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An unusual histiocytic disorder responding to cyclosporine therapy: a case report
P R Ogershok1, F G Keller, B E Favara
1West Virginia University School of Medicine, Morgantown 26506, USA.
Purpose:
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.
Conclusion:
This case appears to represent an atypical histiocytic disorder with unique clinical and pathologic features. The disorder resolved after the initiation of cyclosporine therapy.