An 80-year-old woman with myelofibrosis and diffuse mosaic attenuation on chest computed tomography

Abstract

An 80-year-old woman with myelofibrosis sought evaluation for progressive dyspnea. Her past medical history included essential thrombocytosis, which transformed to myelofibrosis. Inspiratory computed tomography of chest showed diffuse mosaic attenuation with lymphadenopathy. Flexible bronchoscopy with lymph node and pulmonary parenchymal cryo biopsy revealed nodular deposits of extramedullary hematopoiesis in lung parenchyma and moderate to severe vascular medial and intimal thickening of pulmonary vasculature consistent with pulmonary parenchymal extramedullary hematopoiesis associated with pulmonary hypertension (a rare compensatory mechanism in myeloproliferative disorders). In this report, we explore the manifestations, pathogenesis, treatment, and prognosis of pulmonary extramedullary hematopoiesis reported in the literature.

Keywords: BAL, bronchoalveolar lavage; CT, computed tomography; DLCO, diffusion capacity of lung for carbon monoxide; EBUS, endobronchial ultrasound; EMH, extramedullary hematopoiesis; Extramedullary hematopoiesis; FEV1, forced expiratory volume in 1; FNAB, fine-needle aspiration biopsy; FVC, forced vital capacity; LPM, liters per minute; MPO, myeloperoxidase; Mosaic attenuation; Myelofibrosis; NSIP, nonspecific interstitial pneumonia; PEMH, pulmonary extramedullary hematopoiesis; PFT, Pulmonary function testing; PH, pulmonary hypertension; RES, reticuloendothelial system; TBLC, transbronchial lung cryobiopsy; TBNA, transbronchial needle aspiration; TLC, total lung capacity.