Case Report
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World J Hematol. Aug 6, 2013; 2(3): 89-98
Published online Aug 6, 2013. doi: 10.5315/wjh.v2.i3.89
Management of adult Langerhans cell histiocytosis based on the characteristic clinical features
Shinsaku Imashuku, Chihiro Shimazaki, Arinobu Tojo, Toshihiko Imamura, Akira Morimoto
Shinsaku Imashuku, Division of Hematology, Takasago-seibu Hospital, Takasago 676-0812, Japan
Shinsaku Imashuku, the NPO for Japan Langerhans Cell Histiocytosis Study Group, Kyoto 602-8566, Japan
Chihiro Shimazaki, Division of Hematology, Social Insurance Kyoto Hospital, Kyoto 603-8151, Japan
Arinobu Tojo, Division of Stem Cell Therapy, Center for Stem Cell Biology and Regenerative Medicine, the Institute of Medical Science, the University of Tokyo, Tokyo 108-8639, Japan
Toshihiko Imamura, Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto 602-8566, Japan
Akira Morimoto, Department of Pediatrics, Jichi Medical University School of Medicine, Shimotsuke 329-0498, Japan
Author contributions: All authors engaged in part in consulting and providing treatment for the patients presented; Imashuku S and Tojo A designed; Imashuku S wrote the paper; Shimazaki C, Imamura T and Morimoto A provided constructive insights in preparation of the paper; all authors approved the final version of the manuscript.
Correspondence to: Shinsaku Imashuku, MD, Consultant, Division of Hematology, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago 676-0812, Japan. shinim95@mbox.kyoto-inet.or.jp
Telephone: +81-79-4470100 Fax: +81-79-4470180
Received: April 11, 2013
Revised: May 10, 2013
Accepted: June 5, 2013
Published online: August 6, 2013
Abstract

To find out the most appropriate management, clinical features of 18 cases of adult multisystem langerhans cell histiocytosis (LCH) have been analyzed. The patients comprising of 9 males and 9 females were median age of 36 years, ranging from 18-53 years at diagnosis. Regarding the initial symptoms, 7 patients (2 males and 5 females) showed central diabetes insipidus (CDI) and other endocrine symptoms with thickened pituitary stalk or a mass at the hypothalamic region. Additional 2 patients initiated the disease with CDI with no immediate diagnosis. In the remaining patients, the disease begun with single (n = 3) or multiple (n = 1) spinal bone lesion(s) in 4 patients (all males), with multiple bone lesions in 3 patients (1 male and 2 females), with single skull lesion in one female patient and with ambiguous symptoms including hypothyroidism in the remaining one male patient. We also recognized the correlation between pregnancy/childbirth and LCH in 4 patients. In terms of treatment, 9 patients received systemic immuno-chemotherapy alone, of which the majority received vinblastine-based chemotherapy while 4 received 2-chlorodeoxyadenosine. Five had a combination of immuno-chemotherapy with surgical resection or radiotherapy, 2 had immunotherapy alone, 2 had surgical resection followed by observation alone to date. Three patients received hematopoietic stem cell transplantation after extensive chemotherapy. In terms of outcome, 15 patients are alive (9 with active disease, 6 without active disease), with a median of 66 mo (range 17-166 mo), two died of disease while the remaining 1 lost to follow-up. Based on these results, we think that early diagnosis and rapid introduction of appropriate treatment are essential, in order to overcome the problems relevant to adult LCH.

Keywords: Langerhans cell histiocytosis, Adult, Immuno-chemotherapy, 2-chlorodeoxyadenosine, Childbirth

Core tip: Clinical features and treatment in a total of 18 adult patients with langerhans cell histiocytosis (LCH) were reviewed. We found two major groups regarding the initial symptoms; one was central diabetes insipidus and other endocrine symptoms (n = 9) and the other bone diseases (n = 8; 1 skull, 4 spinal and 3 multiple). We also recognized the correlation between pregnancy/childbirth and LCH in 4 patients. Based on the clinical features and outcomes, early diagnosis and rapid introduction of appropriate treatment are essential, in order to overcome the problems relevant to adult LCH.