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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.
Insights
A rare histiocytic disorder in an infant presented with anemia and organomegaly. Cyclosporine therapy led to complete resolution, suggesting a novel treatment approach for this unique condition.
Area of Science:
- Pediatric Hematology
- Histiocytic Disorders
- Immunology
Background:
- Histiocytic disorders are a heterogeneous group of diseases characterized by the proliferation of cells of monocyte-macrophage lineage.
- Accurate diagnosis and classification are crucial for appropriate management and prognosis.
Observation:
- A 7-week-old female infant presented with anemia, thrombocytopenia, and organomegaly.
- Bone marrow and liver biopsy revealed a unique histiocytic disorder with atypical lesional cell phenotype.
Findings:
- The patient's condition showed a prompt, complete, and sustained resolution following treatment with cyclosporine.
- This suggests cyclosporine may be an effective therapeutic agent for this specific atypical histiocytic disorder.
Implications:
- This case highlights an atypical histiocytic disorder with distinct clinical and pathological features.
- Successful cyclosporine treatment offers a potential new therapeutic strategy for similar rare conditions.
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.