II. Pathophysiology

  1. Rare Immune Hemolytic Anemia
  2. Caused by complement fixing IgG autoantibody
    1. Activates classic complement pathway

III. Causes

  1. Idiopathic
  2. Tertiary Syphilis
  3. Acute Viral Infection
    1. Mumps
    2. Measles

IV. 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

V. Signs

  1. Splenomegaly
  2. Hepatomegaly
  3. Transient Jaundice
  4. Pale fingers, toes, and tip of nose

VI. 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

VII. 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

VIII. Course

  1. Viral infection associated Hemolysis usually transient

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Ontology: Cold paroxysmal hemoglobinuria (C0086774)

Concepts Disease or Syndrome (T047)
MSH D006457
ICD10 D59.1 , D59.6
SnomedCT 1468004, 191226006, 191224009, 127057004
English Cold Paroxysmal Hemoglobinuria, Paroxysmal Hemoglobinuria, Cold, Hemoglobinuria, Cold Paroxysmal, Cold haemoglobinuria, Don Lands autoim haemo anaem, PCH - Paroxys cold haemo, Paroxysm cold haemoglobinuria, Paroxysm cold hemoglobinuria, Hemoglobinuria, Paroxysmal Cold, Paroxysmal Cold Hemoglobinuria, hemolytic anemia due to paroxysmal cold hemoglobinuria, hemolytic anemia due to paroxysmal cold hemoglobinuria (diagnosis), hemolytic paroxysmal cold hemoglobinuria, paroxysmal cold hemoglobinuria, paroxysmal cold haemoglobinuria, cold hemoglobinuria, Cold Donath Landsteiner type autoimmune haemolytic anaemia (disorder), Paroxysmal cold hemoglobinuria, Cold agglutinin hemoglobinuria, Cold Donath Landsteiner type autoimmune haemolytic anaemia, Cold Donath Landsteiner type autoimmune hemolytic anemia, Cold hemoglobinuria, PCH - Paroxysmal cold haemoglobinuria, PCH - Paroxysmal cold hemoglobinuria, Paroxysmal cold haemoglobinuria, Cold agglutinin haemoglobinuria, Paroxysmal cold hemoglobinuria (disorder), Cold paroxysmal hemoglobinuria, cold; hemoglobinuria, hemoglobinuria; cold
Portuguese Hemoglobinúria Paroxística ao Frio
Spanish Hemoglobinuria Paroxística a Frigore, hemoglobinuria paroxística inducida por frío (trastorno), hemoglobinuria paroxística inducida por frío
French Hémoglobinurie paroxystique a frigore, HPF (Hémoglobinurie Paroxystique a Frigore), Maladie de Harley, Hémoglobinurie paroxystique essentielle
German Paroxysmale Kältehämoglobinurie
Norwegian Paroksysmal kuldehemoglobinuri
Dutch hemoglobinurie; koude, koude; hemoglobinurie