Adult Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. The clinical presentation of LCH is highly variable. Although the features of this disease have been well described in children, they remain poorly defined in adults. Here, we review the current knowledge about adult LCH, focussing on clinical presentation, diagnosis, treatment, and prognosis.

Cladribine (2-chlorodeoxyadenosine) in frontline chemotherapy for adult Langerhans cell histiocytosis: A single-center study of seven cases

“…Seven males, from 21 to 46 years of age at the time of LCH diagnosis (35 Ϯ 8.5 [7]), were included in this study. Six patients had multisystem disease and one patient (Case 2) was diagnosed with multifocal bone LCH (Table I, Figure 2).…”

“…Cladribine was administered as subcutaneous injection (fi ve patients) or by two-hour intravenous infusion (two patients) at a dose of 5 mg/m 2 over fi ve consecutive days in four week intervals for a median of four cycles (range 4 -6 cycles; 4.9 Ϯ 1.1 [7]). In two patients, aggressive disease courses (rapid progression, excessive disease extent or presence of B-symptoms) were observed making them potential candidates for high-dose chemotherapy with autologous peripheral blood stem cell transplantation.…”

“…7 -8.4; 6.9 Ϯ 1.4 [7]), 147 g/l (range 125 -160; 143.9 Ϯ 12.8 [7]) and 247 ϫ 10 9 cells/l (range 217 -589; 313.6 Ϯ 131.3 [7]), respectively. The median leukocyte count, hemoglobin level and thrombocyte count at the beginning of the last therapy cycle were 5.7 ϫ 10 9 cells/l (range 3.8 -6.7; 5.6 Ϯ 1.0 [7]), 142 g/l (range 129 -166; 144.6 Ϯ 15.4 [7]) and 204 ϫ 10 9 cells/l (range 134 -346; 211.…”

“…For multisystem disease various treatment regimens including glucocorticoids, immunomodulation, chemotherapy, radiotherapy and peripheral blood stem cell transplantation have been proposed but the lack of controlled clinical trials for adult patients proved to be limiting in making fi rm therapy recommendations [7]. Therefore, therapeutic protocols are often derived from experience with pediatric LCH.…”

Langerhans Cell Histiocytosis Presenting as Brown Lichenoid Patches

“…Single system-patients should receive prednisone and vinblastine for 6 wk followed by continuation treatment with 6-mercaptopurine, prednisone, and vinblastine for 6 months. Patients with multisystem disease should receive prednisone and vinblastine for 6 wk followed by continuation treatment with 6-mercaptopurine, prednisone, and vinblastine for 6 or 12 months, respectively 20 .…”