hematology

Hematologic Disorders, Blood Transfusion, and Anemias

Overview of Hematologic Disorders

  • Disorders Affecting Blood Components: Involves red blood cells (RBCs), white blood cells (WBCs), platelets, and clotting factors.

  • Bone Marrow Function: Responsible for the production of RBCs, WBCs, and platelets.

    • Impact: Affects oxygenation, immunity, and the clotting process.

Diagnostic Tests

  • Complete Blood Count (CBC): Key indicators include:

    • Hemoglobin (H&H)

    • RBC Count

    • WBC Count and Differential, which includes:

    • Mean Corpuscular Volume (MCV)

    • Mean Corpuscular Hemoglobin (MCH)

  • Iron Studies:

    • Iron level

    • Total Iron Binding Capacity (TIBC)

  • Coagulation Studies:

    • Prothrombin Time (PT)

    • Activated Partial Thromboplastin Time (aPTT)

    • International Normalized Ratio (INR)

    • D-dimer test

    • Fibrinogen test

    • Fibrin degradation products

  • Other Tests:

    • Bone Marrow Biopsy

    • Peripheral Blood Smear

Normal Ranges for Common Blood Tests

  • WBC Count: 3.8-10.8 × 10³/µL

  • Hemoglobin (Hgb): 13.2-17.1 g/dL (varies with burns)

  • Hematocrit (Hct): 38.5%-50%

  • Platelets: 140-400 × 10³/µL

  • Mean Corpuscular Volume (MCV): 80-100 fL

  • Mean Corpuscular Hemoglobin (MCH): 27-33 pg

  • Mean Corpuscular Hemoglobin Concentration (MCHC): 32-36 g/dL

  • Mean Platelet Volume (MPV): 7.5-11.5 fL

Differential Diagnoses for WBC Counts

  • Increased WBC (A):

    • Causes: Infection, inflammation, necrosis, malignancy, hyperleukocytosis, leukostasis, thyrotoxicosis, eclampsia, medications (like steroids), cigarette smoke, idiopathic reasons, multiple myeloma, hypersensitivity reactions.

  • Decreased WBC (A):

    • Causes: Infection, cytotoxic drugs, malignancy, autoimmune diseases, malnutrition, HIV/AIDS, systemic diseases, stress.

Classification and Anemias

  • Increased RBC/Spherocytosis: Polycythemia vera, leukemia, myelodysplastic syndrome, infections, stress.

  • Decreased Platelets (A): Immune Thrombocytopenia (ITP), Thrombotic Thrombocytopenic Purpura (TTP), Hemolytic Uremic Syndrome (HUS), Heparin-Induced Thrombocytopenia and Thrombosis (HITTS), Disseminated Intravascular Coagulation (DIC), Hemolysis, chronic liver disease.

Used to Classify Types of Anemia

  • Macrocytosis: Iron deficiency anemia, thalassemia.

  • Microcytic Anemia: Iron deficiency anemia, thalassemia.

  • Spherocytosis/Hemolytic Anemias: Various causes leading to RBC destruction.

Specific Tests for Anemia Classification

  • Iron Studies: Total iron binding capacity (TIBC) reflects iron binding proteins; Ferritin indicates total iron stores.

  • Additional Tests:

    • Hemoglobin Electrophoresis: Measures and quantifies types of hemoglobin to identify thalassemia and sickle cell disease.

    • Sickle Cell Test: Evaluates sickling in RBCs under reduced oxygen tension.

    • Schilling Test: Assesses vitamin B12 absorption for differentiation of malabsorption vs. pernicious anemia.

    • Bone Marrow Aspiration: Critical for diagnosing aplastic anemia.

Coagulation Testing Ranges

  • PT: 10-12 seconds

  • aPTT: 25-35 seconds

  • INR: 0.9-1.2 seconds (normal); typically 2-3 seconds for warfarin therapy.

  • D-Dimer: <300 µg/L

Iron Disorders Panel

  • Key Tests Include:

    • Fasting Serum Iron: 40-180 mcg/dL

    • Hemoglobin (Hgb): 12-14 g/dL

    • Serum Ferritin: 50-100 ng/mL

    • Total Iron Binding Capacity (TIBC): 250-450 mcg/dL

    • Transferrin-Iron Saturation % (TS%): 25-35%

Bone Marrow Aspiration/Biopsy

  • Purpose: Diagnoses blood disorders, identifies bone marrow diseases such as leukemia, infections, and stages lymphoma or other cancers.

  • Pre-Procedural Considerations: Informed consent, positioning (prone or side-lying), conscious sedation, local anesthetic considerations (patient may hear sounds).

  • Intra-Procedural Considerations: Sedation monitoring, assisting provider while maintaining sterility.

  • Post-Procedural Considerations: Pressure application to the site to control bleeding, 30-60 minutes of bedrest, infection monitoring, patient education about potential bruising and avoiding anticoagulants like aspirin.

Blood and Blood Product Transfusions Overview

  • Purpose: Restore blood volume, improve oxygen-carrying capacity, provide clotting factors.

  • Types of Blood Products:

    • Whole blood, packed RBCs, washed RBCs, platelets, fresh frozen plasma (FFP), cryoprecipitate.

  • Transfusion Types:

    • Standard donation, autologous donation (client’s collected blood for future use), intraoperative blood salvage (collection and filtration of blood lost during procedures).

Indications for Blood Transfusions

  • Excessive Blood Loss: Use packed RBCs.

  • Severe Anemia: Hgb < 6, utilize packed RBCs.

  • Kidney Failure: Packed RBCs are indicated.

  • Coagulation Disorders (e.g. hemophilia): Fresh Frozen Plasma (FFP).

  • Thrombocytopenia: Platelet transfusion.

  • Burns and Hypoproteinemia: Albumin administration.

Specific Transfusion Types and Administration

  • Platelet Transfusion: Typically around 200-300 mL/bag. Retrieve from blood bank and administer within 30 minutes of obtaining. Monitor vital signs at baseline, 15 minutes after starting, and post-infusion.

  • Plasma Transfusion: FFP is frozen shortly after donation and must be thawed prior to administration. Infuse 200 mL of FFP rapidly over 15-30 minutes through standard Y-set or straight filtered tubing.

  • WBC Transfusion: Rarely done due to reaction risks. If receiving Amphotericin B, a 4-6 hour interval from WBC transfusion is recommended to prevent hemolysis. Infuse in 400 mL plasma over 45-60 minutes while monitoring vital signs every 15 minutes.

  • Washed RBCs: Guidelines state a unit of 200 mL is to be infused over 2-4 hours, necessary for patients with previous transfusion reactions or those who have undergone hematopoietic stem cell transplants.

  • Transfusion Protocol: Includes adherence to compatibility testing, type and cross-match checks, and specific protocols for plasma products to verify ABO compatibility. Rh factor importance: Rh-negative individuals may develop antibodies if sensitized to Rh-positive blood.

Nursing Actions with Blood Transfusions

  • Patient Education: Ensure client understands procedures and risks.

  • Vital Signs: Assess and document pre-transfusion and every 15 minutes during initial infusion.

  • Lab Review: Observe lab results pre and post-transfusion.

  • Consent Verification: Ensure proper consent is obtained prior to transfusion.

  • Patient History: Inquire regarding previous transfusions and any potential reactions.

  • IV Access: Large bore IV (18 or 20g) is required along with following proper protocols for blood product procurement.

  • Product Verification: Confirm blood products and ensure safe disposal post-transfusion, along with necessary paperwork completion.

Blood Compatibility and Types

  • ABO Blood Groups & Rh Factor:

    • Universal Donor: O-

    • Universal Recipient: AB+

Blood Type Compatibility

Blood Type

Gives

Receives

A+

A+, AB+

A+, A-, O+, O-

B+

B+, AB+

B+, B-, O+, O-

AB+

Everyone

AB+, AB-, A-, B-, O-

O+

O+, A+, B+, AB+

O+, A-, B-, AB-

A-

A-, AB-

A-, O-

B-

B-, AB-

B-, O-

AB-

AB-

AB-, A-, B-, O-

O-

O-

O-

Transfusion Reactions

  • Acute Hemolytic Reaction:

    • Onset: Immediate.

    • Cause: Transfusion of incompatible blood.

    • Signs: Fever, chills, hematuria, low back pain, tachycardia, flushing, hypotension, anxiety.

    • Nursing Actions: Stop transfusion, maintain IV access with normal saline, send blood and tubing to the lab.

  • Febrile Reaction:

    • Onset: Commonly within 2 hours of infusion.

    • Cause: Anti-WBC antibodies develop.

    • Signs: Fever, chills, flushing, hypotension.

    • Nursing Actions: Use WBC filter for administration; stop transfusion, administer antipyretics, initiate normal saline with new tubing.

  • Allergic Reaction:

    • Onset: During or up to 24 hours post transfusion.

    • Cause: Sensitivity to transfused components.

    • Signs: Itching, urticaria, anaphylaxis.

    • Nursing Actions: Stop transfusion, administer antihistamines, and monitor closely.

  • Bacterial Reaction:

    • Onset: During or several hours post transfusion.

    • Cause: Contaminated blood products.

    • Signs: Wheezing, dyspnea, chest tightness, shock.

    • Nursing Actions: Stop transfusion and administer antibiotics.

  • Circulatory Overload Reaction:

    • Onset: Can occur during transfusion.

    • Cause: Rapid transfusion rate.

    • Signs: Crackles, dyspnea, anxiety, jugular vein distension.

    • Nursing Actions: Slow/stop infusion, administer oxygen, diuretics.

Anemias

  • Definition: Decreased levels of RBCs or hemoglobin, leading to impaired oxygen delivery to tissues.

  • Causes: Blood loss, inadequate RBC production, increased RBC destruction (hemolytic), nutritional deficiencies (iron, folic acid, vitamin B12), and bone marrow suppression.

    • Specifics:

    • Iron deficiency anemia due to inadequate intake is prevalent among children, adolescents, and pregnant clients.

    • Blood loss leading to iron deficiency anemia is common in postmenopausal women and males.

    • Menstruating clients may develop anemia secondary to menorrhagia.

Types of Anemia

  • Iron Deficiency Anemia: Characterized by low iron levels, pallor, fatigue.

  • Vitamin B12 Deficiency: Leads to neurological symptoms.

  • Folic Acid Deficiency: Results in megaloblastic cells.

  • Aplastic Anemia: Bone marrow fails to produce adequate blood cells.

  • Hemolytic Anemia: Often includes sickle cell and G6PD deficiency.

Health Promotion and Disease Prevention

  • Recommendations for Pregnant/Menstruating Clients: Ensure diet includes sufficient iron-rich foods.

  • Dietary Suggestions for Iron Deficient Individuals: Integrate iron-rich options that are not red or organ meats (e.g., iron-fortified cereals, fish, poultry).

  • Folic Acid Consumption: Promote regular intake of folate-rich foods (spinach, lentils).

Risk Factors of Anemia

  • Blood loss (acute or chronic), trauma, menstruation, GI bleed, surgical complications, environmental exposures, rapid metabolism, pregnancy, infections, hemolytic conditions, dietary inadequacies, bone marrow suppression, aging, etc.

Expected Findings in Anemia

  • May be asymptomatic initially but common signs include pallor, fatigue, dizziness, shortness of breath, smooth sore tongue, and paresthesia in cases of Vitamin B12 deficiency.

Laboratory Assessments for Anemia

  • Obtain CBC with differential; understand that RBCs are essential for hemoglobin transport.

  • Analyze RBC indices (MCV, MCH, MCHC) to classify anemia types based on cell size and hemoglobin content.

Iron Studies Explanation

  • Total Iron Binding Capacity (TIBC): Reflects proteins binding with iron; utilized for diagnostics.

  • Ferritin: A critical measure of total iron storage in the body.

  • Low blood iron levels with elevated TIBC: Suggest iron deficiency anemia.

Additional Diagnostic Tests for Anemia

  • Tests such as Hgb electrophoresis help identify and differentiate types of hemoglobin, and further investigations guide towards treatment plans.

Sickle Cell Anemia Overview

  • Mechanism: Mutation generates abnormal hemoglobin chains; RBCs take on a sickle shape, leading to clumping and blockage.

  • Prevalence: Particularly common in individuals of African descent, with approximately 1 in 3 carrying the trait.

Patient-Centered Care in Anemia

  • Encourage nutrient intake (vitamins B12, folic acid), monitor oxygen saturation, administer medications as prescribed, educate clients on energy conservation, and outline expected outcomes.

Medications for Anemia Treatment

  • Iron Supplements: Including Ferrous sulfate, Ferrous fumarate, and Ferrous gluconate for oral replenishment. Parenteral iron for severe cases, with instructions to monitor response and dietary absorption (Vitamin C aids absorption).

  • Erythropoietin (Epoetin alfa): A helper in increasing RBC production; monitor hemoglobin levels closely to avoid rapid BP increase.

  • Vitamin B12 (Cyanocobalamin): Essential for conversion processes; administer based on absorption needs. Clients with pernicious anemia require lifelong injections.

  • Folic Acid: Necessary for RBC production; high doses can mask Vitamin B12 deficiencies or turn urine dark yellow.

Complications of Anemia

  • May lead to heart failure due to increased workload; monitor patient closely for vital signs, oxygen saturation, and manage weight and blood transfusion orders.

Leukemia Overview

  • Type of blood cancer characterized by an uncontrolled WBC production, often following Myelodysplastic Syndrome (MDS).

    • Acute Leukemia: Affects younger populations and progresses rapidly.

    • Chronic Leukemia: Common in older populations, with a more insidious onset.

  • Assessment Findings: Abnormal lab findings with low H&H, low platelets, and changes in WBCs.

Common Symptoms of Leukemia

  • Systemic: Weight loss, fever, infections.

  • Respiratory: Shortness of breath.

  • Muscular: Weakness.

  • Psychological: Fatigue.

  • Hematological: Bruising, skin changes, and enlarged lymph nodes.

Leukemia Treatment Modalities

  • Chemical Therapy: Aimed for remission with varied stages - induction, intensification, consolidation, and maintenance therapies.

  • Complications to Monitor: Risk for infections from reduced immunity, injury risks due to thrombocytopenia, and fatigue levels.

Malignant Lymphomas

  • Includes Hodgkin and Non-Hodgkin's Lymphomas. Diagnosed through lymph node biopsies and additional imaging studies.

  • Hodgkin’s Lymphoma: Presence of Reed-Sternberg cells, common age groups involved are 15-20 years, and older adults. Symptoms include swollen lymph nodes, fever, and night sweats.

  • Non-Hodgkin’s Lymphoma: More widespread lymphatic involvement, often seen in older adults, requiring chemotherapy for management.

Thrombocytopenic Purpura Overview

  • Destructive selection resulting in reduced circulating platelets.

  • Types:

    • ITP: Idiopathic Thrombocytopenic Purpura - leads to purpura, and is treated with corticosteroids, IVIG, or splenectomy.

    • TTP: Thrombotic Thrombocytopenic Purpura - life-threatening due to small blood clots; requires urgent plasma exchange.

    • HIT: Heparin-Induced Thrombocytopenia - spontaneous resolution; necessitates discontinuation of heparin and the use of alternative anticoagulation treatment.