Abstract
Autoimmune diseases are rare in patients with severe combined immunodeficiency (SCID). The authors describe an 11-month-old infant girl with SCID with fatal warm autoimmune hemolytic anemia (AIHA) resulting from IgM autoagglutinins. Serologic evaluation revealed IgM autoantibodies that caused in vitro hemagglutination at 37°C. The patient had clinical evidence of ongoing hemolysis and agglutination despite aggressive treatment. She had three strokes and died 6 weeks after unsuccessful bone marrow transplantation. Autoimmune disease is an unexpected complication of SCID. The presence of warm reactive IgM autoagglutinins in AIHA confers a dismal prognosis.
Original language | English (US) |
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Pages (from-to) | 250-252 |
Number of pages | 3 |
Journal | American Journal of Pediatric Hematology/Oncology |
Volume | 23 |
Issue number | 4 |
DOIs | |
State | Published - 2001 |
Externally published | Yes |
Keywords
- Autoimmune hemolytic anemia
- In vivo hemagglutination
- Severe combined immunodeficiency
- Warm reactive IgM antibody
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology