Fatal warm autoimmune hemolytic anemia resulting from IgM autoagglutinins in an infant with severe combined immunodeficiency

Anna Nowak-Wegrzyn, Karen E. King, R. Sue Shirey, Allen R. Chen, Colleen McDonough, Howard M. Lederman

Research output: Contribution to journalArticle

22 Scopus citations

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 languageEnglish (US)
Pages (from-to)250-252
Number of pages3
JournalAmerican Journal of Pediatric Hematology/Oncology
Volume23
Issue number4
DOIs
StatePublished - Jan 1 2001
Externally publishedYes

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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

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