99mTc-Sulfur Colloid Bone Marrow Scintigraphy in Diagnosis of Diffuse Pulmonary Extramedullary Hematopoiesis Secondary to Myelofibrosis

Abstract

Our objective was to define the role of combined ^99mTc-sulfur colloid bone marrow (SC BM) scintigraphy, SPECT or SPECT/CT, and chest CT in diagnosing diffuse pulmonary extramedullary hematopoiesis (PEMH) in patients with myelofibrosis. Methods: We retrospectively reviewed ^99mTc-SC BM scintigraphy scans performed at our institution for the diagnosis of diffuse PEMH, as well as accompanying chest CT and SPECT/CT imaging findings. Relevant clinical information, including respiratory manifestations, pulmonary hypertension, and subjective response to whole-lung radiation therapy, was also summarized. Results: Twenty-two myelofibrosis patients with 27 ^99mTc-SC BM scintigraphy scans were diagnosed with diffuse PEMH. In 21 patients (95%) with accompanying chest CT and SPECT/CT scans, the most common CT findings were ground-glass opacity, interstitial infiltration, and pleural effusion. Of 20 patients (91%) who underwent 2-dimensional echocardiography studies, 12 (55%) were diagnosed with pulmonary hypertension. All 12 patients exhibited the aforementioned nonspecific CT imaging findings, with 8 (66%) of them presenting with respiratory symptoms, including dyspnea, shortness of breath, and cough. In the remaining 8 patients, without pulmonary hypertension, half had similar respiratory symptoms. Fourteen patients (64%) of this cohort received whole-lung radiation therapy, of whom 7 (50%) experienced symptom relief after therapy. Conclusion: Nonspecific respiratory symptoms should raise concern about pulmonary hypertension and diffuse PEMH in patients with advanced-stage myelofibrosis. Combined ^99mTc-SC BM scintigraphy and SPECT/CT is a promising noninvasive imaging tool to diagnose this rare clinical entity.Key Words: hematology; respiratory; SPECT/CT; pulmonary hematopoiesis; Tc-99m sulfur colloid scintigraphy; myelofibrosis.