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      Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy

      case-report

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          Abstract

          We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed multiple thin‐walled cavities. Transbronchial lung cryobiopsy (TBLC) specimens revealed Langerhans cells, which were positive for CD1a and S‐100 expression. Similar histological findings were detected in the submandibular gland, skin, and tooth. On the basis of these findings, the patient was diagnosed with MS‐LCH and subsequently treated with steroid therapy. From the literature review, case reports of non‐smokers with pulmonary lesions that worsened and required treatment are rare. Almost all cases recurred and needed additional treatment. This case study contributes to our understanding of the potential role of steroid therapy in MS‐LCH treatment. Additionally, TBLC is a novel, potentially safer, diagnostic tool that has not been previously described for LCH.

          Abstract

          Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy for a subacute exacerbation: a case report and review of the literature.

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          Adult pulmonary Langerhans' cell histiocytosis.

          M Tazi (2006)
          Adult pulmonary Langerhans' cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20-40 yrs of age. In adults, pulmonary involvement with Langerhans' cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans' cell granulomas infiltrating and destroying distal bronchioles. High-resolution computed tomography (HRCT) of the chest is essential to the diagnosis, typically showing a combination of nodules, cavitated nodules, and thick- and thin-walled cysts. A high macrophage count in bronchoalveolar lavage (BAL) fluid is a common but nonspecific finding that merely reflects exposure to tobacco smoke. BAL is useful for eliminating infections and the other infiltrating lung disorders that can be seen in young adults. Langerhans' cells can be identified in BAL fluid, but, in contrast to what was initially hoped, this test shows a very low sensitivity and is rarely useful in the diagnosis of the disease. The definite diagnosis of pulmonary Langerhans' cell histiocytosis requires identification of Langerhans' cell granulomas, which is usually achieved by surgical lung biopsy at a site selected by chest HRCT. In practice, however, lung biopsy is performed on a case-by-case basis. No effective treatment is available to date, and improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans' cell histiocytosis is urgently needed, and should help in the development of specific therapeutic strategies for patients with this orphan disease.
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            The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study

            Abdellatif Tazi, Constance de Margerie-Mellon, Jean Marc Naccache … (2015)
            Background The natural history of pulmonary Langerhans cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported. Methods In this prospective, multicentre study, 58 consecutive patients with newly diagnosed PLCH were comprehensively evaluated over a two-year period. Our objectives were to estimate the incidence of early progression of the disease and to evaluate the impact of smoking status on lung function outcomes. Lung function deterioration was defined as a decrease of at least 15% in FEV1 and/or FVC and/or DLCO, compared with baseline values. At each visit, smoking status was recorded based on the patients’ self-reports and urinary cotinine measurements that were blinded for the patients. The cumulative incidence of lung function outcomes over time was estimated using the non-parametric Kaplan-Meier method. Multivariate Cox models with time-dependent covariates were used to calculate the hazards ratios of the lung function deterioration associated with smoking status with adjustment for potential confounders. Results The cumulative incidence of lung function deterioration at 24 months was 38% (22% for FEV1 and DLCO, and 9% for FVC). In the multivariate analysis, smoking status and PaO2 at inclusion were the only factors associated with the risk of lung function deterioration. The patients’ smoking statuses markedly changed over time. Only 20% of the patients quit using tobacco for the entire study period. Nevertheless, being a non-smoker was associated with a decreased risk of subsequent lung function deterioration, even after adjustment for baseline predictive factors. By serial lung computed tomography, the extent of cystic lesions increased in only 11% of patients. Conclusions Serial lung function evaluation on a three- to six-month basis is essential for the follow-up of patients with recently diagnosed PLCH to identify those who experience an early progression of their disease. These patients are highly addicted to tobacco, and robust efforts should be undertaken to include them in smoking cessation programs. Trial registration ClinicalTrials.gov: No: NCT01225601. Electronic supplementary material The online version of this article (doi:10.1186/s13023-015-0249-2) contains supplementary material, which is available to authorized users.
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              Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis

              Vincent Grobost, Chahera Khouatra, Romain Lazor … (2014)
              Background Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte proliferation and has been successful in curbing multi-system Langerhans cell histiocytosis and isolated PLCH. Methods and patients We retrospectively studied 5 patients (aged 37–55 years, 3 females) with PLCH who received 3 to 4 courses of cladribine therapy as a single agent (0.1 mg/kg per day for 5 consecutive days at monthly intervals). One patient was treated twice because of relapse at 1 year. Progressive pulmonary disease with obstructive ventilatory pattern despite smoking cessation and/or corticosteroid therapy were indications for treatment. Patients were administered oral trimethoprim/sulfamethoxazole and valaciclovir to prevent opportunistic infections. They gave written consent to receive off-label cladribine in the absence of validated treatment. Results Functional class dyspnea improved with cladribine therapy in 4 out of 5 cases, and forced expiratory volume in 1 second (FEV1) increased in all cases by a mean of 387 ml (100–920 ml), contrasting with a steady decline prior to treatment. Chest high-resolution computed tomography (HRCT) features improved with cladribine therapy in 4 patients. Hemodynamic improvement was observed in 1 patient with pre-capillary pulmonary hypertension. The results suggested a greater treatment effect in subjects with nodular lung lesions and/or thick-walled cysts on chest HRCT, with diffuse hypermetabolism of lung lesions on positron emission tomography (PET)-scan, and with progressive disease despite smoking cessation. Infectious pneumonia developed in 1 patient, with later grade 4 neutrocytopenia but without infection. Discussion Data interpretation was limited by the retrospective, uncontrolled study design and small sample size. Conclusion Cladribine as a single agent may be effective therapy in patients with progressive PLCH.
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                Author and article information

                Contributors
                Haruka Ito:
                ORCID: https://orcid.org/0000-0003-4476-3088
                haruka_k_0706@yahoo.co.jp
                Journal
                Journal ID (nlm-ta): Respirol Case Rep
                Journal ID (iso-abbrev): Respirol Case Rep
                Journal ID (doi): 10.1002/(ISSN)2051-3380
                Journal ID (publisher-id): RCR2
                Title: Respirology Case Reports
                Publisher: John Wiley & Sons, Ltd (Chichester, UK )
                ISSN (Electronic): 2051-3380
                Publication date (Electronic): 12 June 2020
                Publication date Collection: August 2020
                Volume: 8
                Issue: 6 ( doiID: 10.1002/rcr2.v8.6 )
                Electronic Location Identifier: e00603
                Affiliations
                [ 1 ] Department of Respiratory Medicine Yokohama Rosai Hospital Yokohama Kanagawa Japan
                [ 2 ] Department of Pathology Yokohama Rosai Hospital Yokohama Kanagawa Japan
                [ 3 ] Department of Pulmonology Yokohama City University Graduate School of Medicine Yokohama Kanagawa Japan
                [ 4 ] Department of Pathology Kanagawa Cardiovascular and Respiratory Center Yokohama Kanagawa Japan
                [ 5 ] Department of Respiratory Medicine Kanagawa Cardiovascular and Respiratory Center Yokohama Kanagawa Japan
                Author notes
                [*] [* ] Correspondence

                Haruka Ito, Department of Respiratory Medicine, Yokohama Rosai Hospital, 3211 Kodukue‐cho, Kouhoku‐ku, Yokohama‐shi, Kanagawa 216‐0033, Japan. E‐mail: haruka_k_0706@ 123456yahoo.co.jp

                Author information
                https://orcid.org/0000-0003-4476-3088
                Article
                Publisher ID: RCR2603
                DOI: 10.1002/rcr2.603
                PMC ID: 7290287
                PubMed ID: 32547768
                SO-VID: 637f9d24-a04e-4960-90e4-e4c98b4d5d64
                Copyright © © 2020 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 25 December 2019
                Date revision received : 11 April 2020
                Date accepted : 29 May 2020
                Page count
                Figures: 2, Tables: 1, Pages: 4, Words: 1857
                Categories
                Subject: Case Report
                Subject: Case Reports
                Custom metadata
                source-schema-version-number 2.0
                cover-date August 2020
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:5.8.4 mode:remove_FC converted:12.06.2020

                Keywords: corticosteroid therapy,langerhans cell histiocytosis,non‐smoker,transbronchial lung cryobiopsy
                Data availability:
                Keywords: corticosteroid therapy, langerhans cell histiocytosis, non‐smoker, transbronchial lung cryobiopsy

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