Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report
Clinical Medicine Research
Volume 6, Issue 4, July 2017, Pages: 139-142
Received: May 21, 2017;
Accepted: Jun. 28, 2017;
Published: Jul. 26, 2017
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Veljko Flego, Department of Pulmonology, Clinical Hospital Centre Rijeka, Rijeka, Croatia
Darian Volaric, Primary Health Care Office, Health Centre of Primorsko-Goranska County, Rijeka, Croatia
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Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with a Langerhans 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.
Langerhans-Cell Histiocytosis, Pulmonary Tuberculosis, Multislice Computed Tomography, Corticosteroids, Multiple Organ Failure
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Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report, Clinical Medicine Research.
Vol. 6, No. 4,
2017, pp. 139-142.
Copyright © 2017 Authors retain the copyright of this article.
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