AN UNUSUAL HISTIOCYTIC DISORDER RESPONDING TO CYCLOSPORINE THERAPY - A CASE-REPORT

Purpose: To describe the clinical and pathologic presentation and cour se of a 7-week-old girl with anemia, thrombocytopenia, and organomegal y who was found to have a histiocytic disorder distinct from previousl y reported cases. Methods: Bone marrow specimens were studied with con ventional methods. A liver biopsy specimen was evaluated by routine an d immunohistochemical methods and electron microscopy. Results: The pa tient was found to have a unique histiocytic disorder in which lesiona l cells displayed an atypical phenotype. Cyclosporine therapy was asso ciated with a prompt, complete, and apparently permanent resolution of disease.Conclusion: This case appears to represent an atypical histio cytic disorder with unique clinical and pathologic features. The disor der resolved after the initiation of cyclosporine therapy.