Castelli Roberto, Graziadei Giovanna, Karimi Mehran, Cappellini Maria Domenica
Unità Operativa di Medicina d'Urgenza e Pronto Soccorso, IRCCS Ospedale Maggiore Policlinico IRCCS Via f. Sforza 35, 20122 Milan, Italy.
Am J Med Sci. 2004 Nov;328(5):299-303. doi: 10.1097/00000441-200411000-00012.
Extramedullary hematopoiesis often occurs in hemoglobinopathies, hemolytic anemias, leukemias, lymphomas, and myeloproliferative disorders. Liver, spleen, and lymph nodes are frequently involved. However, extramedullary hematopoiesis may also develop in other sites such as thymus, kidney, retroperitoneum, and paravertebral areas of the thorax. Extramedullary hematopoietic masses are often microscopic and asymptomatic, but sometimes they lead to tumor-like masses. We describe massive intrathoracic extramedullary hematopoiesis in a 41-year-old man with compound heterozygosis for beta-thalassemia and sickle cell anemia and functional asplenia. We also describe a 39-year-old man with beta-thalassemia intermedia, who was initially diagnosed as having tumor masses, but was later proved, by magnetic resonance imaging, to have extramedullary erythropoietic tissue. These observations provide further support to include extramedullary hematopoiesis among the differential diagnosis of tumor-like masses in patients with hematologic diseases.
髓外造血常见于血红蛋白病、溶血性贫血、白血病、淋巴瘤和骨髓增殖性疾病。肝脏、脾脏和淋巴结常受累。然而，髓外造血也可能发生在其他部位，如胸腺、肾脏、腹膜后以及胸部椎旁区域。髓外造血肿块通常微小且无症状，但有时会导致肿瘤样肿块。我们描述了一名41岁男性，患有β地中海贫血和镰状细胞贫血复合杂合子且功能性无脾，出现了大量胸腔内髓外造血。我们还描述了一名39岁的中间型β地中海贫血男性，最初被诊断为患有肿瘤肿块，但后来通过磁共振成像证实为髓外造血组织。这些观察结果进一步支持将髓外造血纳入血液病患者肿瘤样肿块鉴别诊断的范畴。
