Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen

Pediatr Hematol Oncol. 1986;3(2):135-42. doi: 10.3109/08880018609031209.

Abstract

We report the case history of a 6 1/2-month-old girl with a hemophagocytic syndrome, pancytopenia, and excessive hepatosplenomegaly. Some extraordinary histological features present in this case--restricted organ involvement, excessive hemosiderosis, and fibrosis of the spleen--further contributed to the well-known problem of distinguishing between infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Diagnostic Errors
  • Female
  • Fibrosis
  • Hemosiderosis / etiology*
  • Histiocytosis / classification
  • Histiocytosis / diagnosis
  • Histiocytosis / genetics
  • Histiocytosis, Non-Langerhans-Cell / complications
  • Histiocytosis, Non-Langerhans-Cell / diagnosis*
  • Histiocytosis, Non-Langerhans-Cell / pathology
  • Humans
  • Infant
  • Respiratory Tract Infections / complications
  • Splenic Diseases / etiology*
  • Splenic Diseases / pathology
  • Thymus Gland / pathology