Zaharopoulos P
Department of Pathology, University of Texas Medical Branch, Galveston, Texas 77555-0548, USA.
Diagn Cytopathol. 2001 Oct;25(4):248-52. doi: 10.1002/dc.2048.
The case of a 22-yr-old male who after a brief febrile episode developed autoimmune hemolytic anemia and right pulmonary infiltrate with pleural effusion is presented. Cytologic examination of the pleural fluid revealed lymphocytosis and hemophagocytosis, primarily of red blood cells (RBCs) by mature histiocytes. There was accompanying splenomegaly, laboratory evidence of hepatic dysfunction, and retroperitoneal lymphadenopathy. Besides profound reduction of red blood cells in the peripheral blood, there was reduction of lymphocytes and platelets. As a neoplastic process was ruled out by bone marrow and pleural biopsies, the disease was considered to be virus-induced and was halted and progressively regressed with early institution of vigorous antiinflammatory therapy with adrenocortical steroids. Upon reviewing the case, examination of the bone marrow biopsy disclosed limited hemophagocytosis of RBCs and lymphocytes by histiocytes and considerable viral cytopathic effect on hematopoietic cells (red and white cell precursors and megakaryocytes), which by appropriate immunolabelling was identified as induced by Epstein-Barr virus. A virus-related acquired hemophagocytic syndrome in its early stages was probably present, yet an undesirable clinical outcome was averted by early institution of vigorous steroid therapy. The need to recognize early hemophagocytic changes in cytologic specimens for early institution of appropriate therapy is emphasized. The possibility of erythrophagocytosis, also manifested during the course of an autoimmune hemolytic process and unrelated to hemophagocytic syndrome, is discussed.
本文报告了一名22岁男性患者的病例，该患者在经历短暂发热后，出现了自身免疫性溶血性贫血以及伴有胸腔积液的右肺浸润。胸腔积液的细胞学检查显示淋巴细胞增多和噬血细胞现象，主要是成熟组织细胞对红细胞（RBC）的吞噬。同时伴有脾肿大、肝功能障碍的实验室证据以及腹膜后淋巴结病。除外周血红细胞显著减少外，淋巴细胞和血小板也减少。由于骨髓和胸膜活检排除了肿瘤性病变，该疾病被认为是病毒诱导的，通过早期给予肾上腺皮质类固醇进行积极抗炎治疗，病情得到控制并逐渐好转。回顾该病例时，骨髓活检检查发现组织细胞对红细胞和淋巴细胞的噬血细胞现象有限，并且造血细胞（红细胞和白细胞前体以及巨核细胞）存在明显的病毒细胞病变效应，通过适当的免疫标记确定为Epstein-Barr病毒诱导。早期可能存在与病毒相关的获得性噬血细胞综合征，但通过早期积极的类固醇治疗避免了不良的临床结局。强调了在细胞学标本中早期识别噬血细胞变化以便早期进行适当治疗的必要性。还讨论了在自身免疫性溶血过程中也会出现的、与噬血细胞综合征无关的红细胞吞噬现象的可能性。
