Friedman-Klabanoff DeAnna, Ball Allison, Rutare Samuel, McCall Natalie, Blackall Douglas P
*Department of Pediatrics, School of Medicine, Duke University, Durham, NC†Department of Pediatrics, Geisel School of Medicine, Dartmouth College, Hanover, NH‡Department of Pediatrics, Kigali University Teaching Hospital, Kigali, Rwanda§Department of Pediatrics, School of Medicine, Yale University, New Haven, CT∥Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
J Pediatr Hematol Oncol. 2016 Jul;38(5):e158-61. doi: 10.1097/MPH.0000000000000561.
This report describes 3 Rwandan children with massive splenomegaly and pancytopenia who underwent splenectomy. Each was diagnosed with Epstein-Barr virus-associated lymphoproliferative disorder (EBV LPD) based on lymphocyte morphology, lymphocyte immunophenotype, and the results of EBV in situ hybridization studies. The differential diagnosis of splenomegaly, with a special emphasis on the sub-Saharan African context, is discussed along with EBV and associated disorders. These cases serve as a call to consider EBV LPD in the differential diagnosis of splenomegaly in children in whom common causes have been ruled out.
本报告描述了3例接受脾切除术的患有巨大脾肿大和全血细胞减少症的卢旺达儿童。根据淋巴细胞形态、淋巴细胞免疫表型以及EBV原位杂交研究结果，每例均被诊断为 Epstein-Barr病毒相关淋巴增生性疾病（EBV LPD）。本文讨论了脾肿大的鉴别诊断，特别强调了撒哈拉以南非洲地区的情况，并探讨了EBV及相关疾病。这些病例提醒我们，在排除常见病因的儿童脾肿大鉴别诊断中应考虑EBV LPD。
