Langerhans Cell Histiocytosis: Clinical Experience with 124 Patients (original) (raw)

Pediatric Dermatology, 1988

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 infiltralion. Liver disease was noted in 50% of patients and lung disease in 23% he‐matologic 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 palienis with generalized disease or organ dysfunclion were Lrealed with systemie chemotherapy. The actuarial survival curve at 10 Years was 63%

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