Mundada Manasi C, Ahmed Faiq, Khera Rachna, Murthy Sudha, Rajappa Senthil, Santa A, Mallavarapu Krishnamohan
Department of Laboratory Medicine, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, India.
Department of Medical Oncology, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, India.
South Asian J Cancer. 2020 Jun;9(2):105-108. doi: 10.1055/s-0040-1721194. Epub 2020 Dec 14.
Plasmablastic lymphoma (PBL) is a rare aggressive B cell lymphoma that is commonly encountered in patients with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS). In this case series, we describe the clinicopathological features of cases of PBL seen at a tertiary care center in South India. Medical records of patients diagnosed with PBL between January 2009 and November 2017 were reviewed. PBL was defined as per the World Health Organization 2016 classification for hematopoietic and lymphoid neoplasms. The slides were reviewed with hematoxylin and eosin along with immunohistochemistry (IHC) including CD45, CD20, PAX5, CD79a, CD3, CD5, CD138, MUMI, EMA, ALK, and Ki67. Epstein-Barr virus (EBV) association was documented by rapid in situ hybridization (RISH) studies wherever possible. The demographic data, clinical presentation, treatment details, and outcomes are elaborated using descriptive statistics. During the study period, nine patients with PBL were identified. The median age at presentation was 47 years (range: 36-54 years). All patients had associated HIV/AIDS, eight (89%) had extranodal disease, and six (66%) had advanced clinical stage (stage III). All biopsies were positive for CD45, CD138, and MUM1, and negative for CD79a and T cell markers with a high Ki67 proliferation index (85-90%); CD20 was faint positive in one patient, and CD56 was positive in one (11%) patient. EBV-RISH was tested in two patients and was positive in one. Bone marrow was uninvolved in all the cases. At the time of last follow-up, three patients were alive. Treatment details were available in six patients. With frontline therapy, four patients achieved a complete remission (CR) and one patient developed progressive disease. Three of four patients in CR are alive till the last follow-up. PBL is a rare form of lymphoma with predominant association with HIV, extranodal location, and characteristic IHC pattern.
浆母细胞淋巴瘤（PBL）是一种罕见的侵袭性B细胞淋巴瘤，常见于人类免疫缺陷病毒（HIV）/获得性免疫缺陷综合征（AIDS）患者。在本病例系列中，我们描述了在印度南部一家三级医疗中心所见的PBL病例的临床病理特征。
回顾了2009年1月至2017年11月期间诊断为PBL的患者的病历。PBL根据世界卫生组织2016年造血与淋巴组织肿瘤分类标准进行定义。对切片进行苏木精和伊红染色以及免疫组织化学（IHC）检查，包括CD45、CD20、PAX5、CD79a、CD3、CD5、CD138、MUM1、EMA、ALK和Ki67。尽可能通过快速原位杂交（RISH）研究记录爱泼斯坦-巴尔病毒（EBV）的相关性。使用描述性统计详细阐述人口统计学数据、临床表现、治疗细节和结果。
在研究期间，共确定了9例PBL患者。就诊时的中位年龄为47岁（范围：36 - 54岁）。所有患者均合并HIV/AIDS，8例（89%）有结外病变，6例（66%）处于临床晚期（III期）。所有活检标本CD45、CD138和MUM1均为阳性，CD79a和T细胞标志物均为阴性，Ki67增殖指数高（85 - 90%）；1例患者CD20弱阳性，1例（11%）患者CD56阳性。2例患者进行了EBV-RISH检测，1例阳性。所有病例骨髓均未受累。在最后一次随访时，3例患者存活。6例患者有治疗细节。一线治疗后，4例患者达到完全缓解（CR），1例患者疾病进展。4例CR患者中有3例存活至最后一次随访。
PBL是一种罕见的淋巴瘤形式，主要与HIV、结外部位和特征性IHC模式相关。
