Langerhans cell histiocytosis: clinical experience with 124 patients.

Abstract:

:We cared for 124 pediatric patients with a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory, and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltration. Liver disease was noted in 50% of patients and lung disease in 23%; hematologic changes were also frequent. Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All patients with generalized disease or organ dysfunction were treated with systemic chemotherapy. The actuarial survival curve at 10 years was 63%.

journal_name

Pediatr Dermatol

journal_title

Pediatric dermatology

authors

Rivera-Luna R,Martinez-Guerra G,Altamirano-Alvarez E,Martinez-Avalos A,Cardenas-Cardoz R,Ayon-Cardenas A,Ruiz-Maldonado R,Lopez-Corella E

doi

10.1111/j.1525-1470.1988.tb01160.x

subject

Has Abstract

pub_date

1988-08-01 00:00:00

pages

145-50

issue

3

eissn

0736-8046

issn

1525-1470

journal_volume

5

pub_type

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