Hematological System PDF

Hematologic System Structures & Functions of Hematologic System Bone marrow (yellow & red) - red marrow is found in flat bones and is responsible for making blood cells. Blood is a connective tissue that transports, regulates, and protects (maintains homeostasis of coagulat...

Hematologic System Structures & Functions of Hematologic System Bone marrow (yellow & red) - red marrow is found in flat bones and is responsible for making blood cells. Blood is a connective tissue that transports, regulates, and protects (maintains homeostasis of coagulation) ○ Plasma (55%) - composed mainly of water, but also contains proteins, electrolytes, gasses, nutrients, and waste. ○ Blood cells (45%) Erythrocytes (RBCs) - O2 transportation Leukocytes (WBCs) - protects from infection Thrombocytes (Platelets) - promotes blood coagulation Anemia is a deficiency in: Number of erythrocytes (RBCs) Quantity or quality of hemoglobin (Hgb) Volume of packed RBCs (hematocrit) Causes Decreased RBC production Blood loss Increases RBC destruction Hereditary (intrinsic) Because RBCs transport O2, RBC disorders can lead to tissue hypoxia. Manifestations of anemia result from the body's response to hypoxia Normal CBC Hemoglobin (Hgb) ○ Female: 12-16 g/dL ○ Male: 14-18 g/dL WBC - 5000 - 10,000/uL Platelets - 150,000 - 400,000 Mild (10-12g/dL) Moderate (6-10g/dL) Severe ( < 6g/dL) Asymptomatic Fatigue Pallor Palpitations - bounding Dizziness Response to heavy exercise pulse headache/vertigo/impaired Palpitations Dyspnea thought process Dyspnea exhausted/lethargic Mild fatigue Severe palpitations, tachy Orthopnea, dyspnea at rest Sensitive to cold, anorexia  Morphology Etiology Normocytic, Normochromic Acute blood loss, hemolysis, chronic kidney Normal size and color disease, sickle cell anemia, pregnancy, cancer MCV 80-100fL, MCH 27-34 pg Microcytic, hypochromic Iron deficiency anemia, Vit B6 deficiency, Small size, pale color Thalassemia, lead poisoning MCV < 80 fL MCH < 27 pg Macrocytic (megaloblastic), Normochromic Cobalamin (Vit B12 deficiency), folic acid Large size, normal color deficiency, liver disease, effects of ETOH abuse MCV > 100 fl MCH > 34 pg Diagnostic Studies for Anemia CBC (Hgb, Hct, WBC, platelets) Reticulocyte count Red blood cell indices - MCV, MCH Serum ferritin - measures iron stores. First to drop. Serum iron - measures circulating iron, carried by transferring. Drops after serum ferritin. TIBC (transferrin & iron binding capacity) B12 Folate Schilling Test - to evaluate vit absorption (rarely done) Etiology and Management of Anemia Decreased RBC production - iron deficiency thalassemia Decreased number of RBC precursors - aplastic anemia Nutritional anemias - iron deficiency, macrocytic or megaloblastic anemias (B12 or folate deficiency) Blood loss - acute or chronic Hereditary (intrinsic) - sickle cell anemia Assessment ○ H&H Clinical problems - depends on H&H Planning - goal = normal activities Nursing Implementation Varies ○ Numerous causes of anemia ○ Patient-specific needs  ○ Acute interventions may include blood transfusions, drug therapy (eg. iron supplements), and O2 therapy. Patients with fatigue ○ Alternate rest and activity ○ Prioritize activities Accommodate energy levels Maximize O2 supply for vital functions ○ Aid to minimize risk of injury from falls ○ Monitor cardiorespiratory response ○ Evaluate nutrition needs Acute interventions ○ Blood transfusions ○ Drug therapy (iron supplements) ○ O2 therapy National Patient Safety - Eliminate Transfusion Error GOAL: eliminate transfusion errors related to patient misidentification. “PATIENT SAFETY” Two-person patient identification process includes: ○ Person who will give the blood ○ Another person who is qualified to identify the patient Match blood to the provider’s order. Match patients to blood. Nutritional Anemia Iron Deficiency Anemia Causes ○ Chronic blood loss (GI or GU systems), or hemolysis ○ Menstruation ○ Inadequate dietary intake ○ Inadequate absorption of iron from GI tract (small intestine is where iron is best absorbed) - think about if they’ve had GI surgery ○ GI surgeries, involving bypass of duodenum ○ Malabsorption syndromes Signs/Symptoms - depends on age and severity Few symptoms until HCT < 30 ○ Pallor, glossitis (inflammation of tongue), cheilitis (inflammation of lips), headache, paresthesia History & Physical ○ Menstrual history ○ Onset & duration of symptoms ○ Change in stool patterns ○ Color of stool ○ Dietary intake ○ Medication history  ○ Observe skin - pallor, jaundice, pruritus ○ Examine tongue - atrophic glossitis, burning sensation ○ Look at the corners of the mouth - cheilosis ○ Examine nails - koilonychias (spooning) ○ Palpate abdomen tenderness ○ Splenomegaly ○ Obtain stool for occult blood Diagnostics ○ Labs Low Hgb Decreased serum iron - measures circulating iron, carried by transferring. Drops after serum ferritin. High TIBC - ability of cells to bind to iron Low serum ferritin - measures iron stores. First to drop. Low MCV Low MCH **normally is inversely related to serum iron, eg. when serum iron is low, TIBC is high ** ○ Stool occult blood test ○ Endoscopy and colonoscopy ○ Bone marrow biopsy Nursing Management ○ Goal - treat underlying problems causing loss, reduced intake, or poor absorption of iron ○ Replace iron Nutrition therapy Oral iron supplements Transfusion of packed RBCs Treatment: Self-Care Plan ○ Oral iron therapy ○ Teach about foods high in iron - eggs, lean beef, red kidney beans, oats, spinach ○ Take iron between meals or one hour before - iron is best absorbed in acidic environment ○ *Taking with vitamin C improves iron absorption ○ *May cause constipation - patient should start on stool softeners or laxatives ○ Correct cause of bleeding ○ What do you need to teach patients about their stool when taking iron?? - stools may be black in color Thalassemia A group of diseases involving inadequate production of normal Hgb Results in decreased RBC production Due to absent or reduced globulin protein - abnormal Hgb synthesis Hemolysis occurs Genetic link - autosomal recessive genetic basis Clinical Manifestations Thalassemia minor (Thalassemia trait) ○ Often asymptomatic** ○ Mild to moderate anemia (Hgb 6 - 12) ○ Mild splenomegaly, bronzed skin color, and bone marrow hyperplasia ○ Body adapts to the reduction of Hgb - thus no treatment is indicated Thalassemia major ○ Life-threatening disease** ○ Growth and development deficits ○ Jaundice is prominent ○ Splenomegaly, hepatomegaly, cardiomyopathy ○ Symptoms develop in childhood ○ *Bone marrow responds to the reduced O2-carrying capacity of the blood by increasing RBC production ○ Cardiac complications from iron overload, lung disease, HTN ○ Endocrine problems, thrombosis Interprofessional Care Thalassemia major ○ Blood transfusions or exchange transfusions with chelating agents that bind to iron to reduce iron overloading ○ Luspatercept-aamt (Reblozyl) Improves Hgb levels and reduces transfusion needs Blocks inhibitors of late-stage RBC production ○ Splenectomy ○ Hematopoietic stem cell transplantation (HSCT) Megaloblastic Anemias (Macrocytic) Characterized by abnormally large RBCs, which are easily destroyed Caused by impaired DNA synthesis - result sin defective RBC maturation B12 (Cobalamin) Deficiency Most common cause = pernicious anemia (prevents body from absorbing B12) Defect of the gastric mucosa resulting in decreased formation of intrinsic factor (IF) necessary for absorption of B12 Gastrectomy, gastric bypass Other causes preventing absorption - small bowel resection, involving ileum Ileitis (inflammation of ileum) Crohn's disease Signs/Symptoms *Smooth beefy red tongue, fatigue, lab values Also desire to eat ice or other non-food things (pica) Neurological symptoms ○ Peripheral neuropathy - paresthesia hands and feet, loss of balance (ataxia) ○ Altered mental status - impaired memory, confusion to dementia **may be confused with alzheimer’s disease - this is a nutritional problem Diagnostics Hgb may be low, MCV is high (macrocytic), everything else may be fine Transferrin and ferritin may be up Vitamin B12 is low Folate level is normal Abnormal schilling test - pernicious anemia cause Demonstrates inability to absorb vit B12, without IF Elevated serum MMA and homocysteine levels Interprofessional and Nursing Management Parenteral or intranasal administration of cobalamin is the treatment of choice ○ Patients will die in 1-3 years without treatment ○ Anemia can be reversed with ongoing treatment, but long-standing neuromuscular complications may not be reversible Folic Acid Deficiency Folic acid is needed for DNA synthesis ○ RBC formation and maturation Manifestations are similar to B12 deficiency, but if neurologic symptoms present, may be caused by thiamine deficiency Common causes include ○ Diet deficiency, malabsorption syndromes ○ Alcohol use and anorexia ○ Loss during hemodialysis Folate is a water-soluble vitamin It is not stored in the body. Deficiency can occur in just a few weeks. Diagnostics Hgb may be low MCV is high (macrocytic) Transferrin and ferritin may be elevated Vitamin B12 is normal Folate level is low Signs/Symptoms Symptoms of anemia as previously mentioned ○ Smoothie beefy red tongue, fatigue, lab values Neural tube defects in pregnant individuals ○ Deficiency can result in spina bifida  Folate supplements are given when pregnant Anemia of Chronic Disease - Anemia of Inflammation Underproduction of RBCs and mild shortening of RBC survival Can be caused by: ○ Cancer ○ Autoimmune and infectious disorders (HIV, hepatitis, malaria) ○ Chronic inflammation ○ Heart failure ○ Bleeding episodes Usually develops after 1-2 months of disease activity Can become severe if the underlying disorder is not treated Diagnostics High serum ferritin Increased iron stored Normal folate and cobalamin Treat underlying causes is best Blood transfusions for severe cases Limited use of erythropoietin therapy History Determine onset, duration of symptoms Obtain a complete medical and surgical history Physical Exam Guided by history Symptoms similar to that of iron deficiency anemia Findings depend more on the nature of underlying disease than on the anemia ALWAYS obtain stool for occult blood. Aplastic Anemia Pancytopenia (decrease in ALL blood cell types) ○ RBCS, WBCs, platelets Hypocellular bone marrow Ranges from moderate to very severe - potentially fatal *Activity of bone marrow is depressed or has ceased Affects all cells made by the bone marrow (pancytopenia) Cause - congenital or acquired; idiopathic or autoimmune Exposure to certain drugs (antineoplastics, sulfonamides, anticonvulsants such as dilantin) Infections (hepatitis B & C, cytomegalovirus) Radiation Clinical Manifestations Abrupt or insidious development Symptoms caused by suppression of any or all bone marrow elements General manifestations of anemia ○ Fatigue, dyspnea ○ CV and cerebral responses ○ Neutropenia, thrombocytopenia Diagnosis by laboratory studies Decreased Hgb, WBC, platelet values Decreased reticulocyte count Elevated serum iron and TIBC Hypocellular bone marrow with increases yellow marrow (fat content) Management Prognosis of severe untreated aplastic anemia is poor Immunosuppressive therapy and HSCT transplantation can be curative Anemia Caused by Blood Loss (Acute & Chronic) Anemia from blood loss may be caused by acute or chronic problems Acute blood loss occurs because of sudden bleeding ○ Trauma, complications or surgery, problems that disrupt vascular integrity ○ 2 clinical concerns: hypovolemic shock, compensatory increased plasma volume within diminished O2 carrying RBCs Clinical manifestations ○ Caused by the body’s attempt to maintain adequate blood volume and meet oxygen requirements ○ Clinical s/s Pain - internal bleeding, tissue distention, organ displacement, nerve compression Retroperitoneal bleeding - numbness, pain in lower extremities ○ SHOCK is a major complication Interprofessional and Nursing Management ○ Replace blood volume ○ Promote coagulation ○ Find the source of bleeding/stop blood loss ○ Correct RBC loss ○ Provide supplemental iron ○ May be challenging to prevent blood loss if caused by trauma ○ Postoperative patients - monitor blood loss, give blood products for anemia Chronic blood loss ○ Sources: bleeding ulcer, hemorrhoids, menstrual and postmenopausal blood loss ○ Management involves Identifying the source and stop bleeding Providing supplemental iron as needed Hemolytic Anemia Destruction or hemolysis of RBCs at a rate that exceeds production ○ Caused by problems intrinsic or extrinsic to the RBCs Intrinsic forms are usually hereditary and result from defects in RBCs themselves RBCs are normal in acquired forms, but damage is caused by external factors General manifestations of anemia Specific manifestations - jaundice, enlargement of spleen and liver Maintenance of renal function is a major focus of treatment Signs/symptoms ○ Cold reacting symptoms (Raynaud’s disease) Red cells clump in capillary beds Cyanosis, pain, paresthesia ○ Warm reacting symptoms Severe hemolysis-jaundice, splenomegaly CHF, palpitation Diagnosis ○ Coombs test - presence of antibody or complement Treatment - corticosteroids, transfusion, splenectomy Sickle Cell Disease (SCD) Group of inherited, autosomal recessive disorders (genetic) ○ An abnormal form of Hgb in RBC ○ Genetic disorder usually found routine neonatal screening ○ incurable , significantly affects quality of life An inherited disorder that affects the synthesis of Hgb HB S (sickle), replaces HB A (normal) Can carry oxygen, but when oxygen levels fall, the cells go into sickle shape which causes plugs in the circulation, leading to tissue ischemia also RBCs have a shortened life spain (7-20 days) Predominantly in african-american population Symptoms The results of blockage from the sickle cells ACUTE: ○ Pain in back, chest, extremities ○ Low grade fever, 1-2 days after onset of pain ○ Could have a stroke ○ Jaundiced CHRONIC: ○ Kidney ○ Vision ○ Musculoskeletal  ○ Spleen Complications Infection is a major cause of mortality ○ Spleen function becomes compromised from sickled RBCs ○ Pneumococcal pneumonia most common ○ Severe infections can cause gallstones ○ Can lead to shutdown of RBC production Acute chest syndrome ○ Pneumonia, tissue infarction, and fat embolism ○ Fever, chest pain, cough, lung infiltrates, and dyspnea ○ Pulmonary infarctions may cause: pulmonary hypertension, MI, cor pulmonale, HF, retinal detachment and blindness, renal failure, stroke Management Maintain adequate fluids Pain management Oxygen therapy Avoid high altitudes - why? - low oxygen! Treat infections promptly Screening for retinopathy Immunizations - pneumococcal, H. influenzae, Hepatitis Urgent visit to the hospital during crises Patient and caregiver support Polycythemia The production and presence of increased number of RBCs Primary polycythemia/polycythemia vera ○ A bone marrow disorder leading to an abnormal increase in blood cells: RBCs (mostly HCT) ○ Cause: genetic defect (primary) or oxygen deficit (secondary) ○ Result: HCT rises (>48% in women and > 52% in men); blood viscosity is high and patient is at risk for blood clots - DVT, strokes, MI ○ Increased blood viscosity and blood volume - now at risk for clotting!! ○ Splenomegaly and hepatomegaly Secondary polycythemia ○ Can be hypoxia-driven or hypoxia-independent ○ Hypoxia-driven hypoxia stimulates the kidneys to make EPO which stimulates RBC production ○ Hypoxia-independent - cancer or benign tumor tissue makes EPO Clinical Manifestations ○ Headache, dizziness, blurred vision, tinnitus, plethora, or ruddy complexion ○ Erythromelalgia (painful redness and burning of hands and feet) ○ Thrombo-embolism events, with stroke the most common event ○ Hemorrhagic events: bruising, GI bleed Treatment ○ Monitor Hgb, HCt levels ○ Periodic phlebotomy to maintain normal Hgb and HCT ○ Hydration to reduce blood viscosity ○ Chemotherapy to reduce number of RBCs or interferon to lower blood counts