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Paroxysmal Cold Hemoglobinuria
Aka: Paroxysmal Cold Hemoglobinuria, Donath-Landsteiner Hemolytic Anemia
- See Also
- Autoimmune Hemolytic Anemia
- Pathophysiology
- Rare Immune Hemolytic Anemia
- Caused by complement fixing IgG autoantibody
- Activates classic Complement Pathway
- Causes
- Idiopathic
- Tertiary Syphilis
- Acute Viral Infection
- Mumps
- Measles
- Symptoms
- Exposure to cold classically precedes symptoms
- Headache
- Back pain and Leg Pain
- Abdominal cramps
- Vomiting and Diarrhea
- Fever and chills
- Signs
- Splenomegaly
- Hepatomegaly
- Transient Jaundice
- Pale fingers, toes, and tip of nose
- 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
- Management
- Mild single course
- Supportive therapy
- Protect from cold exposure
- Heat transfused blood or fluids to 37 degrees
- Chronic or recurrent course (rare)
- Glucocorticoids
- Immunosuppressive agents
- Course
- Viral infection associated Hemolysis usually transient