A rare case of systemic mastocytosis with pulmonary involvement is being presented at the American Thoracic Society’s 2022 International Conference, being held May 13-18, 2022.

The case involved a 78-year-old woman who presented with dyspnea of two months evolution. She was diagnosed with mastocytosis based on the abnormal mast cell population identified on bone marrow biopsy.

The pulmonary involvement was extensive, including left and right pleural effusions, patchy consolidations, and ascites. She also had a cirrhotic liver and hepatomegaly, portal hypertension, eosinophilic hepatitis, and stage 2 fibrosis, researchers reported.

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A literature review by the authors of the case found very few cases of pulmonary involvement along with mastocytosis. “Mastocytosis is an extremely rare disorder and pulmonary involvement is even rarer,” they wrote.

In this case, the patient developed progressive hypercapneic and hypoxemic respiratory failure. She declined treatment and elected hospice care instead.

The authors suggest that in cases of pleural effusions and chest consolidations on computer tomography (CT), a suspicion of this combined condition may be warranted, and early identification and treatment could improve outcomes.

Reference

Moore JL, Margolskee S, Lee M, et al. Pulmonary involvement in systemic mastocytosis. Am J Respir Crit Care Med. 2022; 205:A2234. doi:10.1164/ajrccm-conference.2022.205.1