PDF(Pubmed)
Abstract：
Multicentric Castleman disease (MCD) is a poorly understood, heterogeneous lymphoproliferative disorder with benign hyperplastic lymph nodes and systemic inflammatory symptoms. Human herpesvirus-8 (HHV-8) may be associated with MCD, whether or not the patient is infected with the human immunodeficiency virus (HIV). A 74-year-old man presented with anaemia, thrombocytopenia and bilateral axillary adenomegaly of unknown origin. The patient was admitted to the hospital two years ago with clinical signs of weight loss, asthenia, anorexia and a maculopapular rash on the trunk and back. Blood analysis showed pancytopenia (haemoglobin 7.7 g/dL, leucocytes 2.55 x 109/L and platelets 41 x 109/L), elevated acute phase reactants (such as C-reactive protein, erythrocyte sedimentation rate, ferritin and fibrinogen), hypoalbuminemia and hypergammaglobulinemia, and HIV serology was negative. Thoracic, abdominal and pelvic axial tomography showed generalised lymphadenopathy. The bone marrow biopsy showed only reactive changes, and the histology of an excisional biopsy of the adenopathy was consistent with the plasmablastic variant of MCD associated with HHV-8. The HHV-8 viral load was 3.8 x 104 copies/mL (4.5 log). He was started on prednisolone 60 mg/day and rituximab. He had a poor response to therapy, despite a reduction in the HHV-8 viral load, with clinical deterioration, transfusion-dependent anaemia and progression to multi-organ dysfunction leading to death three weeks after starting treatment. Our patient had a fulminant course of MCD despite treatment with rituximab. Further studies are needed to validate the different treatment modalities and to better understand the prognosis of this disease.
摘要：
多中心Castleman病（MCD）知之甚少，伴有良性增生性淋巴结和全身炎症症状的异质性淋巴增生性疾病。人类疱疹病毒-8（HHV-8）可能与MCD有关，患者是否感染了人类免疫缺陷病毒（HIV）。一名74岁的男子出现贫血,不明原因的血小板减少症和双侧腋窝腺肿大。该患者两年前因体重下降的临床症状入院,虚弱,厌食症和躯干和背部的斑丘疹。血液分析显示全血细胞减少（血红蛋白7.7g/dL，白细胞2.55x109/L和血小板41x109/L），急性时相反应物升高(如C反应蛋白，红细胞沉降率,铁蛋白和纤维蛋白原)，低蛋白血症和高丙种球蛋白血症，HIV血清学结果为阴性。胸科,腹部和盆腔轴位体层摄影术显示广泛性淋巴结肿大。骨髓活检显示只有反应性改变,淋巴结肿大的切除活检的组织学与与HHV-8相关的MCD的浆细胞变体一致。HHV-8病毒载量为3.8×104拷贝/mL(4.5log)。他开始使用泼尼松龙60mg/天和利妥昔单抗。他对治疗反应不佳，尽管HHV-8病毒载量减少，随着临床恶化，输血依赖性贫血和进展为多器官功能障碍导致开始治疗3周后死亡.尽管接受了利妥昔单抗治疗，我们的患者仍有暴发性MCD。需要进一步的研究来验证不同的治疗方式并更好地了解这种疾病的预后。
