Hemophagocytic syndrome with restricted organ involvement: excessive hemosiderosis and fibrosis of the spleen.
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.
Zoubek
A
A
St. Anna Kinderspital, Vienna, Austria.
Haas
O A
OA
Ladenstein
R
R
Gadner
H
H
Radaszkiewicz
T
T
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