Severe Autoimmune Hemolytic Anemia After Unrelated Umbilical Cord Blood Transplant for Familial Hemophagocytic Lymphohistiocytosis: Significant Improvement After Treatment With Rituximab

J Pediatr Hematol Oncol. 2007 Feb;29(2):125-7. doi: 10.1097/MPH.0b013e3180320b23.


A 4-month-old girl diagnosed with familial hemophagocytic lymphohistiocytosis underwent a matched unrelated, umbilical cord blood transplant. Six weeks later she developed severe acute autoimmune hemolytic anemia and thrombocytopenia requiring multiple transfusions. This was refractory to high-dose steroid and intravenous immunoglobulin, but did respond to Rituximab (anti-CD20 monoclonal antibody) 375 mg/m2. Hemolysis recurred after steroid tapering but responded to a second course of Rituximab. This case report highlights the difficulty in managing posttransplant autoimmune hemolytic anemia.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anemia, Hemolytic, Autoimmune / drug therapy*
  • Anemia, Hemolytic, Autoimmune / etiology*
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Cord Blood Stem Cell Transplantation / adverse effects*
  • Female
  • Fetal Blood / transplantation
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use*
  • Infant
  • Lymphohistiocytosis, Hemophagocytic / therapy*
  • Rituximab
  • Thrombocytopenia / drug therapy
  • Thrombocytopenia / etiology


  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Rituximab