title: Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen.
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.
authors.affiliation: St. Anna Kinderspital, Vienna, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Zoubek
authors.forename: O A
authors.initials: OA
authors.lastname: Haas
authors.forename: R
authors.initials: R
authors.lastname: Ladenstein
authors.forename: H
authors.initials: H
authors.lastname: Gadner
authors.forename: T
authors.initials: T
authors.lastname: Radaszkiewicz
mesh_terms: D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D005260:Female; D005355:Fibrosis; D006486:Hemosiderosis / Q000209:etiology*; D015614:Histiocytosis / Q000145:classification / Q000175:diagnosis / Q000235:genetics; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D007223:Infant; D012141:Respiratory Tract Infections / Q000150:complications; D013158:Splenic Diseases / Q000209:etiology* / Q000473:pathology; D013950:Thymus Gland / Q000473:pathology