Hematology Vocabulary

Hematology Pathophysiology

Introduction

• Professor Michelle R. Tolbert, MSN, RN, IP, PMHNP-Candidate
• NUR 2063 Patho- Mod 07

Key Aspects of Hematology

• Production function of blood cells
• Lifespan of blood cells
• Tissue Hypoxia
• Immunosuppression
• Intrinsic and Extrinsic Influences
• Early recognition is critical in Nursing Care

Etiologies of Hematologic Disorders

• Anemia: Reduced oxygen-carrying capacity of blood due to decreased RBCs, Hemoglobin, or both.
• Leukemia: Malignant disorder affecting WBCs, characterized by unregulated proliferation of immature leukocytes.
• Thrombocytopenia: Deficiency of platelets in the blood, increasing the risk of bleeding.
• Genetic and Environmental Factors:
  • Inherited mutations (e.g., Philadelphia chromosome)
  • Germline mutations (can be passed to offspring)
  • Somatic mutations (can lead to myelodysplastic syndromes)

Environmental Influences on Hematologic Disorders

• Carcinogen Exposure: Increases leukemia risk.
• Viral infections: Can lead to lymphomas.
• Toxins: Accumulation in bone marrow cells.

Hematopoiesis

• Differentiation, proliferation, and apoptosis of blood-forming cells.
• Oncogenes: Mutated proto-oncogenes that drive unregulated cell growth.
• Tumor suppressor genes: When inactivated, they allow unchecked proliferation.

Mutation Types and Effects on Hematopoiesis

• Point Mutation
• Chromosomal translocation
• Gene amplification
• Effect on Hematopoiesis:
  • Abnormal protein (e.g., Acute Myeloid Leukemia)
  • Overexpression of oncogenes (e.g., Chronic Myelogenous Leukemia)
  • Excessive growth factor (e.g., Non-Hodgkin Lymphoma)

Clinical Example: Acute Lymphocytic Leukemia (ALL)

• 19-year-old male presents with fatigue, fever, and recurrent nosebleeds.
• Labs reveal pancytopenia and 40% blasts on peripheral smear.
• Bone marrow biopsy confirms Acute Lymphocytic Leukemia (ALL).
• Underlying cause: Chromosomal translocation creating a fusion gene that disrupts normal lymphoid maturation.
• Recognize nonspecific early symptoms.

Clinical Signs and Symptoms of Hematologic Disorders

• Presentation can involve generalized or specific symptoms depending on the type and severity.
  • Fatigue
  • Pallor
  • Bleeding tendencies
  • Recurrent infections
  • Organomegaly
  • Bone pain
• Hallmark of anemia: Fatigue
• Visual assessment findings: conjunctivae, palms, nail beds
• Easy bruising, prolonged bleeding: Thrombocytopenia or Clotting Factor Deficiencies
• Dysfunction in WBC: Leukopenia
• Enlarged liver or spleen: Seen in Leukemia due to overcrowding by abnormal blast

Diagnostic Testing in Hematology

• CBC: Hgb, HCTZ, RBC, WBC, and Platelet count. Key in initial assessment.
• Peripheral blood smear: Helpful in hemolytic anemias and leukemias.
• Bone marrow aspiration and biopsy: Essential for diagnosing leukemias, lymphomas, and marrow failure syndromes.
• Genetic Testing: Detects sickle cell disease or thalassemia.
• Coagulation studies (PT, aPTT, INR): Assesses clotting efficiency.

Treatment Modalities

• Blood Transfusion: RBC, platelet. Monitor for transfusion reactions (fever, chills, back pain).
• Chemotherapy: For leukemia and lymphoma to eradicate malignant cells. Causes Myelosuppression. Monitor for neutropenia, bleeding, and anemia.
• Bone Marrow/Stem cell transplant (BMT/SCT): Replaces diseased marrow. Autologous cells can be used.
• Immunotherapy: Monoclonal antibodies (e.g., rituximab).
• Pharmacologic agents: Erythropoiesis-stimulating agents – increases RBC production.
• Iron, B12, folate supplementation.
• Anticoagulants or clothing factors for bleeding disorders.

Clinical Example: Iron-Deficiency Anemia

• 56-year-old female reports fatigue, shortness of breath, and heavy menstrual bleeding.
• CBC reveals HGB of 7.8 g/dL. Iron studies confirm iron-deficiency anemia.
• She receives a unit of packed RBCs, iron IV infusion, and is referred for gynecological evaluation to address the bleeding source.

Key Takeaways

• Hematologic disorders result from disruptions in blood cell production, function, or survival. Causes include genetic mutations, autoimmune mechanisms, environmental exposures, or neoplastic growth.
• Inherited and acquired mutations disrupt hematopoiesis and are central to leukemia and lymphoma.
• Fatigue, pallor, bruising, and infections reflect underlying dysfunction.
• CBC w/diff, peripheral smears, and bone marrow biopsy are diagnostic tools in determining type and severity.
• Treatments include blood transfusions, chemotherapy, bone marrow transplants, immunotherapies, and supportive medications like growth factors.
• Nurses monitor for transfusion reactions, myelosuppression, and signs of treatment effectiveness or toxicity.
• Nurses educate patients on the course of therapy and safety precautions.

Nursing Hints

• Prioritize nursing care.
• Monitor CBC trends.
• Neutropenic precautions.
• Bleeding risk management.
• Energy conservation strategies.