A Case of Uncommon Extrapulmonary Manifestations of Pulmonary Langerhans Cell Histiocytosis

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Langerhans Cell Histiocytosis (LCH) is a rare histiocytic disorder seen in all age groups that affects a variety of organs. While osteolytic lesions are most common, the histiocytes, derived from myeloid progenitor cells, can infiltrate any organ. Pulmonary involvement, while uncommon, typically manifests as a cystic interstitial lung disease in young adult smokers. Pulmonary Langerhans Cell Histiocytosis (PLCH) usually occurs as a single system disease; however, extra-pulmonary manifestations have been described.
Case Description:
A 33-year-old female with a history of tobacco abuse, PLCH complicated by prior spontaneous pneumothoraxes, and depression with prior suicide attempts presented from an outside hospital after being found unresponsive at home. She was intubated due to unresponsiveness. On arrival to our facility, her labs were notable for transaminases >2,000. Acetaminophen toxicity was suspected, and N-acetylcysteine was administered with improvement of transaminitis. On hospital day #2, the patient, while intubated, developed polyuria producing 250-300 cc/hr with a serum sodium increase from 138 on admission to 152. Additional history revealed the patient had been drinking numerous gallons of water daily for several months. Endocrinology was consulted and an MRI brain revealed lack of posterior pituitary brightness, suggestive of infiltrative disease and diabetes insipidus (DI). Desmopressin began to restore serum sodium to normal levels. Following extubation, the patient described depression exacerbated by her excessive fluid intake and nocturia, and complained of hearing difficulty. Audiometry revealed severe mixed conductive and sensorineural hearing loss, which raised suspicion for histiocytic infiltration of the temporal bone. The patient was discharged with plans for close outpatient specialty follow up and further work up.
Pulmonary involvement is seen in approximately 10% of LCH cases with smoking being the key etiologic factor. Common presentations of PLCH include spontaneous pneumothorax. Extrapulmonary LCH occurs inDue to the possibility of extrapulmonary manifestations, patients with PLCH require close attention. Earlier identification of this patient’s DI may have prevented her intentional overdose. Close follow up is essential as patients with DI from LCH may demonstrate deficiencies of other pituitary hormones. In two series of patients initially presenting with DI, 80% had deficiency in another pituitary hormone within five years. Temporal bone involvement is an exceptionally rare phenomenon in LCH with case reports suggesting low-dose radiotherapy may help restore hearing. Chemotherapy is the treatment of choice for patients with multisystem LCH.


Medicine and Health Sciences


Department of Medicine, Department of Medicine Faculty, Department of Medicine Fellows and Residents, Fellows and Residents

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