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Phillip Podret

Hematology Chapter 30 (665-683), Chapter 31 (684-737)

I. Structures and Functions of Hematologic System a. Hematology study of blood and blood-forming tissues (bone marrow, blood, spleen, lymph system) i. knowledge of hematology useful in clinical settings to evaluate pts ability to transport oxygen and carbon dioxide, coagulate blood, and combat infections. b. Bone Marrow blood cell production (hematopoiesis) happens in red bone marrow i. Is stimulated by erythropoietin that cause differentiation of stem cells into one of the committed hematopoietic cells c. Blood (2 major components: plasma and blood cells i. A connective tissue for transportation, regulation and protection ii. Plasma: ~55% of blood; primarily water but also proteins, electrolytes, gases, nutrients, and waste; 1. Serum = plasma minus its clotting factor. 2. Plasma proteins = albumin, globulin, and clotting factors (which are mostly fibrinogen) iii. Blood Cells: 3 types of blood cells (erythrocytes (RBCs), leukocytes (WBCs), thrombocytes (platelets)) 1. Erythropoiesis is stimulated by hypoxia and controlled by erythropoietin (a growth factor made and released by kidney) a. Influenced by availability of nutrients b. Endocrine hormones influence erythrocyte (RBC) production i. Thyroxine, corticosteroids, testosterone 1. E.g. hypothyroidism can cause microcytic anemia. 2. Reticulocyte several distinct cell types evolve during erythrocyte maturation; this is an immature erythrocyte. a. *Retic count measures rate at which new RBCs appear in circulation. b. Retics can develop into mature RBCs within 48 hours of release into circulation 3. Hemolysis destruction of RBCs by monocytes and macrophages removes abnormal, defective, damaged, and old RBCs from circulation. a. Normally happens in: i. Bone marrow ii. Liver iii. Spleen

b. Hemolysis increased bilirubin to be processed by body. i. When hemolysis occurs via normal mechanisms, the liver is able to conjugate and excrete all bilirubin that is released. 1. Conjugate = be

combined with or joined to reversibly : bilirubin is then conjugated by liver enzymes and excreted in the bile.
2. Normal life span of an erythrocyte = 120 days c. Leukocytes (WBCs): i. 3 types of Granulocytes and function: 1. Neutrophil = 50-70% of all WBCs; phagocytosis is main function (especially during early/acute phase of inflammation). a. Mature = segmented b. Immature = band 2. Eosinophil = 2-4% of all WBCs; phagocytosis (NOT as effective as neutrophil), allergic response, protection from parasitic infections a. One of primary functions to engulf antigen-antibody complexes formed during allergic response 3. Basophil = <2% WBCs; inflammatory response and allergic response; release of bradykinin, heparin, histamine, serotonin; limited phagocytosis a. these cells have cytoplasmic granules that contain heparin, serotonin, and histamine. b. If a basophil is stimulated by an antigen or by tissue injury it will respond by releasing substances within the granules ii. 2 Types of Agranulocytes and function:

1. Lymphocyte - ~20-40% WBC; form basis for cellular and humoral immune response; TWO TYPES OF LYMPHOCYTES: a. Humoral = relating to

the body fluids, esp. with regard to immune responses involving antibodies in body fluids as distinct from cells

b. B cells c. T cells these cells migrate to the thymus gland for further differentiation into tcells (originate in bone marrow). 2. Monocyte 4-8% of WBCs; phagocytosis and cellular immune response a. *potent phagocytic cells b. can ingest small or large masses of matter such as bacgeria, dead cells, tissue debris, and old or defective RBCs. c. Are the 2nd type of WBCs to arrive at scene of an injury. d. Are only present in blood for a short time before they migrate into tissues and become macrophages. d. Thrombocytes (platelets) i. Primary fx to initiate the clotting process by an initial platelet plug in the early phases of the clotting process. ii. At site of capillary damage, platelet activation is initiated. iii. *Platelets Also fx in process of clot shrinkage and retraction iv. Thrombopoietin released from liver and

kidneys and regulates platelet production; growth factor acting on bone marrow v. platelets have life span of only 5-9 days d. Spleen i. 4 Major Fxs: 1. hematopoietic spleens ability to produce RBCs during fetal development. 2. Filtration removes old and defective RBCs from circulation by the mononuclear phagocyte system. a. Also involves reuse of iron; breaks down hemoglobin released by hemolysis and returns iron to bone marrow for reuse. 3. Immunologic spleen has a rich supply of stored lymphocytes, monocytes, and immunoglobulins. 4. Storage storage site for RBCs and platelets. a. ~30% platelet mass stored in spleen e. Gerontologic Considerations effects of aging on the hematologic system i. Decreased Hb (hemoglobin) levels may cause hypoxia ii. Decreased iron intake** iii. Transfuse to these pts sooner then healthy adults (small blood loss significant to geri pt) Assessment of hematologic system a. Subjective Data i. . look at class notes Diagnostic Studies of the hematologic system a. Complete blood count i. Pancytopenia when entire CBC is suppressed (RBCs, WBCs, and platelets) 1. care is directed toward anemia, infection, and hemorrhage b. Red blood cells i. Hemoglobin (Hb) value normal = 13-16 1. Reduced due to: anemia, hemorrhage, state of hemodilution (fluid volume is excessive) 2. Increased due to: polycythemia, state of hemoconcentration (develop from volume depletiondehydration) ii. Hematocrit (Hct) = centrifuge and RBCs settle to bottom; the crit value represents the percentage of RBCs compared with the total blood volume. 1. Variations in Hct are for same reason as conditions for Hb c. White Blood Cells i. WBC count over 11,000 is associated with infection, inflammation, tissue injury or death, and malignancies (e.g. leukemia, lymphoma). ii. A WBC < 4000 = leucopenia; associated with bone marrow depression, severe or chronic illness, and/or some types of leukemia






Neutropenia when bone marrow does not produce enough neutrophils; when absolute neutrophil count (ANC) < 1000 1. results from bone marrow depression (such as leukemia) and is associated with high risk of infection* Platelets normal = 150,000 400,000 i. Thrombocytopenia platelet count < 100,000; bleeding may occur 1. spontaneous hemorrhage possible once platelet count <20,000 Erythrocyte Sedimentation Rate (ESR OR sed rate) i. Measures settling of RBCs and used as a nonspecific measure of many diseases (especially inflammatory conditions) 1. Increased ESR common during acute and chronic inflammatory reactions when cell destruction is increased ii. **C-reactive protein (CRP) is something specifically measured in this Iron Metabolism (serum iron, total iron-binding capacity (TIBC), serum ferritin, and transferrin saturation) i. Serum iron measurement of amount of protein-bound iron circulating in serum ii. TIBC provides a measurement of all proteins available to bind or transport iron between the tissues and bone marrow. 1. Iron bound to transferrin is readily available for the body to use 2. Better indicator of availability of iron for erythropoiesis than serum iron iii. Ferritin = major iron storage protein; normally present in blood in concentrations directly related to iron storage iv. transferrin = largest of proteins that bind to iron; increased in majority of people with iron deficiency anemia v. transferrin saturation % = decerased in iron deficiency anemia and increased in hemolytic and megaloblastic anemia. vi. Bilirubin = measurement of degree of RBC hemolysis or livers inability to excrete normal quantities of bilirubin; increase in indirect bilirubin with hemolytic problems. Radiologic Studies i. Main radiologic study for hematology system = computed tomography (CT) or magnetic resonance imagine (MRI) for evaluating the spleen, liver, and lymph nodes. ii. *CT scan = #1 needed action for blood/bleeding problem 1. Tomography = technique for displaying a


representation of a cross section through a human body or other solid object using X-rays or ultrasound.


iii. PET (positron emission tomography) scanning = invaluable diagnostic tool to detect active malignancy because of its ability to highlight areas with increased metabolism. h. Biopsies (preferred site = posterior iliac crest) i. Bone Marrow to look at bone marrow failure 1. Allows full evaluation of hematopoiesis 2. Ability to obtain specimens for cytopathology and chromosomal abnormalities ii. Lymph node biopsy if concerned with cancer source Anemia a. Definition and classification i. Anemia = a decreased number of erythrocytes (RBCs), the quantity of hemoglobin, and/or the volume of packed RBCs (hematocrit). 1. Clinical manifestations of anemia are caused by the bodys response to tissue hypoxemia. ii. Caused by blood loss, impaired production of erythrocytes, or increased destruction of erythrocytes iii. TABLE 31-1 (PAGE 685) for etiologic classifications of anemia: 1. Decreased Erythrocyte Production a. May be caused by kidney failure; bone marrow disorder 2. Decreased Hemoglobin synthesis a. Iron Deficiency (most common anemia) b. Thalassemias (decreased globin synthesis) c. Sideroblastic anemia (decreased porphyrin) 3. Defective DNA synthesis a. Cobalamin (vit B12) deficiency b. Folic acid deficiency 4. Decreased number of Erythrocyte Precursors a. Aplastic Anemia b. Anemia of myeloproliferartive diseases (e.g. leukemia) and myelodysplasia c. Chronic diseases or disorders 5. Chemotherapy 6. Acute Blood Loss a. Trauma b. Blood Vessel rupture 7. Chronic Blood Loss a. Gastritis b. Menstrual flow c. Hemorrhoids 8. Increased Erythrocyte Destruction [Hemolytic anemia]. a. Intrinsic: i. Abnormal Hemoglobin (sickle cell anemia) ii. Enzyme deficiency (G6PD)

iii. Membrane abnormalities b. Extrinsic: i. Physical trauma (prosthetic heart valves, extracorporeal circulation) ii. Antibodies (isoimmune and autoimmune) iii. Infectious agents, medications, and toxins (malaria) iv. Since RBCs transport oxygen, erythrocyte disorders can lead to tissue hypoxia (this accounts for many s/s of anemia) v. Integumentary changes: 1. Pallor, jaundice, pruritus. vi. Cardiopulmonary manifestations of severe anemia: 1. Cardiac output maintained by increasing heart rate & stroke volume 2. Low viscosity of blood contributes to development of systolic murmurs and bruits. b. Iron-deficiency anemia i. Most common anemia ii. Susceptible = very young, poor diets, reproductive women iii. Causes: inadequate dietary intake, malabsorption, blood loss, or hemolysis 1. Surgery removing duodenum 2. *assess for GI bleed: black tarry stools? iv. Clinical manifestations: 1. Early pt may be free of symptoms 2. TABLE 31-3 = mild/moderate/severe anemic clinical manifestations 3. Pallor is most common finding 4. Glossitis inflammation of the tongue; 2nd most common finding [smooth red tongue; red, swollen, burning 5. chelitis inflammation of the lips v. Tx: 1. Main goal is to Treat underlying disease that is causing reduced intake (e.g. malnutrition, alcoholism) or absorption of iron. 2. replace iron 3. pt teaching of which foods are good sources of iron 4. Ferrous sulfate =iron is best absorbed as this 5. Vitamin C (ascorbic acid) enhances iron absorption 6. Parenteral administration (IV or IM) a. Use z-track method c. Vitamin B12 (Cobalamin) deficiency i. Megaloblastic anemia ii. Intrinsic factor (IF) secreted by parietal cells of gastric mucosa and IF is required for cobalamin absorption 1. Cobalamin normally absorbed in distal ileum

Pernicious Anemia most common cause of cobalamin deficiency; disease in which the gastric mucosa is not secreting IF because of antibodies being directed against the gastric parietal cells and/or IF itself. 1. Other causes: gastrectomy, gastritis, nutritional deficiency, chronic alcoholism, hereditary enzymatic defects of cobalamin utilization, long-term H2 blocker use, iv. Care: 1. Parenteral or intranasal administration of cobalamin (without pt will die in 1-3 years). v. Signs and Symptoms: 1. *nerve damage that causes tingling and numbness in the hands and feet (neuropathy) 2. feeling tired and weak 3. bright red and/or smooth shiny tongue 4. symptoms usually develop slowly over time 5. impaired thought processes (confusion to dementia) d. Folic Acid Deficiency (usually hear this for women trying to get pregnant) i. Causes: poor nutrition; malabsorption; drugs that impede absorption (*H2-blockers block a lot of things); alcohol abuse with anorexia; hemodialysis. 1. This also causes megaloblastic anemia (RBCs are large [macrocytic] and are easily destroyed because they have fragile cell membranes) ii. Folic acid is required for DNA synthesis leading to RBC formation and maturation iii. Signs and Symptoms: 1. Same as pernicious anemia (*LOOK IN PTS MOUTH DURING ASSESSMENT) 2. Lack of neurologic involvement differentiates folic acid deficiency from cobalamin deficiency. iv. Tx: 1. Replacement therapy (folic acid 1 mg/day PO) 2. Dietary management: foods with folic acid in them like green leafy vegetables, fruits, dried beans, peas and nuts. Enriched breads, cereals and other grain products also contain folic acid. v. Nursing management for BOTH Megaloblastic anemias: 1. NCP 31-1 p. 688 2. Nurse should ensure that injuries are not sustained because of diminished sensations to heat and pain resulting from neurologic impairment 3. Pt must be protected from falling, burns, and trauma 4. If heat therapy required, the pts skin must be evaluated at frequent intervals to detect redness. e. Aplastic Anemia


i. Disease in which the pt has peripheral blood Pancytopenia and hypocellular bone marrow ii. Clinical manifestations: 1. LOOK AT CLASS NOTES iii. Nursing and collaborative management of aplastic anemia 1. Nursing actions are directed at preventing complications fro infection and hemorrhage. a. NCP 31-1 (anemia) b. NCP 31-2 (thrombocytopenia) c. NCP 31-3 (neutropenia) 2. Prognosis of severe untreated aplastic anemia is poor 3. Hematopoietic stem cell transplant [HSCT]; immunosuppressive therapy with antithymocyte globulin [ATG] and cyclosporine or high-dose cyclophosphamide a. ATG = horse serum that contains polyclonal antibodies against human T cells. f. Acute Blood Loss (quicker onset of symptoms vs. chronic) i. *look at pts signs and symptoms for Acute blood loss 1st (not lab values) 1. if losing too much blood you dont have time to look at the #s 2. may have delayed changes in Hb, Hct** (compensatory volume change made by body after gradual acute blood loss) ii. Clinical manifestations: 1. 10% volume loss = none 2. 20% volume loss = no detectable signs or symptoms at rest; tachycardia with exercise and slight postural hypotension 3. 30% volume loss = normal supine blood pressure and pulse at rest, postural hypotension and tachycardia with exercise 4. 40% volume loss = blood pressure, central venous pressure, and cardiac output below normal at rest; rapid, thready pulse and cold, clammy skin. 5. 50% volume loss = shock and potential death. iii. Collaborative Care 1. Initial concerns: a. FIRST replace blood volume to prevent shock b. Next identifying source of hemorrhage and stopping the blood loss 2. Once volume replacement is established, attention directed to correcting the RBC loss a. 2-5 days for body to make more RBCs in response to increased erythropoietin 3. Pt may also need supplemental iron because availability of iron affects the marrow production or RBCs iv. Nursing Management

1. Postoperative pt must monitor blood loss from drainage tubes and dressings 2. Administer blood products 3. Once source of hemorrhage identified, blood loss is controlled, and fluid and blood volumes are replaced anemia should correct itself if acute blood loss g. Sickle Cell Disease (Intrinsic hemolytic anemia) i. Defects in RBCs caused by abnormal hemoglobin; hereditary ii. Hemoglobin S (Hb S) causes RBC to stiffen and elongate in response to low oxygen levels iii. *must learn/know pts trigger for sickling crisis (e.g. increased altitude/stress, surgery, dehydration) iv. Sickle cell anemia most severe of Sickle cell disease syndromes 1. Occurs when a person is homozygous for hemoglobin S (Hb SS); the person has inherited Hb S from both parents v. Sickle cell trait 1. Autosomal recessive disorder (both parents have trait for child to get it) 2. 25% chance each pregnancy that their child will have sickle cell anemia vi. Sickling Episodes 1. Can happen anywhere (*especially the spleen*) a. Nonfunctioning spleen increased risk for infection 2. Sickle cell lives 10 days (vs. normal RBC = 120 days) 3. Hypoxia or deoxygenation of RBCs can be caused by viral or bacterial infection, high altitude, emotional or physical stress, surgery, and blood loss. a. *infection is most common precipitating factor vii. Nursing and collaborative care 1. Teach pt to avoid high altitudes, maintain adequate fluid intake, treat infections promptly (AVOIDANCE OF TRIGGERS!!!) 2. Immunizations should be administered 3. Hospitalization a. Oxygen to tx hypoxia and control sickling b. Rest instituted to reduce metabolic requirements/demands c. Fluids and electrolytes are administered to reduce blood viscosity and maintain renal function. d. Transfusion therapy indicated when an aplastic crisis occurs i. May require chelation therapy to reduce transfusion-produced iron overload (*PROTECTS LIVER from failure due to high increases in serum iron level)

4. PCA pump of morphine/hydromorphone are analgesic drugs of choice for acute pain from sickling crisis. a. Contraindicated: Demerol can cause seizures. 5. Hydroxyurea only antisickling agent to be clinically beneficial; increases Hb F causes a reduction in hemolysis, an increase in hemoglobin concentration, and a decrease in sickled cells. 6. Hematopoietic stem cell transplantation (HSCT/bone marrow transplant) is only available tx that can cure some pts with SCD h. Immune Thrombocytopenic Purpura (ITP) i. This is a syndrome of abnormal destruction of circulating platelets. 1. An autoimmune disease ii. In ITP, platelets are coated with antibodies. Although these platelets function normally, when they reach the spleen, the antibody-coated platelets are recognized as foreign and are destroyed by macrophages. iii. Platelets normally survive 8-10 days; in ITP survival is 1-3 days iv. Symptoms: 1. Usually pt is asymptomatic 2. Bleeding into skin or mucosa (nose, mouth) 3. Prolonged bleeding after injection 4. Petechiae (pin-point bleed on skin) 5. Purpura (patches bleed on skin) 6. Ecchymosis 7. Cerebral hemorrhage v. Tx: 1. Corticosteroids (prednisone) are used to initially treat ITP because of their ability to suppress the phagocytic response of splenic macrophages. a. This alters the spleens recognition of platelets and increases the life span of the platelets. b. Corticosteroids also depress antibody formation c. Corticosteroids also reduce capillary fragility and bleeding time d. If neurologic manifestations R/T intracranial bleeding, then administer high-dose methylprednisolone IV i. Can also be used if pt resistant to prednisone 2. Splenectomy indicated if pt does not respond to prednisone initially or requires unacceptably high doses to maintain an adequate platelet count. 3. High doses of IV immunoglobulin (IVIG) = suppressive affects) a. Work by competing with antiplatelet antibodies for macrophage receptors

i. Effectively raises the platelet count, but the beneficial effects may be temporary 4. Danazol used along with steroids in some patients; this increases CD4 T cells, thereby reducing the immune response. a. Rituxan, Cytoxan, Imuran, cyclosporine, CellCept. 5. Platelet transfusions used to increase platelet counts in lifethreatening hemorrhage a. Usual indication < 10,000 or < 5,000 (in some studies) or if there is bleeding before a procedure. b. ABO compatibility is NOT a necessary prerequisite for platelet transfusions (need a match if doing this multiple times though) 6. Discourage OTC meds containing aspirin; i. Thrombotic Thrombocytopenic Purpura i. Normally the cause is due to decreased plasma enzyme that breaks down the von Willebrand clotting factor into normal size 1. Without the enzyme, there is unusual platelet aggregation/clumping ii. Leads to bleeding under the skin and purple-colored spots called purpura. iii. Red blood cells break apart and be destroyed prematurely iv. Low platelets, low RBC count, abnormal kidney function, and problems with nervous system v. May be related to cancer, chemotherapy, HIV infection, hormone replacement therapy and estrogens, and a number of commonly used medications (ticlopidine, copidogrel, and cyclosporine A) vi. Symptoms: 1. Fever; weakness; easy fatigue; pallor; SoB on exertion; HR over 100 beats/min; purpura = problem with platelets; bleeding into skin or mucous membranes; headache; confusion; speech changes; changes in consciousness; jaundice; platelet count low; CBC shows anemia; bilirubin is high; blood smear shows broken RBCs (schistocytes) and fragmented blood cells; urinalysis shows protein and very tiny blood particles; creatinine level is high; mucous membrane biopsy shows platelet clots in small blood vessels vii. Tx: 1. Plasmapheresis (plasma exchange) may be needed to aggressively reverse the process a. Reverses platelet consumption by supplying the appropriate vWF and enzyme and removing the large vWF molecules binding with platelets. i. remove unwanted substances from blood and to replace the missing enzyme that

normally breaks down proteins (protease). b. This is done daily until blood tests show improvements 2. No response to plasmapheresis a. Splenectomy b. Immune suppression (corticosteroids) 3. If unsuccessful, complications are: a. Kidney failure b. Hemorrhage c. Stroke d. DEATH. viii. NURSING management for thrombocytopenia (both conditions above) 1. Health Promotion: discrouge excessive use of OTC meds (aspirin reduces platelet adhesiveness contributes to bleed); prolonged nose bleed [epistaxis] or petechiae need complete medical evaluation; *nurse must observe for early signs of thrombocytopenia in the pt receiving cancer chemotherapy drugs. 2. Acute Intervention: a. Goal during acute episodes of thrombocytopenia is to prevent or control hemorrhage (NCP 31-2) b. 50 ml of blood will soak a sanitary napkin completely