Hemolytic Anemia and Coagulation Quiz

Hematologic Disorders - Objectives

• Relate anatomy and physiology to nursing care for individuals with hematologic and immunologic problems.
• Integrate pathophysiologic changes of normal blood cells with clinical assessments in adult patients.
• Develop nursing interventions for adults with hematologic and immunologic function alterations.
• Discuss major care concerns for patients and families undergoing hematologic and immunologic treatments.

Coagulation Cascade

• Control bleeding by initiating a triggering event.
• Landslide effect: cascades through two paths.
• Intrinsic pathway (slower – scab formation): XII, XI, IX, VIII, common pathway.
• Extrinsic pathway (faster): tissue factor, VII, common pathway.
• Common pathway: X, V, Ca++, lipids, prothrombin (II), thrombin, fibrinogen (I), fibrin clot (XIII).
• Outcome is formation of a fibrin clot leading to coagulation .
• Fibrin clot stops bleeding.

Hematologic Assessment - Common Lab Tests

• Complete blood count (CBC):
  • White blood cell count (WBC)
  • Red blood cell count (RBC)
  • Platelet count
  • Hematocrit (HCT)
  • Hemoglobin (Hb)
  • Oxygen-carrying protein in red blood cells.
• Differential white blood cell count (WBC differential)
• Prothrombin time (PT): measures clotting time.
• Partial thromboplastin time (PTT): evaluates bleeding and clotting disorders; monitors anticoagulation.
• International Normalized Ratio (INR): used in monitoring anticoagulant therapies
• Red blood cell (RBC) indices:
  • Mean corpuscular volume (MCV): average RBC size.
  • Mean corpuscular hemoglobin (MCH): hemoglobin amount per red blood cell.
  • Mean corpuscular hemoglobin concentration (MCHC): hemoglobin concentration per red blood cell size.

Absolute Neutrophil Count (ANC)

• Reflects the number of mature white blood cells (WBCs).
• Important for patients receiving chemotherapy (may suppress immune system)
• Needs to be above 3,000.
• Calculation: (Neutrophils + Bands) x Total WBC = ANC
  • Neutrophils: white blood cells
  • Bands: immature white blood cells

Hemolytic Anemia

• Body destroys red blood cells (RBCs).
• Abnormal or premature destruction of RBCs.
• Intrinsic (e.g., sickle cell anemia) or result of infection, chemical, immune response, or radiation.
• Hemolytic crisis: extreme physiologic or emotional stress.
• Can be triggered by anything.
• Diagnosis: decreased haptoglobin, elevated bilirubin, LDH, cold agglutinins, reticulocyte counts, peripheral blood smear
• Signs/symptoms may include fever, headache, joint pain, abdominal pain.

Diagnosing Hemolytic Anemia

• Initial laboratory tests:
  • Haptoglobin: Decreased (Binds free hemoglobin)
  • Lactate dehydrogenase: Elevated (Released from lysed RBCs)
  • Peripheral blood smear: Abnormal RBC shapes
  • Reticulocyte count: Increased (Marrow response to anemia)
  • Unconjugated bilirubin: Increased (Increased hemoglobin breakdown)
  • Urinalysis: Urobilinogen, positive for blood (Free hemoglobin and metabolites).
• H&H: hemoglobin and hematocrit
• LDH: Liver enzyme, elevated when liver is working to process destroyed RBCs
• Reticulocyte counts: immature red blood cells indicating high/rapid turnover
• Hepatoglobin: Proteins made in liver to get rid of circulating hemoglobin
• Hematocrit: Percentage of blood volume composed of RBCs
• Bilirubin: substance produced by breakdown of red blood cells

Aplastic Anemia

• Inability of erythrocyte-producing organs (bone marrow) to produce RBCs.
• Usually involves pancytopenia (reduction in all blood cell types).
• Causes: antineoplastic or antimicrobial agents, immunological disorders, body attacks.
• Diagnosis: decreased H&H, WBC, platelets, reticulocyte count, prolonged bleeding time.
• Signs/symptoms: fever, infection, weakness, bleeding, dizziness, dyspnea, oral ulcerations.

Management of Aplastic Anemia

• Determine and discontinue the cause.
• Transfusion with packed RBCs or frozen plasma.
• Transfusion of platelets.
• Psychosocial support
• Bone Marrow Transplants may be an outcome if other methods aren't effective.
• Antibiotics or similar may be prescribed if infection is involved.

Leukemia - Basic Physiology

• Malignant transformation of stem cells, causing abnormal proliferation of a specific leukocyte stem cell type.
• Proliferations shuts down normal bone marrow production.
• Results in anemia, thrombocytopenia, leukopenia of unaffected WBC types.

Leukemia - Causes and Types

• Many immature white blood cells in the bone marrow.
• May be acute or chronic.
• Categorized by the specific maturational pathway from which the abnormal cell arose.
  • Lymphocytic/lymphoblastic
  • Myelocytic/myelogenous

Leukemia - Acute and Chronic

• Acute Lymphoblastic Leukemia:
  • Malignant proliferation of WBC precursors (blasts).
  • Accumulate in bone marrow, body tissues, and blood vessels.
  • Prevent normal WBC maturation; immature cells accumulate in bone marrow.
• Chronic Leukemia:
  • Malignant proliferation of abnormal immature WBCs.
  • Prevents maturation of WBCs.

Diagnosing Leukemia

• Acute: Fever, diffuse petechia (little hemorrhages under the skin), bleeding, bruising, anorexia, increased WBC, uric acid, PT, and PTT.
• Chronic: Fatigue, anorexia, weight loss, increased WBC, decreased hemoglobin

Common Symptoms of Leukemia

• Systemic: weight loss, frequent infections
• Lungs: easy shortness of breath
• Muscular: weakness
• Bones/joints: pain or tenderness
• Psychological: fatigue, loss of appetite
• Lymph nodes: swelling
• Spleen/liver: enlargement
• Skin: night sweats, easy bleeding, bruising, purplish patches or spots.

Management of Leukemia

• Chemotherapy
• Radiation (chronic)
• Blood product support
• Bone marrow transplantation
• Psychosocial support

Leukemia Etiology

• Many genetic and environmental factors involving gene damage to cells.
• May be viral pathogenesis
• Can be secondary to bone marrow damage from radiation.

Diagnosing Leukemia - Lab Assessment

• Decreased H&H
• Decreased platelets
• Altered WBC (low, normal, elevated: usually 20,000 to 100,000)
• Bone marrow aspiration/biopsy identifies types.

Leukemia - Drug Therapy

• Intensive combination chemotherapy
• Major side effects:
  • Bone marrow depression
  • Increased vulnerability to infection

Malignant Lymphoma

• Cancer of the lymph nodes.
• Malignancies characterized by proliferation of committed lymphocytes rather than stem cell precursors.
• Secondary to solid tumors affecting lymph nodes and spleen
• May involve Hodgkin's or Non-Hodgkin's lymphomas.

Hodgkin's Lymphoma - Incidence and Causes

• Disease of young people.
• Peaks in mid to late 20s.
• Males under 50 affected more than males over 50.
• Probably viral or chemical causes.
• Usually originates in a single or chain of lymph nodes.

Hodgkin's Lymphoma - Assessment

• Enlarges painless lymph nodes
• Fever
• Malaise (feeling yucky), night sweats.

Hodgkin's Lymphoma - Diagnosis

• Biopsy reveals Reed-Sternberg cells.
• Staging is crucial.

Hodgkin's Lymphoma - Treatment

• Stage 1-2: extensive radiation.
• Stage 3-4: more extensive radiation with aggressive multi-agent chemotherapy
• Nursing management focuses on side effects.
• Staging (I-IV): crucial for treatment planning.

Non-Hodgkin's Lymphoma

• Cancer of lymph tissue that is not Hodgkin's.
• Most common in older adults.
• Cause unknown.
• Suspected causes include viral exposure, chemical exposure or radiation
• Prognosis depends on cell type (ranges from excellent to poor),
• Treatment and nursing care similar to Hodgkin's lymphoma.

Autoimmune Thrombocytopenic Purpura (AITP)

• Autoimmune disorder.
• Body produces anti-platelet antibodies, destroying the platelets.
• Platelet count typically less than 20,000
• Normal platelet count is 150,000.
• Signs/symptoms may include bleeding, bruising, and other abnormal bleeding complications.

Clotting Factor Disorders

• Mostly congenital gene abnormalities of one clotting factor.
• Examples: Hemophilia A & B, von Willebrand's disease

Hemophilia

• Inherited bleeding disorder characterized by a deficiency of one or more clotting factors (most often VIII & IX).
• Individuals primarily males are usually affected; females are usually asymptomatic carriers.

Diagnosis of Hemophilia

• Diagnosed by blood tests (prolonged PTT, bleeding time, activated clotting time, normal platelet, PT, thrombin time, fibrinogen levels), and low factor activity (VIII and IX).

Hemophilia (Management)

• Factor transfusion (#1).
• Transfusion of fresh frozen plasma (FFP) containing clotting factors.
• Cryoprecipitate: specific form of plasma containing clotting factor concentration.
• Desmopressin (DDAVP).
• Aminocaproic acid (amicar).
• Psychosocial support.

Anemia of Chronic Disease - Renal Dysfunction

• Anemia resulting primarily from deficient erythropoietin (EPO).
• Kidneys not producing enough EPO.
• Treatment: Epoetin (EPO, Procrit, Epogen), supplementation with EPO, and sometimes iron.
• Treatment includes measures to correct the underlying disorder.

Sickle Cell Disease

• Genetic disorder causing abnormal red blood cell shape (sickle-shaped).
• Rigid cells cause blockages in blood vessels, leading to pain and organ damage
• Most common in African, Central/South American, Cuban, Indian, Saudi Arabian, Mediterranean, and Hispanic populations.

Vasoclusion in Sickle Cell Anemia

• Without enough blood flow= organ damage
• Causes acute and chronic organ damage.
• Acute complications: sickle cell vaso-occlusive pain crisis, hepatic crisis, splenic crisis, priapism.
• Chronic complications: stroke, chronic lung disease with pulmonary hypertension, renal failure, avascular necrosis.

Sickle Cell Crisis (precipitating factors)

• Hypoxia
• Acidosis
• Fever
• Infection
• Dehydration
• Exposure to cold
• High altitudes

Sickle Cell Anemia - Painful Events - Management

• Administer O2
• Correct fluid/electrolyte abnormalities; hypotonic fluids
• Treat any underlying illnesses using opioid analgesics (morphine, but not meperidine).
• Avoid blood transfusions for uncomplicated pain episodes.
• Incentive spirometry during waking hours.

Nursing Actions (Anemic patients or blood loss)

• Administer oxygen, blood products (transfusion) and erythropoietin as prescribed.
• Rest between activities, elevate head of bed during episodes of shortness of breath.
• Provide extra blankets to keep the patient warm
• Teach patients about underlying pathophysiology and how to manage the anemia.

Bleeding Precautions (thrombocytopenia)

• Electric razor.
• Discontinue Aspirin and other medications that cause bleeding.
• Limit needle sticks for punctures.
• Evaluate for other issues that may indicate blood loss.
• Restrict visitors, who may cause unnecessary physical contact.

Practice Questions (Answers may vary):

• A 32-year-old with sickle cell anemia and a sickle cell crisis: Administer oxygen first.
• Bleeding precaution for a thrombocytopenia patient: Use and electric razor; discontinue aspirin; and limit the number of needle sticks.