Hematology and Oncology Book

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Hemolytic AnemiaAka: Hemolysis

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  1. See Also
    1. Anemia
    2. Anemia with Reticulocytosis
  2. Types
    1. Extravascular Hemolysis (most common)
      1. Splenic and hepatic clearance of defective RBCs
    2. Intravascular Hemolysis
      1. RBC membrane injury (trauma, autoimmune, infection)
  3. Causes
    1. See Hemolytic Anemia Causes
      1. See Autoimmune Hemolytic Anemia
      2. See Microangiopathic Hemolytic Anemia
    2. Common Causes
      1. Lymphoproliferative Disorders
        1. Chronic Lymphocytic Leukemia
        2. Non-Hodgkin's Lymphoma
      2. Iatrogenic Hemolytic Anemia
      3. Medications (Most common cause)
        1. See Drug-Induced Hemolytic Anemia
      4. Connective Tissue Disorders
        1. Systemic Lupus Erythematosus
        2. Rheumatoid Arthritis
      5. Infection
        1. Epstein Barr Virus (Mononucleosis)
        2. Cytomegalovirus (CMV)
        3. Mycoplasma pneumonia
      6. Paroxysmal Cold Hemoglobinuria
      7. Favism (G6PD Deficiency)
  4. Symptoms
    1. See Anemia
    2. Fatigue
    3. Dyspnea on exertion
    4. Back pain
    5. Fatigue
    6. Dark Red Urine
  5. Signs
    1. See Anemia
    2. Lymphadenopathy
    3. Splenomegaly
    4. Hepatomegaly
    5. Low grade fever
    6. Jaundice
    7. Pallor
    8. Tachycardia
  6. Labs
    1. Complete Blood Count
      1. Normocytic Anemia is most common
    2. Coombs test
      1. Direct Coombs
      2. Indirect Coombs
    3. Reticulocyte Index >3% (Reticulocytosis)
      1. Normal response within 3-5 days of Anemia onset
    4. Serum Haptoglobin decreased
    5. Liver Function Tests
      1. Serum Unconjugated Bilirubin elevated
      2. Lactate Dehydrogenase (LDH) elevated
    6. Urinalysis
      1. Hemosiderinuria (requires prussian blue staining)
    7. Peripheral Smear
      1. Spherocytes (very difficult to identify on Peripheral Smear)
        1. See osmotic fragility with reflex Band 3 Protein below
        2. Congenital spherocytosis (Negative Direct Coombs)
        3. Immune hemolytic Anemia (Positive Direct Coombs)
      2. Schistocytes
        1. Microangiopathic Anemia (eg. Heart valve hemolysis)
      3. Hypochromic Microcytic Anemia
        1. Thalassemia
      4. Sickle Cells
        1. Sickle Cell Anemia
    8. Other labs
      1. G6PD Activity
      2. Radionuclide RBC survival study
      3. Hemoglobin electrophoresis
        1. Indicated for Sickle Cell Anemia or Thalassemia
      4. Infection evaluation (if fever or travel)
        1. Blood Cultures for Clostridium perfringens
        2. Babesia serology
        3. Blood smears for Malarial Hemoglobinuria
      5. Osmotic fragility with reflex to Band 3 Protein (much more sensitive than Spherocytosis)
        1. Available as panel from Mayo
        2. Identifies Hereditary Spherocytosis
  7. Management
    1. Treat underlying cause
    2. Corticosteroids (if not contraindicated)
    3. Splenectomy (refractory to steroids)
    4. IV Immunoglobulin
  8. Management
    1. Golan in Goldman (2000) Cecil Medicine, p. 867-84
    2. Dhaliwal (2004) Am Fam Physician 69:2599

Hemolytic disorder (C1263988)

ConceptsDisease or Syndrome (T047)
ICD9773.2
EnglishHaemolytic disease, Haemolytic disorder, Hemolytic disease, Hemolytic disorder
Spanishtrastorno hemolitico
Parent ConceptsRed blood cell disorder (C0221016), Hemolytic disorder (C1263988), Duplicate concept (C1274013)
SourcesMTHICD9, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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