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首页> 外文期刊>Leukemia and lymphoma >An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma.
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An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma.

机译：血管内淋巴瘤病的亚洲变种：恶性组织细胞病样B细胞淋巴瘤的最新综述。

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Malignant histiocytosis-like B-cell lymphoma (MH-like BCL) is characterized clinically by hemophagocytic syndrome (HPS), bone marrow involvement at presentation, and an aggressive clinical course. Yet, it remains an ill-defined disease entity. We recently described five cases of MH-like BCL and suggested that these may be regarded as a peculiar variant of intravascular lymphomatosis (IVL), based on clinical and pathological observation. Interestingly, this type of lymphoma has been reported exclusively among Asians, with few reports from Western countries, and therefore we propose that this variant be noted as an Asian variant of IVL(AIVL). To evaluate the incidence of this variant, we performed a literature search for English or Japanese studies dealing with MH-like BCL or B-cell IVL associated with HPS (IVL with HPS). We found 18 reports of MH-like BCL and 16 reports of IVL with HPS and re-evaluated the clinicopathologic aspects of each study with respect to AIVL. Although several differences were noted, such as gender, presence of respiratory changes, bone marrow invasion and disseminated intravascular coagulopathy, there were also overlapping profiles and they appeared to be consistent with the AIVL single disease entity. Of particular note was the absence of peripheral lymphadenopathy and tumor formation which posed diagnostic problems in the initial phase of the presentations. Thus, based on analysis of the clinical findings of these 34 cases, the diagnostic criteria for AIVL consists primarily of cytopenia (anemia and/or thrombocytopenia), hepatosplenomegaly, absence of peripheral lymphadenopathy and tumor formation, and erythrocyte-hemophagocytosis along with intravascular proliferation of the neoplastic B cells. The pathologic findings of the 19 autopsied cases which fulfilled the criteria are also summarized, and they reveal morphologic, immunologic, and genotypic features of typical IVL.
机译：恶性组织细胞增生样B细胞淋巴瘤（MH类BCL）的临床特征是吞噬细胞综合征（HPS），表现为骨髓受累以及侵袭性的临床过程。然而，它仍然是一个不确定的疾病实体。我们最近描述了5例MH样BCL病例，并建议根据临床和病理学观察将其视为血管内淋巴瘤病（IVL）的特殊变体。有趣的是，这种类型的淋巴瘤仅在亚洲人中报道过，西方国家几乎没有报道，因此，我们建议将此变异体称为IVL（AIVL）的亚洲变异体。为了评估该变异的发生率，我们进行了文献检索，以寻找与HPS相关的MH样BCL或B细胞IVL（IVS与HPS）相关的英语或日语研究。我们发现18例MH样BCL报告和16例HPS伴有IVL的报告，并就AIVL对每项研究的临床病理学方面进行了重新评估。尽管注意到一些差异，例如性别，呼吸变化的存在，骨髓浸润和弥散性血管内凝血病，但也存在重叠的特征，它们似乎与AIVL单一疾病实体一致。特别值得注意的是，没有外周淋巴结肿大和肿瘤形成，这在报告的初始阶段就构成了诊断问题。因此，根据对这34例患者的临床发现的分析，AIVL的诊断标准主要包括血细胞减少症（贫血和/或血小板减少症），肝脾肿大，无周围淋巴结肿大和肿瘤形成，红细胞血细胞吞噬以及血管内皮增生。肿瘤B细胞。还总结了符合标准的19例尸检病例的病理学发现，它们揭示了典型IVL的形态，免疫学和基因型特征。

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