Pulmonary Langerhans cell histiocytosis in adults: A case report

Abstract

BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thin-walled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.

Histiocitosis de células de Langerhans, una afección pulmonar infradiagnosticada en fumadores: reporte de caso y revisión de la literatura

“…Algunos autores [1,9,33,34] han señalado que este podría ser un hallazgo patognomónico en el contexto de un paciente con antecedentes típicos, y que ante este hallazgo imagenológico no se requeriría de una biopsia pulmonar para hacer el diagnóstico. Sin embargo, cabe señalar que este patrón de TACAR es relativamente poco común [9], y en la mayoría de los pacientes es más frecuente ver quistes o nódulos solos o aleatorios, como en la paciente de nuestra revisión, con hallazgos que coinciden con los reportados en otros casos clínicos de la literatura [17,35,36]. Usualmente, estos hallazgos no son específicos, y a menudo requerirán de confirmación diagnóstica mediante estudios histopatológicos.…”

Pulmonary Langerhans Cell Histiocytosis in the Elderly Smoker

“…The incidence of LCH is three to five cases per million per year in children younger than three years and one to two cases per million per year in adults [2]. Organ involvement also varies with age as in the young population most commonly involved organ is bones (~80%) followed by skin, pituitary gland, lungs, liver, and lymph nodes [3]. Isolated pulmonary Langerhans cell histiocytosis (PLCH) is the most common manifestation in the adult population.…”

Isolated pulmonary langerhans cell histiocytosis: A diagnostic challenge in oncologic patients

“…Isolated pulmonary involvement (pulmonary langerhans cell histiocytosis [PLCH]) primarily affects young adults (20–40 years) with cigarette smoking habit. [ 1 , 2 ]…”

“…The PLCH typical radiological pattern can be diagnostic if correctly evaluated with suggestive clinical features and once established a relevant cigarette smoking attitude. [ 1 , 2 ] HRCT has a pivotal role in PLCH diagnosis because it represents the gold standard in the differential diagnosis of interstitial lung diseases. This is possible because HRCT differential diagnosis was made according to the predominant CT pattern, the distribution of the findings and the additional CT findings.…”