A rare case of adult pulmonary Langerhans' cell histiocytosis

Keerthi A Prakash; Ajay Narasimhan; Sivanthi Sapna

doi:10.4103/japt.japt_29_22

CASE REPORT

Year : 2022 | Volume : 5 | Issue : 2 | Page : 77-79

A rare case of adult pulmonary Langerhans' cell histiocytosis

Keerthi A Prakash¹, Ajay Narasimhan², Sivanthi Sapna¹
¹ Department of Pulmonology, Apollo Hospitals, Chennai, Tamil Nadu, India
² Department of Cardiothoracic Surgery, Apollo Hospitals, Chennai, Tamil Nadu, India

Date of Submission	28-Aug-2022
Date of Acceptance	28-Sep-2022
Date of Web Publication	23-Dec-2022

Correspondence Address:
Dr. Keerthi A Prakash
Department of Pulmonology, Apollo Hospitals, Greams Road, Chennai - 600 006, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/japt.japt_29_22

Abstract

The most common organ affected in smokers is lungs. The usual presentations are obstructive airway disease, interstitial lung disease, and malignancy. Rarely, some individuals with specific genomic characteristics may develop pulmonary Langerhans' cell histiocytosis, which commonly presents as bilateral upper lobe predominant cystic lung disease. The main array of treatment is cessation of smoking.

Keywords: Cystic lung disease, ILD, pulmonary Langerhans' cell histiocytosis, smoker

How to cite this article:
Prakash KA, Narasimhan A, Sapna S. A rare case of adult pulmonary Langerhans' cell histiocytosis. J Assoc Pulmonologist Tamilnadu 2022;5:77-9

How to cite this URL:
Prakash KA, Narasimhan A, Sapna S. A rare case of adult pulmonary Langerhans' cell histiocytosis. J Assoc Pulmonologist Tamilnadu [serial online] 2022 [cited 2023 Jan 27];5:77-9. Available from: http://www.japt.com/text.asp?2022/5/2/77/365083

Introduction

Langerhans' cell histiocytosis is characterized by infiltration of the tissues by Langerhans' cells which may organize into granulomas. It can involve lung, bone, or skin.

The classification of Langerhans' cell histiocytosis (LCH) clinical patterns by the Histiocyte Society is based depending on the number of organs involved:

Acute disseminated LCH (Letterer–Siwe disease) is a multisystemic disease that is seen in young children and it carries a poor prognosis^{[1],[2],[3],[4]}
Multifocal LCH is seen mainly in older children and adolescents (Hand–Schuller–Christian syndrome or multifocal eosinophilic granuloma) and has a varied presentation and it follows a favorable prognosis^{[1],[2],[3],[4]}
Single-system disease (eosinophilic granuloma and primary pulmonary histiocytosis) is characterized by single-organ affection and has a good prognosis.^{[1],[2],[3],[4]}

Pulmonary Langerhans' cell histiocytosis (PLCH) is uncommon (0.27 per 100,000) that occurs almost exclusively in smokers.^[5] Here, we present a rare case of isolated pulmonary LCH.

Case Report

A 46-year-old man with no comorbidities came to our outpatient department with the complaints of progressive dyspnea of Grade III mMRC and nonproductive cough for the past 6 months. He had no history of wheeze or features of connective tissue disorder. He had no history of fever or prior antituberculosis treatment. He was a chronic smoker with 26 pack-years.

On examination, he was not tachypnoeic. Clubbing was absent. His SpO2 at room air was 96%. On auscultation, bilateral crackles heard all over the lung area. Cardio vascular system examination: S1, S2 heard. No loud P2 heard.

Pulmonary function test showed a mixed obstructive and restrictive pattern. Diffusing capacity of the lung for carbon monoxide was disproportionately reduced. Six-minute walk test showed desaturation to 91% with 265 m over 6 min.

Echo showed mild pulmonary arterial hypertension with preserved left ventricular function.

Computed tomography (CT) chest showed bilateral multiple thin-walled bizarre cysts with upper lobe predominant [Figure 1], preserved lung volume, and subpleural sparing [Figure 2].

Figure 1: Bilateral multiple thin walled bizarre cysts with upper lobe predominant

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Figure 2: Multiple thin walled bizarre cysts with preserved lung volume, and subpleural sparing

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Langerhans' cell histiocytosis was suspected. No evidence of other system involvement was seen. Uniport video-assisted thoracoscopic surgery right lung biopsy was done. Postoperative period was uneventful. Histopathological examination showed discrete nodular infiltration of histiocytes arranged as a peribronchial pattern [Figure 3] which has a convoluted nucleus [Figure 4] and pale eosinophilic cytoplasm.

Figure 3: Discrete nodular infiltration of histiocytes arranged as a peri bronchial pattern

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Figure 4: Histiocytes with convoluted nucleus and pale eosinophilic cytoplasm

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He was advised smoking cessation and was started with steroids and cladribine. He is on regular follow-up.

Discussion

PLCH, also called eosinophilic granuloma of the lung and pulmonary histiocytosis X, is an uncommon cystic interstitial lung disease that primarily affects young adults. It is caused by a disorder of myeloid dendritic cells. Tobacco smoke is found to be a contributing factor.

It is characterized by abnormal infiltration by Langerhans' cells, which are highly differentiated myeloid dendritic cells.

Pulmonary involvement may present as a component of multifocal LCH or as a single system disease. It can also be associated with malignancy such as lymphoma or can present as a premalignant condition.

PLCH is usually present in a male in the age group of 20–40 years. Almost all PLCHs have a somatic mutation in mitogen-activated protein kinase pathway.^[6] Somatic BRAF V600E mutations are thought to contribute in developing malignancy. It usually presents as an incidental finding or pneumothorax or with worsening respiratory symptoms such as chest pain, dyspnoea, and non-productive cough.

CT chest usually shows the progression of nodules to cavitating to cystic lesions over a period of time. The characteristic findings are combination of multiple cysts and nodules with a middle-to-upper zone predominance with interstitial thickening in a young smoker. Bronchoalveolar lavage (BAL) shows lymphocytosis with CD4:CD8 ratio decreased. Langerhans' cells can be identified by anti-S-100 protein antibodies. BAL of >5% Langerhans' cells implies a high possibility of LCH.^[7]

Histopathological examination is the gold standard for diagnosis. It shows pathological cell type – Langerhans' cells which contain pentalaminar inclusion bodies called Birbeck granules and langerin. Stellate lesions are also seen.

Precapillary pulmonary hypertension is a complication of LCH. Fluorodeoxyglucose-positron emission tomography scan can reveal other system involvements in a known case of pulmonary LCH.

Treatment involves smoking caseation. Patients with costophrenic angle involvement usually progress over time. Corticosteroids and cytotoxic drugs such as cladribine are initiated for progressive lesions. Inhaled bronchodilators are used for relief of airway obstruction. Recurrent pneumothorax can be alleviated by pleurodesis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Acknowledgment

We thank the Department of Pulmonology, Cardiothoracic Surgery, Radiology, and Pathology, Apollo Hospitals, Chennai, Tamil Nadu, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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