Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Am J Clin Pathol. 2012 May;137(5):786-94. doi: 10.1309/AJCPP40MEXWYRLPN.
Hemophagocytic lymphohistiocytosis (HLH) is an often fatal hyperinflammatory syndrome. HLH may be inherited, but it more commonly arises secondary to Epstein-Barr virus (EBV) or other infections, hematologic malignancies, or rheumatologic diseases. We identified 17 patients diagnosed with HLH who had flow cytometric analysis of peripheral blood or bone marrow performed at the time of diagnosis. Two patients had primary HLH, and the others had HLH secondary to EBV infection, hematologic malignancies, rheumatologic conditions, or tuberculosis. The marrow typically showed a reactive lymphocytosis and a marked left shift in myelopoiesis regardless of the etiology. Qualitative abnormalities were also found in several cases, including T-cell abnormalities in the majority of the EBV-associated HLH cases. While not specific, flow cytometric findings in HLH are different from the findings in uninvolved marrow samples, and care should be taken not to overinterpret immunophenotypic findings in these cases as indicative of a primary marrow disorder or lymphoma.
噬血细胞性淋巴组织细胞增生症（HLH）是一种常致命的过度炎症综合征。HLH 可以是遗传性的，但更常见的是继发于 EBV（Epstein-Barr virus）或其他感染、血液系统恶性肿瘤或风湿性疾病。我们鉴定了 17 例在诊断时进行了外周血或骨髓流式细胞分析的 HLH 患者。2 例为原发性 HLH，其余为 EBV 感染、血液系统恶性肿瘤、风湿性疾病或结核继发的 HLH。无论病因如何，骨髓通常显示反应性淋巴细胞增多和明显的左移性髓系造血。在一些病例中还发现了定性异常，包括大多数 EBV 相关 HLH 病例中的 T 细胞异常。虽然不具有特异性，但 HLH 的流式细胞检查结果与未受累骨髓样本的结果不同，在这些病例中，应注意不要过度解释免疫表型结果，以免提示原发性骨髓疾病或淋巴瘤。
