Preciado M V, De Matteo E, Fallo A, Chabay P, Drelichman G, Grinstein S
Laboratory of Virology, Ricardo Gutiérrez Children Hospital, Gallo 1330, 1425 Ciudad de Buenos Aires, Argentina.
Leuk Lymphoma. 2001 Jun;42(1-2):231-4. doi: 10.3109/10428190109097696.
An 8-years-old boy was admitted with fever of unknown origin, cervical lymphadenopathy and hepatosplenomegaly and weight loss. His mother's HIV infection was diagnosed two weeks before his hospitalization, so he was diagnosed as perinatally acquired AIDS. Serology and serial cultures were negative for viral infections, toxoplasmosis, chagas, tuberculosis and atypical mycobacterium. The patient met clinical and laboratory criteria for hemophagocytic syndrome (HS) that was confirmed on bone marrow aspirate and biopsy. A cervical lymph node biopsy was performed which was diagnosed as Hodgkin's disease (HD) diffuse fibrosis lymphocyte depletion subtype. EBERs in situ hybridization and LMP-1 immunohistochemistry on the lymph node biopsy established the EBV association. On the basis of a sequence of appearance of the clinical, laboratory and histological signs, HIV, EBV or HD may have triggered HS as the last fatal event in this pediatric patient.
一名8岁男孩因不明原因发热、颈部淋巴结肿大、肝脾肿大及体重减轻入院。其母亲在他住院前两周被诊断为感染艾滋病毒，因此他被诊断为围产期获得性艾滋病。血清学检查和系列培养对病毒感染、弓形虫病、恰加斯病、结核病和非典型分枝杆菌均呈阴性。该患者符合噬血细胞综合征（HS）的临床和实验室标准，骨髓穿刺和活检证实了这一诊断。进行了颈部淋巴结活检，诊断为霍奇金淋巴瘤（HD）弥漫性纤维化淋巴细胞消减亚型。淋巴结活检的EBERs原位杂交和LMP-1免疫组化确定了与EB病毒的关联。根据临床、实验室和组织学征象出现的顺序，艾滋病毒、EB病毒或霍奇金淋巴瘤可能触发了噬血细胞综合征，成为该儿科患者的最后致命事件。
