KSHV- and EBV-associated germinotropic lymphoproliferative disorder
KSHV- and EBV-associated germinotropic lymphoproliferative disorder
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DOI：
10.1182/blood-2002-02-0487
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发表时间：
2002-11-01
期刊：
影响因子：
20.3
通讯作者：
Isaacson, PG
中科院分区：
文献类型：
--
作者：
Du, MQ;Diss, TC;Isaacson, PG
已知卡波西肉瘤相关疱疹病毒（KSHV）与3种不同的淋巴组织增生性疾病相关：原发性渗出性淋巴瘤（PEL）、多中心Castleman病（MCD）和MCD相关浆淋巴瘤。我们报告3例以前未描述的KSHV相关的淋巴组织增生性疾病。该疾病表现为局限性淋巴结肿大，并显示出良好的反应，化疗或放疗。组织学上，淋巴细胞增殖的特征是浆母细胞优先参与淋巴滤泡的生发中心，形成融合的聚集体。它们对CD 20、CD 27、CD 79 a、CD 138、BCL 6和CD 10呈阴性，但显示单型κ或λ轻链。在部分卵泡中发现了CD 10（+）CD 20（+）残留卵泡中心细胞簇。浆母细胞对KSHV和EBV均呈阳性反应，且大多数浆母细胞还表达病毒性白细胞介素-6（vIL-6）。出乎意料的是，整个组织切片或显微切割的KSHV阳性聚集体的分子分析表明免疫球蛋白（IG）基因重排的多克隆或寡克隆模式。浆母细胞中IG基因重排存在体细胞突变和克隆内变异，其中1例表达转换的IG重链（伊加），提示其来源于生殖中心B细胞。我们建议将这种独特的实体称为“KSHV相关的嗜生殖细胞淋巴增生性疾病”。"
Kaposi sarcoma-associated herpesvirus (KSHV) is known to be associated with 3 distinct lymphoproliferative disorders: primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma. We report 3 cases of a previously undescribed KSHV-associated lymphoproliferative disorder. The disease presented as localized lymphadenopathy and showed a favorable response to chemotherapy or radiotherapy. Histologically, the lymphoproliferation is characterized by plasmablasts that preferentially involved germinal centers of the lymphoid follicles, forming confluent aggregates. They were negative for CD20, CD27, CD79a, CD138, BCL6, and CD10 but showed monotypic kappa or lambda light chain. Clusters of CD10(+)CD20(+) residual follicle center cells were identified in some of the follicles. The plasmablasts were positive for both KSHV and EBV, and most of them also expressed viral interleukin-6 (vIL-6). Unexpectedly, molecular analysis of whole tissue sections or microdissected KSHV-positive aggregates demonstrated a polyclonal or oligoclonal pattern of immunoglobulin (Ig) gene rearrangement. The plasmablasts showed somatic mutation and intraclonal variation in the rearranged Ig genes, and one case expressed switched Ig heavy chain (IgA), suggesting that they originated from germinal center B cells. We propose calling this distinctive entity "KSHV-associated germinotropic lymphoproliferative disorder."