Document Detail

Malignant histiocytosis X. A distinct clinicopathologic entity.
MedLine Citation:
PMID:  1913475     Owner:  NLM     Status:  MEDLINE    
Histiocytosis X (HX) is characterized morphologically by a proliferation of Langerhans' cells (LC), and most often has an indolent, chronic course. To determine whether a distinct clinicopathologic entity of malignant histiocytosis X exists, the authors examined tissues from 31 patients with HX and divided them into four categories. Group A (19 patients) was characterized morphologically by benign-appearing LC and had an indolent course. The male:female (M:F) ratio was 10:9, and the mean age was 21 years (range, 2 months to 60 years). The immunophenotype of this group was S-100+, vimentin+, LN-2+, LN-3+, lysozyme-, LCA-, Leu-M1-. Group B (three patients) had benign-appearing LC, yet had an aggressive clinical course. All patients were male, with a mean age of 47 years (range, 3 years to 72 years). Organs involved included the liver, spleen, heart, thymus, lung, kidney, and pancreas. The immunophenotype was the same as for Group A. Group C (two patients) had atypical and malignant appearing LC, yet a relatively benign clinical course. The ages were four and 65 years, with one female and one male patient. In both patients, the cells were S-100+, vimentin+, LN-2+, LN-3+, and LCA-. Group D (seven patients) was characterized by atypical and malignant-appearing LC and an aggressive clinical course. The mean age was 25 years (range, congenital to 54 years) with one female and six male patients. Organs involved were the thymus, lungs, spleen, liver, kidney, brain, heart, pancreas, stomach, and muscle. Birbeck granules were found in two patients, and the one patient on which fresh tissue was available was CD1+. The typical immunophenotype was S-100+, vimentin+, LN-2+, LN-3+, Leu-M1-, lysozyme-. The results of our study indicate that (1) a distinct clinical entity of malignant HX, characterized morphologically by malignant-appearing LC and clinically by male predominance, atypical organ involvement, and an aggressive clinical course, does exist; and (2) the morphologic appearance of the LC is an imperfect predictor of the clinical severity of HX.
J Ben-Ezra; A Bailey; N Azumi; G Delsol; R Stroup; K Sheibani; H Rappaport
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Cancer     Volume:  68     ISSN:  0008-543X     ISO Abbreviation:  Cancer     Publication Date:  1991 Sep 
Date Detail:
Created Date:  1991-11-12     Completed Date:  1991-11-12     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0374236     Medline TA:  Cancer     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1050-60     Citation Subset:  AIM; IM    
James Irvine Center for the Study of Leukemia and Lymphoma, Department of Pathology, City of Hope National Medical Center, Duarte, California 91010.
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MeSH Terms
Child, Preschool
Histiocytic Sarcoma / immunology,  pathology*
Histiocytosis, Langerhans-Cell / immunology,  pathology*
Langerhans Cells / pathology
Middle Aged
Grant Support

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