Ajayi Folasade, Nali Meenakshi S, Ali Ruhma, Patel Aditya, Shaaban Hamid
Internal Medicine, Saint Michael's Medical Center, Newark, USA.
Hematology/Oncology, Saint Michael's Medical Center, Newark, USA.
Cureus. 2022 Jan 17;14(1):e21334. doi: 10.7759/cureus.21334. eCollection 2022 Jan.
Extramedullary hematopoiesis can occur during normal fetal development or as a compensatory mechanism in chronic anemia when the primary sites of hematopoiesis fail. When it occurs, it is mostly seen in sites such as the liver, spleen, and lymph nodes. Extramedullary hematopoiesis is seen in patients with abnormal morphology of red blood cells like thalassemia and abnormal red blood cell shape like hereditary spherocytosis. The incidence of extramedullary hematopoiesis in sickle cell disease is very rare. We report a case of focal adrenal extramedullary hematopoiesis in a 21-year-old patient with sickle cell disease who presented with pain in the right thorax. A retroperitoneal mass was seen in the MRI of the abdomen of the patient suggestive of extramedullary hematopoiesis. Our case highlights the importance of physician awareness of this rare pathology for proper diagnosis and management.
髓外造血可发生于正常胎儿发育期间，或在慢性贫血时作为一种代偿机制，此时主要造血部位功能衰竭。当发生髓外造血时，多见于肝脏、脾脏和淋巴结等部位。在患有红细胞形态异常（如地中海贫血）或红细胞形状异常（如遗传性球形红细胞增多症）的患者中可见髓外造血。镰状细胞病患者髓外造血的发生率非常低。我们报告一例21岁镰状细胞病患者出现右胸疼痛，其肾上腺局灶性髓外造血病例。在该患者腹部的MRI检查中发现一个腹膜后肿块，提示髓外造血。我们的病例强调了医生认识这种罕见病理情况对于正确诊断和管理的重要性。
