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Diffuse Lung Disease |

Pulmonary Hypertension in Myelofibrosis

QinHao Jonathan Ye, MBBS; Christopher Willis, MD; Lee Lan Phoa, MBBS
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KTPH, Singapore, Singapore


Chest. 2014;146(4_MeetingAbstracts):402A. doi:10.1378/chest.1962269
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Abstract

SESSION TITLE: ILD Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: We report a rare case of extramedullary haematopoiesis (EMH) in the lung resulting in pulmonary hypertension and right heart failure.

CASE PRESENTATION: A 64 years old Chinese gentleman with a background of myelofibrosis presented with shortness of breath for 2 days associated with lower limb swelling and decreased in effort tolerance. Examination findings were consistent with right heart failure. Chest radiography showed cardiomegaly and a mild right-sided pleural effusion. 2D echocardiogram revealed severe dilatation of the right ventricle with severe right ventricle systolic dysfunction and severe tricuspid regurgitation. There was no evidence of pulmonary embolism on CT Pulmonary Angigram. A Technectium-99 sulfur colloid scintigraphy with Single-Photon Emission Computed Tomography (SPECT) showed higher tracer uptake in the spleen, ribs, distal femur and diffusely in both lungs suggestive of EMH. The patient was started on radiotherapy and showed improvement in symptoms, lower limb swelling and a decreased parasternal heave at 1-month follow-up.

DISCUSSION: Pulmonary hypertension due to EMH in patients with myeloproliferative disease is rare with only 37 reported cases since 19621. Our case was the only reported one of Asian descent. Diagnosis is made either via direct tissue biopsy or using Technectium-99 sulfur colloid scintigraphy2. As Technetium-99m sulfur colloid is phagocytosed by cells of the reticuloendothelial system, EMH will show an abnormal distribution of uptake outside of the marrow, liver and spleen with increased uptake in rare sites like the lung, bowels and skin. In our patient, the scan showed diffuse increased uptake of the tracer colloid in the lungs suggesting the presence of EMH in the lungs. Low dose radiation for EMH has been described with reported clinical improvement.3 Our patient received 4 doses of radiation with initial improvement of symptoms but subsequently deteriorated and passed away several months later.

CONCLUSIONS: EMH in myeloproliferative disorders is a rare cause of pulmonary hypertension. Whole lung radiation therapy has been reported as a possible treatment for pulmonary hypertension resulting from EMH with clinical improvement.

Reference #1: K.Trow et al. A 71 year old woman with myelofibrosis, hypoxemia and pulmonary hypertension. Chest 2010;138(6):1506-1510

Reference #2: Glenn et al. Tc-99m Sulfur colloid demonstration of diffuse pulmonary interstitial extramedullary hematopoiesis in a patient with myelofibrosis. Clin Nucl Med 1994;19:1079-1084

Reference #3: Steensma DP et al. Low-dose, single-fraction, whole-lung radiotherapy for pulmonary hypertension associated with myelofibrosis with myeloid metaplasia. Br J Haematol. 2002 Sep;118(3):813-6

DISCLOSURE: The following authors have nothing to disclose: QinHao Jonathan Ye, Christopher Willis, Lee Lan Phoa

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