Hemolysis

Paroxysmal Cold Hemoglobinuria

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Paroxysmal Cold Hemoglobinuria, Donath-Landsteiner Hemolytic Anemia

  • Pathophysiology
  1. Rare Immune Hemolytic Anemia
  2. Caused by complement fixing IgG autoantibody
    1. Activates classic complement pathway
  • Causes
  1. Idiopathic
  2. Tertiary Syphilis
  3. Acute Viral Infection
    1. Mumps
    2. Measles
  • Symptoms
  1. Exposure to cold classically precedes symptoms
  2. Headache
  3. Back pain and Leg Pain
  4. Abdominal cramps
  5. Vomiting and Diarrhea
  6. Fever and chills
  • Signs
  1. Splenomegaly
  2. Hepatomegaly
  3. Transient Jaundice
  4. Pale fingers, toes, and tip of nose
  • Labs
  1. Urinalysis
    1. Hemoglobin in fresh urine
    2. No urine RBCs present
  2. Direct Coombs
    1. Weakly positive for IgG and complement
  3. Donath-Landsteiner Cold Hemolysin Test
    1. Hemolysis activated by cold, present on rewarming
  • Management
  1. Mild single course
    1. Supportive therapy
    2. Protect from cold exposure
    3. Heat transfused blood or fluids to 37 degrees
  2. Chronic or recurrent course (rare)
    1. Glucocorticoids
    2. Immunosuppressive agents
  • Course
  1. Viral infection associated Hemolysis usually transient