PERIPHERAL T-CELL LYMPHOMA PRESENTING AS HEMOPHAGOCYTIC SYNDROME
PERIPHERAL T-CELL LYMPHOMA PRESENTING AS HEMOPHAGOCYTIC SYNDROME
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DOI：
10.1002/hon.2900070404
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发表时间：
1989-07-01
影响因子：
3.3
通讯作者：
HO, FCS
中科院分区：
文献类型：
--
作者：
CHAN, EYT;PI, D;HO, FCS
本文报告三例外周T细胞淋巴瘤，临床表现为发热、洗涤、肝脾肿大及全血细胞减少，但无骨髓炎性淋巴瘤的发生。在疾病早期，当没有明显的淋巴结肿大时，这些病例造成了巨大的诊断混乱。虽然临床特征提示恶性组织细胞增生症（MH），但骨髓表现为吞噬性组织细胞，表现不典型，不能满足MH的诊断标准。淋巴结肿大仅在症状发作后14、5和8周检测到，然后通过淋巴结活检诊断为T淋巴瘤。尝试了多药化疗治疗。两名患者在治疗开始后3天和11周死亡，第三名患者失访。与文献中报道的大多数病例相反，我们的病例显示反应性噬血细胞综合征可能是潜在T细胞淋巴瘤的早期和突出表现。与其他原因的噬血细胞综合征的鉴别可能是困难的，并且在最初阶段缺乏恶性肿瘤的组织学证据，从而延误了明确的诊断和治疗。
Three patients with peripheral T-cell lymphoma presenting with pyrexia, washing, hepatosplenomegaly and pancytopenia in the absence of myelophthisic lymphomatous involvement are reported. Early in the course of the disease when there was no significant lymphadenopathy, these cases created enormous diagnostic confusion. Although the clinical features were suggestive of malignant histiocytosis (MH), marrow findings showed phagocytic histiocytes which did not appear atypical, and the criteria for diagnosis of MH could not be satified. Lymph node enlargement was detected only after 14, 5, and 8 weeks from the onset of symptoms, and the diagnosis of T-lymphoma was then made on lymph node biopsies. Treatment with multiple agent chemotherapy was attempted. Two patients died 3 days and 11 weeks after treatment was started and the third was lost of follow-up. In contrast with most of the cases reported in the literature, our cases show that a reactive hemophagocytic syndrome can be an early and prominent manifestation of an underlying T-cell lymphoma. Differentiation from other causes of hemophagocytic syndrome can be difficult and lack of histological proof of malignancy in the initial stage of ten delays definitive diagnosis and treatment.