II. Pathophysiology
- Rare Immune Hemolytic Anemia
- Caused by complement fixing IgG autoantibody
- Activates classic Complement Pathway
III. Causes
- Idiopathic
- Tertiary Syphilis
- Acute Viral Infection
IV. Symptoms
V. Signs
- Splenomegaly
- Hepatomegaly
- Transient Jaundice
- Pale fingers, toes, and tip of nose
VI. Labs
-
Urinalysis
- Hemoglobin in fresh urine
- No Urine RBCs present
-
Direct Coombs
- Weakly positive for IgG and complement
- Donath-Landsteiner Cold Hemolysin Test
- Hemolysis activated by cold, present on rewarming
VII. Management
- Mild single course
- Supportive therapy
- Protect from cold exposure
- Heat transfused blood or fluids to 37 degrees
- Chronic or recurrent course (rare)
VIII. Course
- Viral Infection associated Hemolysis usually transient
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Related Studies
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 |