Ha Duc, Liao Xiaoyan, Wang Huan-You, Jamieson Catriona, Magaña Marisa, Makani Samir
*Division of Pulmonary, Critical Care, and Sleep Medicine †Department of Pathology ‡Division of Hematology-Oncology, University of California San Diego, La Jolla, CA.
J Bronchology Interv Pulmonol. 2016 Oct;23(4):343-346. doi: 10.1097/LBR.0000000000000296.
Thoracic extramedullary hematopoiesis (EMH) is a rare manifestation in patients with myeloproliferative neoplasm. A 76-year-old woman with a long-standing history of polycythemia vera presented with a 2-month history of worsening dyspnea and left-sided wheezing. A chest computed tomography showed an ill-defined soft tissue mass encasing the left mainstem bronchus causing airway obstruction, associated with paratracheal and paraesophageal lymphadenopathy. Endobronchial ultrasound-guided fine needle aspiration of the soft tissue mass and mediastinoscopy with excisional biopsy of a paratracheal lymph node demonstrated EMH with increased myeloid blasts. A bone marrow biopsy confirmed postpolycythemic myelofibrosis consistent with progression of polycythemia vera to myelofibrosis. We describe the bronchoscopic management of a case of EMH presenting as a mediastinal mass, mimicking malignancy.
胸段髓外造血(EMH)是骨髓增殖性肿瘤患者的一种罕见表现。一名76岁有真性红细胞增多症长期病史的女性,出现了2个月的进行性呼吸困难和左侧哮鸣音。胸部计算机断层扫描显示一个边界不清的软组织肿块,包绕左主支气管导致气道阻塞,并伴有气管旁和食管旁淋巴结肿大。经支气管超声引导下对软组织肿块进行细针穿刺抽吸,并通过纵隔镜对气管旁淋巴结进行切除活检,结果显示为髓外造血伴髓系原始细胞增多。骨髓活检证实为真性红细胞增多症后骨髓纤维化,符合真性红细胞增多症进展为骨髓纤维化。我们描述了一例表现为纵隔肿块、酷似恶性肿瘤的髓外造血病例的支气管镜处理。