Fatal Evans' syndrome after matched unrelated donor transplantation for hyper-IgM syndrome

Eur J Haematol. 2004 Jun;72(6):444-7. doi: 10.1111/j.1600-0609.2004.00256.x.

Abstract

A 3 and 1/2-yr-old boy underwent matched unrelated stem cell transplantation (SCT) for hyper-IgM syndrome. He developed acute and chronic skin graft-vs.-host disease (GVHD). Ten months following SCT he presented with severe hemolytic anemia and thrombocytopenia (Evans' syndrome). Treatment included high-dose steroids, intravenous immunoglobulins, cyclosporine, mycophenolate mofetil, chemotherapeutic agents (cyclophosphamide, vincristine, VP-16), immunoadsorption, and anti-CD20 and anti-CD52 monoclonal antibodies without response. The patient died 16 months after SCT.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Hemolytic, Autoimmune / drug therapy
  • Anemia, Hemolytic, Autoimmune / etiology*
  • Blood Donors
  • Fatal Outcome
  • Graft vs Host Disease / etiology
  • Hematopoietic Stem Cell Transplantation / adverse effects*
  • Humans
  • Immunoglobulin M*
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / therapy
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Male
  • Skin Diseases / etiology
  • Skin Diseases / immunology
  • Syndrome
  • Thrombocytopenia / drug therapy
  • Thrombocytopenia / etiology*
  • Thrombocytopenia / immunology
  • Treatment Failure

Substances

  • Immunoglobulin M
  • Immunosuppressive Agents