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血浆细胞白血病与 HIV 合并感染:南非布隆方丹大学附属医院患者特征及诊治经验。

Plasma cell leukaemia and HIV co-infection: profile of patients and experience at Universitas Academic Hospital in Bloemfontein, South Africa.

机构信息

Division of Anatomy, Department of Surgery, University of Toronto, Toronto, Canada,

出版信息

Int J Hematol. 2013 Dec;98(6):672-80. doi: 10.1007/s12185-013-1461-2. Epub 2013 Nov 21.

Abstract

Plasma cell leukaemia (PCL) is a rare condition with high mortality. HIV-positive patients have a propensity to develop malignancy; however, the occurrence of PCL with HIV infection in South Africa has not been documented. We describe patients with PCL in Universitas Hospital in Bloemfontein, South Africa, and report two new cases of HIV infection concurrent with PCL. A retrospective case series of PCL patients (2006-2012) seen at our Clinical Haematology unit is reported. Patient files were used to obtain information. The median age of patients (n = 9) was 51 years, and 66.7 % of cases were of African ethnicity. The condition was equally distributed between genders. Two patients were HIV positive. Both received combination antiretroviral therapy. The diagnosis of PCL was usually made as an incidental finding, subsequently confirmed on bone marrow aspirate and trephine. Deranged haematological and biochemical parameters, including severe anaemia, hypoalbuminaemia, and hyper-cellular bone marrow, were observed. Only one patient improved markedly on treatment, and remains alive at the time of writing. PCL shows poor response to treatment and predominates among Africans. The small sample size made it difficult to determine whether co-infection with HIV was a coincidental finding or the two diseases are pathophysiologically linked.

摘要

浆细胞白血病(PCL)是一种死亡率很高的罕见疾病。HIV 阳性患者有发生恶性肿瘤的倾向;然而,南非 HIV 感染合并 PCL 的发生尚未有文献记载。我们描述了在南非布隆方丹大学医院的 PCL 患者,并报告了两例新的 HIV 感染合并 PCL 病例。我们报告了我们的临床血液学部门在 2006 年至 2012 年间收治的 PCL 患者的回顾性病例系列。患者的档案被用来获取信息。9 名患者的中位年龄为 51 岁,66.7%的病例为非洲裔。该病症在性别上分布均匀。两名患者 HIV 阳性。两者均接受了联合抗逆转录病毒治疗。PCL 的诊断通常是偶然发现的,随后在骨髓抽吸和活检中得到证实。观察到血液学和生化参数的异常,包括严重贫血、低白蛋白血症和骨髓细胞增多。只有一名患者的治疗效果显著改善,并且在撰写本文时仍然存活。PCL 对治疗反应不佳,在非洲人中更为常见。由于样本量小,难以确定 HIV 合并感染是偶然发现还是两种疾病在病理生理学上有关联。

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