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- W2895580591 abstract "SESSION TITLE: Systemic Disease and the Lung 1 SESSION TYPE: Original Investigations PRESENTED ON: 10/07/2018 01:00 PM - 02:00 PM PURPOSE: Extramedullary hematopoiesis (EMH) is a complication of a variety of myeloproliferative neoplasms. The most common sites of EMH are the spleen and liver; however the involvement of other sites has been reported (1). The lungs and pleura are a rare site of EMH and have been reported to present with a variety of signs and symptoms (2). Here we present a series of 47 patients with confirmed EMH of the lung, the largest series to date. METHODS: We reviewed an internal database that captured 47 patients with EMH of the lung confirmed on nuclear medicine technetium scans or by biopsy from 1997-2012 at the Mayo Clinic in Rochester, Minnesota. RESULTS: Clinical manifestations were largely non-specific (fatigue 93%, dyspnea 85%, cough 30%, weight loss 30%). Imaging abnormalities were similarly non-specific but included pleural effusions (57%) and ground-glass opacities (43%) on chest CT imaging. Twenty-seven patients underwent echocardiographic evaluation of right ventricular systolic pressure(RVSP). Twenty one (78%) had elevated RVSP at time of diagnosis (>36 mm Hg) with an overall mean RVSP of 54.48 mm Hg. Sixteen of 47 (34%) of patients had had prior splenectomy. Twenty nine patients were subsequently treated with low dose (100 centigray) whole lung irradiation. Twenty one (72%) had subjective symptomatic improvement as judged by their hematologist in documentation. Of these 29 patients who received radiation, 13 underwent post-radiation echocardiography and the mean RVSP increased to 59.8 mm Hg, although this may not be clinically significant. In terms of prognosis, 42 of our 47 patients had known date/time of death, and median time from diagnosis to death was 7.5 months. This was increased to 17 months in those who received radiation and reduced to 4.5 months for those who did not. CONCLUSIONS: We conclude that EMH of the lung is associated with a poor prognosis and that radiation therapy may be associated with improved symptoms and longer survival. CLINICAL IMPLICATIONS: Our findings suggest that clinicians need to maintain a high degree of suspicion for EMH of the lung in patients with myeloproliferative neoplasms, as there are no specific signs or symptoms for this entity. Whole lung irradiation seems to be an effective symptomatic treatment for EMH of the lung for many patients, and receiving irradiation may be associated with improved overall survival. Finally, EMH of the lung is a poor prognostic factor and finding it may prompt clinicians to discuss end of life care with their patients. DISCLOSURES: No relevant relationships by Tariq Azam, source=Web Response No relevant relationships by Megan Dulohery Scrodin, source=Web Response" @default.
- W2895580591 created "2018-10-12" @default.
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- W2895580591 date "2018-10-01" @default.
- W2895580591 modified "2023-09-27" @default.
- W2895580591 title "EXTRAMEDULLARY HEMATOPOIESIS OF THE LUNG: A CASE SERIES" @default.
- W2895580591 doi "https://doi.org/10.1016/j.chest.2018.08.831" @default.
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