Pulmonary Vascular Disease: Fellow Case Report Slide: Pulmonary Critical Care Disorders: Think Twice |

Systemic Mastocytosis With Pulmonary Involvement FREE TO VIEW

Ray Pillai, MD; Darren Maehara, MD; Nishay Chitkara, MD
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New York University, New York, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1136A. doi:10.1016/j.chest.2016.08.1246
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SESSION TITLE: Fellow Case Report Slide: Pulmonary Critical Care Disorders: Think Twice

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 23, 2016 at 10:45 AM - 12:00 PM

INTRODUCTION: Mastocytosis is a rare disorder that involves mast cell infiltration in a range of organs. Pulmonary involvement is an even rarer entity. Here we present a case of a patient with systemic mastocytosis with asymptomatic pulmonary involvement.

CASE PRESENTATION: The patient is a 68 year old male who was referred to primary clinic to establish care. He recently presented to the emergency room for abdominal pain of seven months duration. There he was diagnosed with gallstones and an inguinal hernia and discharged with primary care followup. The only other complaint the patient had was persistent pruritus for over one year. On review of systems the patient was noted to have weight loss. He denied any cough or shortness of breath. On exam the patient was cachectic with numerous skin excoriations from scratching. He had mild right upper quadrant tenderness, hepatomegaly, and a reducible inguinal hernia. Labs were notable for an alkaline phosphatase of 652 IU/L and a leukocytosis of 11.8 cells per mL with 29% eosinophils. Serum tryptase was significantly elevated at 130 ng/mL. Abdominal ultrasound demonstrated hepatomegaly with coarse lobulated surface suggestive of chronic liver disease. A chest X-ray obtained during his emergency room visit was notable for diffuse reticular opacities. Chest CT showed pulmonary nodules and hilar lymphadenopathy. Liver biopsy and EBUS were performed with pathology consistent with systemic mastocytosis.

DISCUSSION: Systemic mastocysotosis is a rare disease and involvement of the skeletal muscle, gastrointestinal tract, and lymph nodes is common. Pulmonary involvement is less common and may occur in less than 20% of patients with systemic mastocytosis. Symptoms can include cough, dyspnea, and asthma like symptoms. Findings on chest radiograph include interstitial fibrosis and pulmonary nodules. CT findings include nodular and reticular opacities as well as mediastinal lymph node enlargement.

CONCLUSIONS: Pulmonary involvement is a rare manifestation of systemic mastocytosis. The disease can present with a wide range of findings on chest radiograph and CT. Symptoms can vary between being asymptomatic to having mild asthma like symptoms.

Reference #1: Avila N. A. et al. 1996. Pulmonary and Ovarian Manifestations of Systemic Mastocytosis. AJR; 166: 969-970.

Reference #2: Kelly A. M. et al. 2004. HRCT Appearance of Systemic Mastocytosis Involving the Lungs. J Thoracic Imaging; 19: 52-55.

Reference #3: Schmidt M. et al. 2000. Pulmonary manifestation of systemic mast cell disease. Eur Respir J; 15: 623 - 625.

DISCLOSURE: The following authors have nothing to disclose: Ray Pillai, Darren Maehara, Nishay Chitkara

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