Fatal HHV-8-Associated hemophagocytic syndrome in an HIV-negative immunocompetent patient with plasmablastic variant of multicentric Castleman disease (plasmablastic microlymphoma)
Fatal HHV-8-Associated hemophagocytic syndrome in an HIV-negative immunocompetent patient with plasmablastic variant of multicentric Castleman disease (plasmablastic microlymphoma)
复制标题
DOI：
10.1097/01.pas.0000172293.59785.b4
复制
发表时间：
2006-01-01
影响因子：
5.6
通讯作者：
Chuang, SS
中科院分区：
文献类型：
--
作者：
Li, CF;Ye, HT;Chuang, SS
由HHV-8引发的病毒相关噬血细胞综合征（VAHS）非常罕见，仅在9名免疫功能低下患者中报告。我们报告了第一例HHV-8相关VAHS的HIV阴性，免疫功能正常的患者与浆母细胞瘤变异（浆母细胞瘤微淋巴瘤）的多中心Castleman病（MCD）。这位61岁的男性，以发烧、咳嗽和双侧腹股沟淋巴结肿大就诊。右侧腹股沟淋巴结活检显示浆母细胞MCD伴表达IgM、MUM 1、HHV-8潜伏相关核抗原和病毒白细胞介素-6的浆母细胞结节性聚集。这些浆母细胞对于IG λ轻链表达是单型的，而不是IG κ。所有B细胞克隆性试验，包括IgH-FR 2、IgH-FR 3、DH-JH、IG kappa和IG lambda PCR，均显示多克隆模式。他的血清人白细胞介素-6水平显著升高，EBV急性感染/再激活阴性。骨髓穿刺显示出鲜艳的噬血细胞。他的疾病迅速发展为多系统疾病，1个月后死于急性呼吸衰竭。我们的病例表明，HHV-8可能会触发VAHS在免疫功能正常的患者与血浆抗体MCD。我们推测，我们的患者在与增殖的HHV-8感染的浆母细胞相关的细胞因子风暴下发生VAHS，类似于EBV相关T细胞淋巴瘤患者中EBV触发的VAHS。
Virus-associated hemophagocytic syndrome (VAHS) triggered by HHV-8 is extremely rare and has been reported only in 9 immunocompromised patients. We report the first case of HHV-8-associated VAHS in an HIV-negative, immunocompetent patient with plasmablastic variant (plasmablastic microlymphoma) of multicentric Castleman disease (MCD). This 61-year-old man presented with fever, cough, and bilateral inguinal lymphadenopathy. Biopsy of the right inguinal lymph node revealed plasmablastic MCD with nodular aggregates of plasmablasts expressing IgM, MUM 1, HHV-8 latency-associated nuclear antigen, and viral interleukin-6. These plasmablasts were monotypic for Ig lambda light chain expression but not Ig kappa. All the B-cell clonality assays, including IgH-FR2, IgH-FR3, DH-JH, Ig kappa, and Ig lambda PCR, showed a polyclonal pattern. His serum human interleukin-6 level was markedly elevated and was negative for EBV acute infection/reactivation. The marrow aspirate showed florid hemophagocytosis. His disease progressed rapidly to multisystemic illness, and he died of acute respiratory failure in 1 month. Our case showed that HHV-8 might trigger VAHS in an immunocompetent patient with plasmablastic MCD. We speculated that our patient developed VAHS under the cytokine storm associated with the proliferating HHV-8-infected plasmablasts, similar to the EBV-triggered VAHS in patients with EBV-associated T-cell lymphoma.