Higuchi T, Mori H, Niikura H, Omine M
Division of Hematology, Showa University Fujigaoka Hospital, Yokohama, Japan.
Acta Haematol. 1996;96(2):68-72. doi: 10.1159/000203718.
Serum complement levels and hematological data were evaluated in five patients with immunoblastic lymphadenopathy (IBL) and four with IBL-like T cell lymphoma (IBL-T). Anemia with Hb values below 10.0 g/dl was seen in four patients. A direct Coombs test was positive in five patients and the bone marrow of two of these showed features of pure red cell aplasia. Seven patients were thrombocytopenic with platelet counts below 100 x 10(9)/l. Six of the seven patients had splenomegaly. Platelet-associated IgG was elevated in all three thrombocytopenic patients examined. Whole complement activity (CH50) was reduced in eight patients (89%) at presentation and subsequently normalized in five who were treated either with prednisolone (two patients with IBL) or with multidrug combination chemotherapy (three with IBL-T). One patient achieved complete remission and four partial remission. Remission was accompanied by normalization of hematological abnormalities and elevation of complement activity to the normal range in all cases. These results suggested that complement-mediated mechanisms are responsible, at least in part, for some of the hematological abnormalities observed in IBL and IBL-T and that hypocomplementemia is a common abnormality with significance as a laboratory marker for the disease activity.
对5例免疫母细胞性淋巴结病（IBL）患者和4例IBL样T细胞淋巴瘤（IBL-T）患者的血清补体水平和血液学数据进行了评估。4例患者出现血红蛋白值低于10.0g/dl的贫血。5例患者直接抗人球蛋白试验呈阳性，其中2例患者的骨髓显示纯红细胞再生障碍的特征。7例患者血小板减少，血小板计数低于100×10⁹/L。7例患者中有6例脾肿大。在所有3例接受检查的血小板减少患者中，血小板相关IgG均升高。8例患者（89%）初诊时全补体活性（CH50）降低，随后5例接受泼尼松龙治疗（2例IBL患者）或多药联合化疗（3例IBL-T患者）的患者补体活性恢复正常。1例患者达到完全缓解，4例部分缓解。所有病例缓解均伴有血液学异常的正常化和补体活性升高至正常范围。这些结果表明，补体介导的机制至少部分地导致了IBL和IBL-T中观察到的一些血液学异常，并且补体低下血症是一种常见异常，作为疾病活动的实验室标志物具有重要意义。
