Care of Clients with Hematologic Disorders PDF

8/15/24 Care of Clients with Hematologic Disorders Professor Beryl Ben C. Mergal, RN, PhD 1...

8/15/24 Care of Clients with Hematologic Disorders Professor Beryl Ben C. Mergal, RN, PhD 1 The Hematologic System The hematologic system consists of the blood and the sites where blood is produced, including the bone marrow and the reticuloendothelial system (RES). Text 2 1  8/15/24 Overview of the Anatomy of the Hematologic System 3 The Hematologic System Blood Blood is a specialized organ that differs from other organs in that it exists in a fluid state. blood is also an organ except it is in a FLUID state 4 2  8/15/24 The Hematologic System Blood A liquid connective tissue composed of: 1. Plasma – a clear, light yellow fluid that forms the extracellular matrix of the tissue. 2. Formed Elements – are cells and cell fragments (RBC’s, WBC’s and Platelets) 5 Common Attributes of the Blood CHARACTERISTIC TYPICAL VALUES FOR ADULTS Mean fraction of body weight 8% Volume in adult body Female: 4-5L Male: 5-6 L Mean Temperature 38°C (100.4°F) pH if more than 7.45, acidic ang blood mo vice versa 7.35-7.45 Why do male have more blood compare to females? - because female have monthly menstruation contributing to a lesser blood supply 6 3  8/15/24 Functions of the Blood Blood transport water, oxygen, nutrients, hormones, enzymes, and medications to the cells. Blood transports carbon dioxide and other waste products away from the cells. About 4-5L of blood in the body help regulate fluid volume and electrolyte distribution. The blood regulates the pH and acid-base balance by its buffering ability. Blood assists in regulating body temperature. Blood provides clotting factors for hemostasis. balance 7 Functions of the Blood HOMEOSTASIS TRANSPORT REGULATION PROTECTION transporting hormones, regulation of acid, protection to the individual medications, etc electrolyte, body temp. who has blood loss 8 4  8/15/24 Components and the General Properties of the Blood 92% of plasma is water remember the normal values 9 Plasma Composition A mixture of water, proteins, nutrients, nitrogenous wastes, hormones and gases. A dilute solution which by weight is approximately 92% H2O, 7% protein, and 1% small dissolved dilutes. Serum is the fluid that remains when blood clots and the solids are removed. Albumin is the smallest and most abundant plasma protein. It transports various solutes and buffers the pH of the plasma. 10 5  8/15/24 Plasma Composition 11 Major Types of Plasma Proteins PLASMA MAJOR EXAMPLES and FUNCTIONS PROTEIN SOURCE Albumin Liver Main component of plasma oncotic pressure; binding of various substances a-Globulins & b- Liver Examples include hormone-binding Globulins proteins and the iron carrier protein transferrin Coagulation Liver Examples include plasminogen, proteins prothrombin, antithrombin III, and fibrinogen Immunoglobulins Lymphoid Host defense reactions Tissue Complement Liver Host defense reactions Proteins 12 6  8/15/24 Normal Plasma Composition SUBSTANCE NORMAL VALUE NORMAL RANGE Sodium 140 mM 136-146 mM Potassium 4.5 mM 3.5-5.5 mM Chloride 100 mM 96-107 mM Bicarbonate 24 mM 22-28 mM Calcium 2.5 mM 2.2-2.8 mM pH (arterial) 7.40 7.35-7.45 PCO2 (arterial) 40 mmHg 38-42 mmHg PO2 (arterial) 90 mmHg 80-100 mmHg Glucose (fasting) 80 mg/dL 70-99 mg/dL Urea (BUN) 12 mg/dL 9-18 mg/dL Protein 7 g/dL 6-8 g d/L 13 Albumin and It’s Relationship to the Physical Properties of Blood Albumin influences greatly on the following properties of blood: 1. Viscosity – thickness or stickiness of a fluid that results from cohesion of its particles. 2. Osmolarity – concentration of particles that cannot pass through the walls of blood vessels. When Albumin is lesser = Blood becomes thicker (too viscous) thus resulting for the circulation of blood to slow down through the network of vessels we need enough space for the ventricles for blood to be acommodated when cardiac muscles are hypertropied the ventricles become narrow (?) pag less than 40% ang pag pupump ng blood sa heart it results to heart failure 14 increase osmolarity of blood = increase of your antidiuretic hormone (ADH) decrease osmolarity of blood = decrease production of your ADH 7  8/15/24 Formed (Cellular) Elements of Blood 1. Red Blood Cells (erythrocytes) – essential for the transport of O2 and CO2 in blood. RBC must maintain a biconcave shape to be more flexible or reach the furthest blood vessels (e.g. capillaries) ; they are non-nucleic to enter the capillaries 2. Platelets (thrombocytes) – cellular fragments that play a key role in hemostasis by adhering to sites of damage and through participation in the blood clotting system. they are non-nucleic 3. White Blood Cells (leukocytes) - fully functional nuceated cells in the blood. WBCs play a defensive role in destroying infecting organisms and in the removal of damaged tissue. (approximately 5x109 cells/L of blood) WBC are nucleated, fully functional cells because they all have cell membrane, cytoplasm, nucleus, etc. 15 Formed Elements of the Blood 16 8  8/15/24 Formed Elements of the Blood 17 Hematopoiesis It is the formation of blood cellular components which primarily occurs in the bone marrow. Blood cells are derived from stem cells in the bone marrow and are produced after several stages of cell division and differentiation. 18 9  8/15/24 Four Groups of Blood Forming Cells during Hematopoiesis Hematopoiesis is dependent on these 4 blood forming cells 1. Pluripotent hematopoetic stem cells – capable of forming any type of blood cell. 2. Multipotential progenitor cells – can form a specific but wide range of blood cells. There are 2 types a. Lymphoid Cells b. GEMM (granulocyte erythroid monocyte megakaryocyte) 3. Committed Progenitor Cells – are capable of self-renewal but are able to form only one or two cell type. 4. Maturing Cells – do not divide again and are undergoing structural differentiation to form a specific type of blood cell. 19 Hematopoiesis pluripotent stem cell can form any type (myeloid or lymphoid stem cell) they care committed to a specific type to transform e.g. proerythroblast is committed to transform into reticulocyte into RBC Reticulocyte & Megakaryocyte are an example of a Maturing Cells **when there is enough number of retics (reticulocyte), the body is capable of producing RBC, this is what we call maturing cells 20 10  8/15/24 Red Blood Cells 21 Red Blood Cells Two Main Functions 1. To pick up oxygen from the lungs and transport it to body tissues. 2. To pick up carbon dioxide from the tissues and unload it in the lungs. 22 11  8/15/24 Red Blood Cells Normal lifespan in the circulation is approximately 100-120 days. Aging RBCs become progressively more fragile and are ultimately removed from the circulation by scavenging macrophages (spleen). removal of this aging RBC occurs in the SPLEEN this results to SPLEENOMEGALY, rapid removal of RBC in the spleen causing it to enlarge 23 Red Blood Indices INDEX UNITS / CALCULATION NORMAL RANGE function of hemoglobin Hemoglobin g/dL Adult man: 14-18 g/dL is carrier of oxygen Concentration (Hb) Adult woman: 11-15 g/dL Hematocrit (Hct) % Adult man: 40-54% Adult woman: 34-46% Red Blood Cell (RBC) 10 /L 12 Adult man: 4.5-6.5 Adult woman: 3.9-5.6 it has something to do with Mean Cell Volume (MCV) (% Hct ÷ 100) / RBC (fL) 82-98 fL the average size of your cell the average amount of Mean Cell Hemoglobin (Hb x 10)/ RBC (pg) 27-33 pg hemoglobin contain in 1 single cell of a person’s (MCH) RBC the number of hemoglobin Mean Cell Hemoglobin (100 x MCH) / MCV 30-35 g/dL in a certain amount of blood Concentration (MCHC) (g/dL) volume 24 12  8/15/24 Complementary RBC Indices 1. Blood smear – to investigate variations in cell size and cell shape which indicate errors in RBC maturation. 2. Reticulocyte Count – to assess rate of RBC production. 25 Red Blood Cells Key Cytoplasmic Proteins 1. Hemoglobin – major protein present in red blood cells, and is responsible for the large O2- carrying capacity of blood. 26 13  8/15/24 Red Blood Cells Hemoglobin Hemoglobin (Hgb) is a protein molecule adapted to carry O2 and CO2 as well), and each RBC contains 280 million molecules of Hgb A Hgb molecule consists of 4 large globin proteins (2 alpha and 2 beta chains), each embedding an iron- containing heme center. 27 Red Blood Cells Key Cytoplasmic Proteins 2. Glycolytic Enzymes – needed In order for an RBC to be because RBCs have no since RBC is not a fully functional cell, meaning they GLYCOLYTIC ENZYMES mitochondria and must fully functional they need dont have mitochondria synthesize ATP via glycolysis 3. Carbonic Anhydrase – for CO2 carriage in blood 28 14  8/15/24 Red Blood Cell Formation Erythropoiesis Erythropoietin (EPO) is the primary regulator of erythropoiesis and is released from the kidney when O2 tension in the renal parenchyma is reduced. body senses when there is less oxygen concentration 29 Role of Erythropoietin in Regulation of Erythropoiesis STIMULUS for the release of Erythropoietin 30 15  8/15/24 Red Blood Cell Formation Erythropoiesis it is very important that your RBC MUST MATURE The maturation of RBCs in the bone marrow requires several factors; Iron (for the synthesis of hemoglobin) Folic Acid Vit B12 intrinsic factor of Vit B12 is found in the stomach lineage of your body these three are needed to help mature your RBC 31 Red Blood Cell Formation Erythropoiesis Erythropoiesis increases when states of hypoxia (deficiency) stimulates the kidneys to release the hormone erythropoietin (EPO). 32 16  8/15/24 Requirements for normal red cell production damaged bone marrow = hypoproliferation of RBC 1. Functional bone marrow – hypoproliferation of RBCs may result if the bone marrow is damaged. 2. Erythropoietin – secretion of EPO is needed to stimulate the bone marrow. 3. Adequate nutrient supply for hemoglobin synthesis such as iron deficiency. 33 General Features of the Process of Stem Cell Differentiation (RBCs) Red blood cells are bi-concave discs. Mature RBCs don't have a nucleus or any protein making machinery and are destined to die in about 120 days. Not really cells, but remnants of cells with a very specific purpose – to carry O2 to the tissues of the body. 34 17  8/15/24 Red Blood Cell Formation Reticulocytes The rate of erythropoiesis is measured by the number of immature RBCs (called reticulocytes or “retics”) in the peripheral circulation. A low retic count (2%) indicates a high rate of erythropoiesis. 35 Abnormalities of Erythropoiesis sobrang daming RBC = indicates a problem with the viscosity of the blood 1. Polycythemia - It is an abnormally high level of circulating red blood cells. Caused primarily by increased levels of EPO. Increase in RBC mass causes increases viscosity of blood and therefore increases risk for thrombosis. 2. Hemorrhagic Anemia caused by an actual decrease in the number of circulating RBCs lost because of hemorrhage or bleeding. 3. Aplastic Anemia characterized by abnormally low RBC counts and destruction of bone marrow. In Aplastic Anemia, STEM CELLS ARE DEGRADED Stem Cells are located in the bone marrow and they are the PRECURSOR of your blood 36 18  8/15/24 Abnormalities of Erythropoiesis 4. Deficiency anemia occurs when there is a reduction in RBC brought about by the reduced absorption of blood nutrients blood nutrients - Iron, Folic, Vit B12 37 Abnormalities of Erythropoiesis Types of Deficiency Anemia a. Pernicious Anemia results from a dietary deficiency of Vit B12. b. Folate Deficiency Anemia results from a dietary deficiency in Folic Acid. c. Iron Deficiency Anemia results from a deficiency in Iron. 38 19  8/15/24 Abnormalities of Erythropoiesis 5. Hemolytic Anemia is a structural defect in the Red Blood Cells caused by several factors. In Hemolytic Anemia even before 120 days, RBC is already dead 39 Abnormalities of Erythropoiesis Types of Hemolytic Anemia a. Hemolytic Anemia is condition characterized by a decrease in RBC life span caused by an increased rate of destruction of RBCs. b. Sickle Cell Anemia is genetic disease that results in the formation of limited amounts of abnormal type of Hemoglobin 40 20  8/15/24 Abnormalities of Erythropoiesis Sickle-cell disease (SCD) also called sickle-cell anemia, is an autosomal recessive disorder. A genetic defect in the primary DNA sequence leads to production of a faulty Hgb β chain, and RBCs that take on a rigid, sickle-shape. 41 Sickle Cell Anemia NORMAL ABNORMAL 42 21  8/15/24 Abnormalities of Erythropoiesis Types of Hemolytic Anemia c. Thalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen. The disorder results in excessive destruction of red blood cells, which leads to anemia. hemoglobin present in here is DEFECT (not able to deliver oxygen needed) 43 Common Symptoms of Anemia rapid heart rate = increase need of blood supply for oxygen schemic condition happens if there is an increase demand of oxygen thus resulting chest pain, angina, and heart attack (a.k.a [MI] myocardial infarction) yellowing coloring of your stool rapid breakdown of RBC results to spleen enlargement 44 22  8/15/24 Laboratory Results for Types of Anemia ANEMIA FOLATE Hb Hct Fe Content RBC SIZE Vit B12 CONTENT (VOLUME) Content Aplastic Low to Low to Normal to Anemia Normal Normal Normal High slightly High Normal Pernicious Anemia Normal Low Low High High Low Hemorrhagic Anemia Acute Blood Low to Low to Loss Normal Normal Normal Normal Slightly Low Normal Chronic Blood Loss Normal Low Low Low Low to Normal Normal IDA Normal Low Low Low Low Normal Hemolytic Normal to Anemia Normal Low Low High Normal to High Normal 45 Hemoglobin Breakdown The end product of hemoglobin breakdown in macrophages is bilirubin, which is conjugated in the liver and excreted in the bile. 46 23  8/15/24 Hemoglobin Breakdown Summary of Hemoglobin Breakdown stercobilin in poo is your yellowing component of your poo urubilin in urine is your yellowing component of your urine 47 Hemoglobin Breakdown Life Cycle of the Red Blood Circulation for about Cells 120 days 3 7 Amino Reused for acids protein synthesis Fe3+ Transferrin Globin 4 6 5 Fe3+ 2 Heme Ferritin Fe3+ Transferrin + Bilirubin Globin 9 + Biliverdin Bilirubin 11 Liver Vitamin B12 1 Red blood cell 10 + death and Erythopoietin phagocytosis Small intestine Kidney 8 Erythropoiesis in 13 Bilirubin red bone marrow 12 Urobilin Macrophage in Urobilinogen Bacteria Key: spleen, liver, or red bone marrow in blood Stercobilin Large 14 intestine in bile Urine Feces 48 24  8/15/24 White Blood Cells and the Glory of God 49 Lessons from the White Blood Cells 1. Praise this brilliant God. “I praise you, for I am fearfully and wonderfully made.Wonderful are your works; my soul knows it very well. “ (Psalms 139:14) 50 25  8/15/24 Lessons from the White Blood Cells 2. Fear this great God. “Let all the earth fear the Lord; let all the inhabitants of the world stand in awe of Him“ (Psalms 33:8) 51 Lessons from the White Blood Cells 3. Do not hope in yourself or this world. “Trust in the Lord with all your heart.“ (Proverbs 3:5) 52 26  8/15/24 Lessons from the White Blood Cells 4. Trust in Christ alone to rescue from all viruses. “He himself bore our sins in his body on the tree, that we might die to sin and live to righteousness. By his wounds you have been healed.” (1 Peter 2:24) 53 Lessons from the White Blood Cells 5. Heaven will be villain-less. “And I heard a loud voice from the throne saying, “Behold, the dwelling place1 of God is with man. He will gdwell with them, and they will be his people,2 and God himself will be with them as their God.3 4 hHe will wipe away every tear from their eyes, and ideath shall be no more, jneither shall there be mourning, nor crying, nor pain anymore, for the former things have passed away.” (1 Peter 2:24) 54 27  8/15/24 White Blood Cells 55 White Blood Cells FUNCTIONS OF THE WBC’s WBCs provide the first line of defense against microbial agents. Leukocytes are your precursor of your WBC Leukocytes migrate from the bone marrow cells out into the tissues, and are carried by the bloodstream to locations where they are needed. 56 28  8/15/24 White Blood Cells Leukopoiesis Colony-Stimulating Factors – is a cytokine responsible for the production of WBCs. similar to your Eryhthropoietin From a lymphoid progenitor cells in bone marrow it then develops into: Your WBC comes from your Lymphoid T lymphocytes (differentiates in the thymus gland) B lymphocytes (differentiates in the lymph nodes) 57 White Blood Cells for bacterial infection for parasitic infection increased when clients is exposed to allergen kasi they secretes histamine for making antibodies primary acting as a phagocytic cells 58 29  8/15/24 Leukocytes Granulocytes Agranulocytes Neutrophils Monocytes Eosinophils Lymphocytes Basophils 59 White Blood Cells Granulocytes 1. Neutrophils Most abundant WBCs They are aggressive antibacterial cells where they are quickly attracted to areas of infection or inflammation. Uses phagocytosis to digest bacteria. Elevated neutrophils may indicate an infection. 60 30  8/15/24 White Blood Cells Granulocytes 2. Eosinophils Represent only 2-4% of WBC total. Secretes chemicals that weaken or destroy large parasites such as hookworms or tapeworms, which are too large to be destroyed by WBC. Increased during parasitic infections, allergies, and diseases of the spleen and CNS and fluctuates between day and night during the course of the menstrual cycle. 61 White Blood Cells Granulocytes 3. Basophils Rarest of the WBCs comprising only 0.5-1% of WBCs. Secretes histamine, a vasodilator that widens blood vessels, speeds the flow of blood to injured tissue, and makes the blood vessels more permeable. Secretes heparin, an anticoagulant that inhibits blood clotting and thus promotes the mobility of other WBCs in the area. 62 31  8/15/24 White Blood Cells Granulocytes 2. Basophils Increased in diabetes, chickenpox and various other diseases. 63 White Blood Cells Agranulocytes 1. Lymphocytes Second to neutrophils in abundance at 25%- 33%. Increases in diverse infectious diseases and immune responses. 64 32  8/15/24 White Blood Cells Agranulocytes 2. Monocytes Largest WBCs (in size). About 3-8% of WBC count. Transforms into macrophages which destroys dead or dying host and foreign cells, microorganisms and other foreign material, consuming up to 25% of their own volume per hour. Increases in inflammation and viral infections. 65 Abnormalities in the White Blood Cells malignancy = there is proliferation of an unwanted cells Leukemia A general term used to describe a malignancy characterized by the bone marrow becoming infiltrated with malignant blast cells or immature WBCs immature WBC = there is only a stanting distribution of your WBC; they are dysfunctional = Leukemia 66 33  8/15/24 Abnormalities in the White Blood Cells Leukemia Acute Myeloid Leukemia (AML) – blast cells are of myeloid lineage. Acute Lymphocytic Leukemia (ALL) – blast cells are of lymphoid lineage. 67 Other Abnormalities of the Leukocytes Leukopenia low WBC count may be due to poisoning from lead, mercury, or arsenic or to radiation exposure. Caused by diseases such as AIDS, measles, mumps, chickenpox, influenza, and polio. Certain drugs can also deplete WBC such as glucocorticoids, anticancer drugs and immunosuppressant drugs. 68 34  8/15/24 Other Abnormalities of the Leukocytes Leukopenia low WBC count may be due to poisoning from lead, mercury, or arsenic or to radiation exposure. Caused by diseases such as AIDS, measles, mumps, chickenpox, influenza, and polio. Certain drugs can also deplete WBC such as glucocorticoids, anticancer drugs and immunosuppressant drugs. 69 Common Symptoms of Leukemia 70 35  8/15/24 Platelets 71 Platelets Platelets are small circulating fragments of bone marrow cells called megakaryotes and serve various roles in blood clotting. 2nd most abundant formed element. Smaller than RBCs. 72 36  8/15/24 Platelets Thrombopoiesis Thrombopoietin (TPO) is a cytokine that regulates platelet formation. TPO is secreted by the liver and kidneys. Platelets remove TPO from the circulation and so high levels of TPO result when there is low platelet count (thrombocytopenia). Increased TPO concentrations stimulate megakaryotes to produce more platelets and thereby restore a normal platelet count. 73 Platelets FUNCTIONS OF THE PLATELETS They secrete vasoconstrictors, chemicals that cause spasmodic constriction of broken vessels and thus help reduce blood loss. They stick together to form temporary platelet plugs to seal small breaks in injured blood vessels. They secrete clotting factors that promote blood clotting. 74 37  8/15/24 Platelets FUNCTIONS OF THE PLATELETS They initiate the formation of clot-dissolving enzyme that dissolves blood clots that have outlasted their usefulness. They secrete growth factors that stimulate mitosis in fibroblasts and smooth muscle and thus help maintain and repair blood vessels. 75 Role of Platelets in Blood Clotting 76 38  8/15/24 Physiologic Mechanisms of Hemostasis Step 1 Vascular Spasm The prompt constriction of a broken vessel. This narrows the opening and reduces blood loss. It is triggered in part by serotonin, a vasoconstrictor secreted by the platelets. 77 Physiologic Mechanisms of Hemostasis Step 2 Platelet Plug Formation A sticky mass of platelets that acts as a stopper to close small breaks in a vessel. (Primary Hemostasis) 78 39  8/15/24 Phases of Primary Hemostasis 1. Platelet Adhesion to the exposed subendothelial extracellular matrix which occurs at the site of injury. 79 Mediators of Platelet Adhesion 1. Integrin for binding to extracellular matrix proteins (collagen) 2. Von Willebrand factor (vWF) promotes platelet adhesions (with Factor VIII), forms crosslinks between platelets and with collagen 3. Thromboxane A2 and thrombin further promotes platelet adhesion and activation. 80 40  8/15/24 Von Willebrand’s Disease Is the most common inherited bleeding disorder and is characterized by either a deficiency or a functional defect in vWF. 81 Phases of Primary Hemostasis 2. Platelet Activation Several intracellular signaling pathways are activated when ligands bind to platelet receptors. 82 41  8/15/24 Phases of Primary Hemostasis 2. Platelet Activation Activated platelets then release many factors that promote hemostasis: a) ADP a potent activator for other platelets b) Serotonin and Thromboxane A2 assists hemostasis as vasoconstrictors. c) vWF augments platelet adhesion and aggregation d) Ca2+ and the clotting factors fibrinogen and factor V facilitate coagulation. e) Platelet-derived growth factor promotes wound healing and by stimulating growth and migration of fibroblasts and smooth muscle cells at the site of the injury. 83 Phases of Primary Hemostasis 3. Platelet Aggregation completes the formation of a platelet plug. The signaling molecules released during platelet activation amplify the platelet adhesion and activation responses and recruit more platelets to the site of injury. Prostacyclin and nitric oxide prevents the extension of a platelet plug from the injury site. 84 42  8/15/24 Physiologic Mechanisms of Hemostasis Step 3 Coagulation (Clotting) üOccurs in which a fibrin mesh forms together with platelets and other trapped blood cells. (Secondary Hemostasis) 85 Blood Coagulation Factors FACTOR NAME FUNCTION OR PATHWAY I Fibrinogen Converted to fibrin strands II Prothrombin Converted to thrombin III Thromboplastin Catalyzes conversion of thrombin IV Calcium ions Needed for all steps of coagulation V Proaccelerin Extrinsic/intrinsic pathways VII Serum prothrombin conversion accelerator Extrinsic Pathway VIII Antihemophilic factor Intrinsic Pathway IX Plasma prothrombin component Intrinsic Pathway X Stuart Factor Extrinsic/Intrinsic Pathway XI Plasma Prothrombin antecedent Intrinsic Pathway XII Hageman factor Intinsic Pathway XIII Fibrin stabilizing factor Cross-links fibrin strands to form insoluble clot 86 43  8/15/24 Blood Coagulation Cascade Ca+ Ca+ 87 Fibrinolysis It is a normal body process that keeps naturally occurring blood clots from growing and causing problems. 88 44  8/15/24 Fibrinolysis Tissue plasminogen activator Urokinase-type plasminogen activator PLASMIN a2-antiplasmin PLASMINOGEN PLASMIN 89 45