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Malignant histiocytosis X. A distinct clinicopathologic entity.
J Ben-Ezra1, A Bailey, N Azumi
1James Irvine Center for the Study of Leukemia and Lymphoma, Department of Pathology, City of Hope National Medical Center, Duarte, California 91010.
Cancer
|September 1, 1991
Summary
Malignant Histiocytosis X (HX) is a distinct clinicopathologic entity. Morphologically malignant Langerhans
Area of Science:
- Pathology
- Oncology
- Immunology
Background:
- Histiocytosis X (HX) is a condition characterized by Langerhans' cell (LC) proliferation, typically with an indolent course.
- The existence of a distinct malignant variant of HX requires further clinicopathologic investigation.
Purpose of the Study:
- To determine if a distinct clinicopathologic entity of malignant Histiocytosis X exists.
- To correlate morphologic LC appearance with clinical severity and patient outcomes.
Main Methods:
- Examination of tissues from 31 patients diagnosed with HX.
- Categorization of patients into four groups based on LC morphology and clinical course.
- Immunophenotypic analysis of LC using various markers (S-100, vimentin, LN-2, LN-3, LCA, Leu-M1, lysozyme, CD1).
Main Results:
- A distinct clinicopathologic entity of malignant HX was identified, characterized by atypical LC morphology, male predominance, atypical organ involvement, and aggressive clinical course.
- Morphologic appearance of LC was found to be an imperfect predictor of clinical severity.
- Four distinct groups were identified, differing in LC morphology, clinical aggressiveness, and immunophenotype.
Conclusions:
- A distinct malignant form of Histiocytosis X exists, with specific clinical and morphologic features.
- Langerhans' cell morphology alone is insufficient to predict the clinical behavior of Histiocytosis X.