Nuss R, Ribeiro R C, Bunin N, Behm F, Jenkins J, Berard C, Murphy S B
Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.
Med Pediatr Oncol. 1988;16(3):220-3. doi: 10.1002/mpo.2950160314.
A 16-month-old female infant presenting with pancytopenia and fever was found to have pulmonary aspergillosis and large-cell immunoblastic non-Hodgkin's lymphoma of peripheral post-thymic origin isolated to bone marrow. Extensive noninvasive evaluations failed to demonstrate the disease in other extramedullary sites. The malignant cells were large and polymorphous; lacked terminal transferase; expressed surface CD-2, CD-3, CD-8, and HLA-DR antigens, and showed rearrangements of the T-cell-receptor beta-chain gene. To our knowledge, this type of lymphoma in an infant has not been reported before. Furthermore, aspergillosis is a rare presenting feature in patients with lymphoproliferative disease. In our case, it may reflect an underlying immune deficiency associated with the transformation and proliferation of a suppressor T cell.
一名16个月大的女婴因全血细胞减少和发热就诊，被发现患有肺曲霉菌病以及起源于胸腺后外周且仅累及骨髓的大细胞免疫母细胞性非霍奇金淋巴瘤。广泛的非侵入性评估未能在其他髓外部位发现该疾病。恶性细胞大且形态多样；缺乏末端转移酶；表达表面CD-2、CD-3、CD-8和HLA-DR抗原，并显示T细胞受体β链基因重排。据我们所知，此前尚未报道过婴儿患这种类型的淋巴瘤。此外，曲霉菌病在淋巴增殖性疾病患者中是一种罕见的表现特征。在我们的病例中，它可能反映了与抑制性T细胞转化和增殖相关的潜在免疫缺陷。
