II. Types

  1. Extravascular Hemolysis (most common)
    1. Splenic and hepatic clearance of defective RBCs
  2. Intravascular Hemolysis
    1. RBC membrane injury (Trauma, autoimmune, infection)

IV. Symptoms

  1. See Anemia
  2. Fatigue
  3. Dyspnea on exertion
  4. Back pain
  5. Fatigue
  6. Dark Red Urine

V. Signs

VI. 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

VII. Management

  1. Treat underlying cause
  2. Corticosteroids (if not contraindicated)
  3. Splenectomy (refractory to steroids)
  4. IV Immunoglobulin

VIII. Management

  1. Golan in Goldman (2000) Cecil Medicine, p. 867-84
  2. Dhaliwal (2004) Am Fam Physician 69:2599-606 [PubMed]

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Ontology: Hemolytic disorder (C1263988)

Concepts Disease or Syndrome (T047)
SnomedCT 157130001, 268878003, 157133004, 288278000, 128086004
English Haemolytic disease NOS, diseases haemolytic, hemolytic disorder, disorders haemolytic, disease hemolytic, hemolytic disease, haemolytic disease, Hemolytic disease, Haemolytic disease, Haemolytic disease NOS (disorder), Hemolytic disease NOS (disorder), Haemolytic disorder, Hemolytic disorder (disorder), Hemolytic disorder, disease (or disorder); hemolytic, Hemolytic disease NOS
Spanish enfermedad hemolítica, SAI (trastorno), Hemolytic disease NOS, Haemolytic disease NOS, enfermedad hemolítica, SAI, trastorno hemolítico (trastorno), trastorno hemolítico
Dutch aandoening; hemolytisch