Presenting manifestations of hemophagocytic syndrome in a male patient with systemic lupus erythematosus

Rheumatol Int. 2010 Jan;30(3):387-8. doi: 10.1007/s00296-009-0932-5. Epub 2009 Apr 19.

Abstract

Hemophagocytic syndrome (HPS) is an unusual but sometimes fatal disorder. We reported a case of 21-year-old man who developed HPS and SLE simultaneously. Febrile pancytopenia, hyperferritinemia, and abnormal liver function tests were observed. Hemophagocytic cells were observed by means of bone marrow biopsy and diagnosed as HPS. The patient was treated with high-dose prednisolone, resulting in an excellent outcome. Early diagnosis of HPS by bone marrow biopsy is important for the successful treatment.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Bone Marrow / pathology
  • Bone Marrow / physiopathology
  • Coma / etiology
  • Fever / etiology
  • Humans
  • Immunosuppressive Agents
  • Japan
  • Leukopenia / etiology
  • Lupus Erythematosus, Systemic / complications*
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / etiology*
  • Male
  • Methylprednisolone / therapeutic use
  • Mononuclear Phagocyte System / pathology
  • Mononuclear Phagocyte System / physiopathology
  • Prednisolone / therapeutic use
  • Treatment Outcome
  • Young Adult

Substances

  • Immunosuppressive Agents
  • Prednisolone
  • Methylprednisolone