Journal of Hematology & Oncology (Sep 2009)

Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report

  • Kuwana Masataka,
  • Oyamada Takashi,
  • Yoshihara Takao,
  • Ishida Hiroyuki,
  • Yoshida Hideki,
  • Imamura Toshihiko,
  • Morimoto Akira

DOI
https://doi.org/10.1186/1756-8722-2-40
Journal volume & issue
Vol. 2, no. 1
p. 40

Abstract

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Abstract Hereditary spherocytosis (HS) is a genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice and splenomegaly. Evans' syndrome is a clinical syndrome characterized by autoimmune hemolytic anemia (AIHA) accompanied by immune thrombocytopenic purpura (ITP). It results from a malfunction of the immune system that produces multiple autoantibodies targeting at least red blood cells and platelets. HS and Evans' syndrome have different mechanisms of pathophysiology one another. We reported the quite rare case of an infant who had these diseases concurrently. Possible explanations of the unexpected complication are discussed.