – one dose treatment  Given to pt’s 1 year but less than 19 years old  Normal WBC and hemoglobin  no active bleeding present  no concurrent infection  Diphenhydramine and hydrocortisine are made ready for possible allergic reactions to the medication Platelet transfusion Splenectomy C. NURSING MANAGEMENT 1. Prevent, control and minimize bleeding. A. ASSESSMENT 2. Prevent bruising 1. purpura on lower extremities & abdomen 3. Provide support to client and be sensitive to 2. hemorrhagic bullae, acral cyanosis, focal change in body image. gangrene in skin 4. Protect from infection. 5. Administer analgesics (acetaminophen) as ordered; avoid aspirin. 6. administer meds orally, rectally, or I.V. rather than I.M. 9 MS Abejo Medical and Surgical Nursing Hematology Lecture Notes Prepared by: Mark Fredderick R. Abejo RN,, MAN 2. Allergic reactions BLOOD TRANSFUSIONS  Signs and symptoms  Fever, chills, dyspnea, Laryngospasm, I. OBJECTIVES bronchospasm, Bronchial wheezing, A. To replace circulating blood volume Urticaria, pruritus, skin rashes B. Increase oxygen carrying capacity of the blood  Nursing Management C. Combat infections if decreased WBCs  Stop BT, Notify MD, Flush with PNSS D. Prevent bleeding if decreased PLT  Diphenhydramine administration as ordered II. NURSING MANAGEMENT/PRINCIPLES  If (+) to hypotension, it indicates A. Proper refrigeration anaphylactic shock B. Proper blood typing and cross-matching  Return blood unit to blood bank for re- examination  Obtain urine and blood sample of client for re-examination  Monitor VS and IO 3. Pyrogenic reactions  Signs and symptoms  Fever and chills  Headache  Dyspnea 1. Type O – universal donor  Tachycardia and palpitations 2. Type AB – universal receipient  Diaphoresis 3. 85% of general population is Rh (+)  Nursing Management  Blood expiration:  Stop BT  Platelets : 5 days  Notify MD  RBC: 5-7 days, 250 cc  Flush with PNSS C. Aseptically assemble all materials needed for BT  Administer antipyretics and antibiotics 1. Filter set (BT set) as ordered 2. PNSS to prevent hemolysis  Provide hypothermic blanket 3. 18-19 gauge large bore needle to prevent  Return blood unit to blood bank for re- hemolysis examination D. Instruct another RN to re-check the following:  Obtain urine and blood sample of client 1. Name of patient for re-examination 2. Bt and ct  Monitor VS and IO 3. Expiration date 4. Circulatory overload 4. Serial number  Signs and symptoms E. Check blood unit for presence of bubbles, cloudiness,  Dyspnea sediments and dark color as it may indicate  Rales/crackles contamination  Orthopnea F. NEVER WARM BLOOD PRODUCTS!  ROOM  Nursing Management TEMPERATURE ONLY  Stop BT 1. Warming only done if you have dewarming devise  Notify MD 2. Warming only done during emergency situations  if there is massive blood loss  massive transfusion  Administer loop diuretics as ordered G. Transfusion should be completed in 4 HOURS  NO FLUSHING! because blood exposed to room temperature more than  Monitor VS and IO 2 hours causes blood deterioration 5. Air embolism H. Avoid mixing or administering drug at BT line to 6. Thrombocytopenia prevent hemolysis 7. Citrate intoxication I. Regulate at KVO (10-15 gtts/min) at 100 cc/hour to 8. Hyperkalemia  arrhythmia prevent circulatory overload J. MONITOR VS BEFORE, DURING, & AFTER TRANSFUSION ESPECIALLY EVERY 15 MINUTES FOR THE FIRST HOUR. Majority of BT reactions occurs at these times K. SIGNS OF BT REACTION (HAPCATCH) 1. Hemolytic reactions  life threatening. PRIORITY  Signs and symptoms  Dizziness, Headache, Dyspnea, Hypotension, Flushed skin, Lumbar, flank and sternal pain, diarrhea or constipation, Portwine urine (red urine)  Nursing Management  Stop BT, Notify MD, Flush with PNSS  Administer Isotonic Solution to counteract shock and prevent acute tubular necrosis  Return blood unit to blood bank for re- examination  Obtain urine and blood sample of client for re-examination and send to lab  Monitor VS and IO 10 MS Abejo