An unusual histiocytic disorder responding to cyclosporine therapy: a case report.
OBJECTIVE
To describe the clinical and pathologic presentation and course of a 7-week-old girl with anemia, thrombocytopenia, and organomegaly who was found to have a histiocytic disorder distinct from previously reported cases.


METHODS
Bone marrow specimens were studied with conventional methods. A liver biopsy specimen was evaluated by routine and immunohistochemical methods and electron microscopy.


RESULTS
The patient was found to have a unique histiocytic disorder in which lesional cells displayed an atypical phenotype. Cyclosporine therapy was associated with a prompt, complete, and apparently permanent resolution of disease.


CONCLUSIONS
This case appears to represent an atypical histiocytic disorder with unique clinical and pathologic features. The disorder resolved after the initiation of cyclosporine therapy.
West Virginia University School of Medicine, Morgantown 26506, USA.
P R
PR
Ogershok
F G
FG
Keller
B E
BE
Favara
A K
AK
Ritchey
D000740:Anemia / Q000209:etiology; D017951:Antigen Presentation / Q000187:drug effects; D015415:Biomarkers; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D016572:Cyclosporine / Q000494:pharmacology / Q000627:therapeutic use*; D003713:Dendritic Cells / Q000473:pathology; D005260:Female; D005334:Fever / Q000209:etiology; D006529:Hepatomegaly / Q000209:etiology* / Q000473:pathology; D015614:Histiocytosis / Q000097:blood / Q000145:classification / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008099:Liver / Q000473:pathology; D008264:Macrophages / Q000473:pathology; D011693:Purpura / Q000209:etiology; D009418:S100 Proteins / Q000032:analysis; D013921:Thrombocytopenia / Q000209:etiology*; D011503:Transglutaminases / Q000032:analysis
D015415:Biomarkers; D007166:Immunosuppressive Agents; D009418:S100 Proteins; D016572:Cyclosporine; D011503:Transglutaminases