Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with aLangerhans cell phenotype, which comprises a wide range of clinical presentations. The reported patient is a 55-year old female with multy-sistem LCH and pulmonary tuberculosis. During diagnostic processing, infiltrates of Langerhans cells (S-100+, CD1a+) in the epidermis, intestinal mucosa and bone marrow, were discovered. Transbronchial needle biopsy of the lung revealed non-specific clusters of macrophages (CD68+, CD1a-). Although CD1a was negative, positive CD68, thoracic MSCT and clinical findings indicated pulmonary LCH. Corticosteroid treatment led to significant clinical improvement. Seven months after treatment with corticosteroids the patient developed a spontaneous left-sided pneumothorax. Thoracic HR (high resolution)-MSCT was highly suggestive for active Mycobacterium tuberculosis (MT) infection together with the positive QUANTIFERON-TB test and typical symptoms. One week after starting standard triple antituberculotic therapy the patient developed hypovolemic shock. Despite all medical efforts, the patient died on the thirteenth day of intensive treatment for multiple organ failure.