Crawford Charles K, Arshad Hajra, Kawamoto Satomi, Fishman Elliot K
Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Radiol Case Rep. 2025 Mar 8;20(5):2482-2486. doi: 10.1016/j.radcr.2025.02.006. eCollection 2025 May.
Primary myelofibrosis (PMF) is a rare and progressive bone marrow cancer characterized by fibrosis, abnormal megakaryocyte proliferation, and impaired blood cell production. We present a case of a 65-year-old male diagnosed with accelerated PMF and periportal extramedullary hematopoiesis. Despite a history of stable myelofibrosis, the patient developed progressive hepatosplenomegaly and persistent anemia. Imaging studies, including MRI and CT scans, revealed periportal masses concerning for malignant infiltrative process. Subsequent biopsies confirmed the diagnosis of a sclerosing extramedullary hematopoietic tumor. Treatment included JAK2 inhibitor therapy, blood transfusions, and an eventual bone marrow transplant. This case highlights the complexities in diagnosing and managing PMF, particularly with rare complications like periportal extramedullary hematopoiesis, and underscores the importance of integrating radiological and pathological findings for optimal management.
原发性骨髓纤维化（PMF）是一种罕见的进行性骨髓癌，其特征为纤维化、异常巨核细胞增殖以及血细胞生成受损。我们报告一例65岁男性，诊断为加速期PMF伴门周髓外造血。尽管有骨髓纤维化稳定病史，但患者出现进行性肝脾肿大和持续性贫血。包括MRI和CT扫描在内的影像学检查显示门周肿块，怀疑为恶性浸润性病变。随后的活检证实为硬化性髓外造血肿瘤。治疗包括JAK2抑制剂治疗、输血以及最终的骨髓移植。该病例突出了PMF诊断和管理中的复杂性，尤其是伴有门周髓外造血等罕见并发症时，并强调了整合放射学和病理学检查结果以实现最佳管理的重要性。
