关键词: HHV8-associated lymphoproliferative disorders HHV8-positive DLBCL KSHV Multicentric Castleman disease

Mesh : Male Humans Aged Herpesvirus 8, Human Sarcoma, Kaposi / complications pathology Castleman Disease / complications pathology Ascites / etiology Lymphoma, Large B-Cell, Diffuse / complications diagnosis Lymphadenopathy

来  源:   DOI:10.1016/j.prp.2024.155185

Abstract:
Herein, we report a rare case of Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8)-positive diffuse large B-cell lymphoma (DLBCL), which is characterized by malignant ascites and complex karyotypes. A 72-year-old male patient who tested negative for human immunodeficiency virus presented with thrombocytopenia and lymphadenopathies. He was diagnosed with KSHV/HHV8-associated multicentric Castleman disease (MCD). After three years, he developed progressive lymphadenopathies and massive ascites. The lymphoma cells in the ascitic fluid presented with characteristic immunophenotype and monoclonality, which support the diagnosis of KSHV/HHV8-positive DLBCL. Lymphadenopathies and massive splenomegaly are common manifestations of KSHV/HHV8-positive DLBCL. Nevertheless, peritoneal involvement, as observed in this case, is a rare presentation. This emphasizes the diagnostic complexities of KSHV/HHV8-associated lymphoproliferative disorders. Within the context of preexisting KSHV/HHV8-associated multicentric Castleman disease, the differential diagnosis of this disorder can be challenging.
摘要:
在这里,我们报告了罕见的卡波西肉瘤疱疹病毒/人类疱疹病毒8(KSHV/HHV8)阳性弥漫性大B细胞淋巴瘤(DLBCL),其特征是恶性腹水和复杂的核型。一名72岁的男性患者,对人类免疫缺陷病毒检测呈阴性,表现为血小板减少症和淋巴结病。他被诊断患有KSHV/HHV8相关的多中心Castleman病(MCD)。三年后,他发展为进行性淋巴结病和大量腹水。腹水中的淋巴瘤细胞具有特征性的免疫表型和单克隆性,这支持KSHV/HHV8阳性DLBCL的诊断。淋巴结病和大量脾肿大是KSHV/HHV8阳性DLBCL的常见表现。然而,腹膜受累,正如在这种情况下观察到的,这是一个罕见的演讲。这强调了KSHV/HHV8相关淋巴增生性疾病的诊断复杂性。在先前存在的KSHV/HHV8相关的多中心Castleman病的背景下,这种疾病的鉴别诊断具有挑战性.
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