HIV-negative Plasmablastic Lymphoma Research Articles

HIV-negative plasmablastic lymphoma sustaining long-term response after autologous peripheral blood stem cell transplantation

The patient is a 34-year-old HIV antibody-negative female with normal immunocompetence. The patient was referred to the hospital of the current study due to diarrhea and abdominal pain, which developed in May 2014. On conducting computed tomography (CT), remarkable wall thickening was noted in the terminal ilium over the ascending colon, suggesting a malignant tumor. However, making a definite diagnosis by lower gastrointestinal endoscopic biopsy and left hemicolectomy was not possible. The dense proliferation of plasma cell-like cells and plasmablasts was noted; CD20, CD19, CD79a, CD3, CD4, and Epstein-Barr virus-encoded miRNAs (EBER) were negative and CD138 was positive on immunostaining. Based on the aforementioned data, the patient was diagnosed with plasmablastic lymphoma (PBL). High-dose chemotherapy combined with autologous peripheral blood stem cell transplantation (PBSCT) was performed in the first remission period after the completion of four cycles of hyper CVAD/MTX-AraC alternating therapy. Remission was confirmed by FDG-PET/CT 3 months after autologous PBSCT. No signs of recurrence have been observed in 6 years after the transplantation. Although no standard treatment for PBL has been established, autologous peripheral blood stem cell transplantation combined with high-dose chemotherapy during the first remission period may be a beneficial treatment option.

Clinicopathologic Comparison of Plasmablastic Lymphoma Patients According HIV-Status: Peruvian Experience

Background:Plasmablastic lymphoma (PBL) is an aggressive lymphoma associated mainly to HIV infection, although cases in immunocompetent patients are described as well. Objective:To describe the clinicopathologic features and determine the overall survival of lymphoma patients according human immunodeficiency virus (HIV) status in Peruvian patients. Methods:We reviewed the pathology databases of 2 cancer centers and a general hospital from Peru. Forty cases were documented between 2005 and 2020. Categorical variables were compared using Fisher's exact test. Survival curves were estimated using the Kaplan-Meier method and compared using the log-rank test. Statistical analysis was based on SPSS Program version 23. All cases were reviewed by two pathologists. Results:32 patients (80%) were HIV-positive. The median age for the whole cohort was 40 years (range, 22-86). The median age for HIV-positive and HIV-negative PBL patients were 37 years (range 22-67 years) and 57 years (range 27-86 years), respectively. The proportion of patients ≥60 years was lower in HIV-positive than in HIV-negative patients (8% and 37%, respectively; p= 0.046). 80% of patients in the whole cohort were female, and 84% and 62% in the HIV-positive and HIV-negative group, respectively. Extra-oral primary sites were the most frequent primary sites in both groups (66% and 88%, respectively). There were no statistically significant differences in Ann Arbor stage, Ki-67 expression, LDH levels, IPI scores, albumin levels, and neutrophil/lymphocyte ratio between HIV-positive and HIV-negative patients. All cases showed large lymphoid cells, of plasmablastic morphology with expression of at least one plasma cell marker (CD138, CD38, MUM1), CD45 positivity, variable expression of EMA, CD79 and CD30 and absence of expression of CD20, CD3, CD68 and LMP1; the proliferative index Ki67 varied between 60 and 90%. A small proportion of patients (18%) did not receive chemotherapy because of poor performance status or a personal decision. DA-EPOCH regimen was used in 52% and 25% of HIV+ and HIV- patients, respectively and CHOP/CHOEP regimen in 48 % and 75%, respectively. The overall response rate was 68% and 57% in HIV+ and HIV- patients with complete response (CR) in 32% and 14%, respectively. In the HIV-positive group, 66% of patients were antiretroviral therapy (ART)-naïve. The median progression-free survival (PFS) was 38 months and 7 months for HIV+ and HIV- patients, and the 1-year PFS was 74% and 0%, respectively. The median overall survival (OS) was 43 months (range 0.2-86.5) in HIV-positive patients and 10 months (range 0.5-19.0 months) in HIV-negative patients and the 1-year OS were 59% y 38%, respectively (p=0.27). Conclusions: PBL is a rare lymphoma, specially, if not related to HIV infection. In this study, 60 years or older was the only variable that showed significant difference. In our cohort, HIV-positive patients had better prognosis than HIV-negative PBL patients. Disclosures No relevant conflicts of interest to declare.

Unilateral Maxillary Sinus Plasmablastic Lymphoma in an Immunocompetent Patient. An Unusual Occurrence Report and Literature Review.

Plasmablastic lymphoma (PBL) is a rare and aggressive form of mature B cell neoplasms almost exclusively identified in patients infected with the human immunodeficiency virus (HIV). The small number of HIV-negative PBL cases reported in the literature to date is composed of single case reports and small case series which characteristically are present involving the oral cavity mucosa or gingiva. We present a 72-year-old HIV-negative Australian patient without any cause of immunodeficiency, with an isolated left maxillary sinus PBL.

Clinical characteristics and survival analysis of 15 cases of HIV-negative plasmablastic lymphoma

目的分析HIV阴性的浆母细胞淋巴瘤（PBL）患者的临床病理特征及转归。方法回顾性分析海军军医大学附属长海医院2013年1月至2019年8月诊断及治疗的15例HIV阴性PBL患者的临床病理资料及随访结果。结果15例HIV阴性PBL患者中男8例，女7例，中位年龄59（17~69）岁，均无明确的免疫抑制状态。所有患者均出现结外受累，Ann Arbor-Cotswolds分期Ⅰ、Ⅱ、Ⅲ、Ⅳ期分别为1例（6.7%）、2例（13.3%）、3例（20.0%）、9例（60.0%）。病理形态表现为类似免疫母细胞的大肿瘤细胞弥漫性增生，免疫组化示肿瘤细胞主要表达浆细胞标志CD38、CD138、Mum-1，B细胞标志CD20、CD10、PAX-5、BCL-6少见，Ki-67中位数为80%（70%~90%）。3例患者行EBER原位杂交检测，1例阳性。15例患者均接受化疗，80%（12/15）联合硼替佐米作为一线治疗。最佳疗效6例完全缓解（其中2例分别于治疗后4.9和26.1个月疾病进展），2例部分缓解（分别于治疗后3.0和6.8个月疾病进展），4例疾病进展，3例无法评估。中位随访时间30.3（4.8~61.1）个月，11例患者中位无进展生存（PFS）期6.8（95%CI 2.5~11.1）个月，预计3年PFS率为21.2%（95%CI 1.4%~56.8%）。所有患者中位总生存（OS）期为17.9（95%CI 5.6~30.2）个月，预计3年OS率为38.5%（95%CI 12.0%~65.0%）。结论HIV阴性PBL侵袭性强，结外受累常见，多数患者诊断时分期较晚。通过以硼替佐米为基础的强化疗方案序贯自体造血干细胞移植可望获得长期生存。

HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases.

BackgroundHuman immunodeficiency virus (HIV)-negative plasmablastic lymphoma (PBL) is a rare entity of diffuse large B-cell lymphoma (DLBCL). The clinicopathological features of and optimal treatment for HIV-negative PBL remain largely unknown.MethodsTo gain insight into this distinct lymphoma, we summarized the clinicopathologic characteristics of 8 unpublished HIV-negative PBLs and performed a comprehensive review of 394 published cases.ResultsOf the 8 unpublished PBLs, the median patient age was 53.0 years. Four patients presented with stage IV disease. All 8 patients showed a plasma cell-like immunophenotype. Of the six patients who received anthracycline-based chemotherapy, including two who received bortezomib, three patients achieved a continuous complete response, two patients died due to disease progression, and one patient was lost to follow-up. The other two patients achieved continuous complete response after receiving chemotherapy combined with radiotherapy and surgery. Of the 402 patients, the majority were male, with a mean age of 58.0 years. EBV infection was detected in 55.7% of the patients. The median survival times of the patients who received CHOP or CHOP-like regimens and intensive regimens were not reached and 23.0 months, respectively, and the intensive regimen did not improve the survival outcome (P=0.981). Multivariate analysis showed that EBER remained the only independent factor affecting overall survival (OS).ConclusionHIV-negative PBL is a distinct entity with a predilection for elderly and immunosuppressed individuals. Intensive chemotherapy had no apparent survival benefits over the CHOP regimen in terms of OS; the prognosis of this disease is poor with current chemotherapy methods, and treatment remains a challenge.

A Rare Case of Plasmablastic Lymphoma with Gastric Involvement

Plasmablastic lymphoma (PBL) is a relatively rare, but highly aggressive lymphoma commonly found in the setting of HIV disease.PBL is defined by the World Health Organization as a “diffuse proliferation of large neoplastic cells most of which resemble B-cell immunoblasts, but in which all tumor cells have a plasma cell immunophenotype.”Here we present a unique case of Ann Arbor Stage IV PBL with extranodal gastric involvement diagnosed in an immunocompetent male. An 80-year-old Hispanic male presented to the emergency department with nausea and epigastric pain for four days duration. The patient also endorsed progressive dysphagia to solids and liquids over the past several weeks prior to presentation. Significant findings on physical exam included a pale, well-nourished male with mild epigastric tenderness on light palpation. CT scan revealed diffuse thickening of the gastric wall with a 7.4cm x 6.5cm intraluminal infiltrating mass with luminal narrowing. An EGD was subsequently performed for better visualization and biopsy (Figure 1). Histopathologic studies ultimately identified the mass as malignant plasmablastic lymphoma with aberrant cytokeratin expression. HIV testing was negative but EBV IgG antibody testing was abnormally high. Immunophenotype analysis revealed that CD-138 was strongly positive, CD-20 was negative, CD-45 was weakly positive, and Ki-67 expression was greater than 90% (Figure 2). The patient was started on CHOP chemotherapy regimen.2659_A Figure 1. Large, 5cm, semi-circumferential, easily friable and fungating mass as observed from GE junction during EGD2659_B Figure 2. Histology and Immunophenotype Analysis. (A) High-power magnification showing atypical plasma cells. (B) CD-20 negative. (C) CD-138 positive. (D) Ki-67 Index > 90%.Recent meta-analysis has shown that the median age of diagnosis for HIV-negative PBL is 55 years. Although the pathogenesis of PBL remains incompletely understood, the association with EBV infection and the virus' role in prevention of apoptosis of B cells is well documented.EBV positivity and CD45 expression represent good prognostic indicators for this patient; on the other hand, Ann Arbor Stage IV classification and Ki-67 expression > 80% are typically associated with poor outcome. After the oral mucosa, the GI tract is the most common site of involvement for HIV-negative PBL cases. This case represents a rare phenotypic variant in which the BCL-6 transcription factor is positive, suggesting that perhaps this case is a hybrid of diffuse large B-cell lymphoma (DLBCL) and PBL. Despite the recent advances in the treatment for PBL, prognosis remains poor with the median overall survival of nine months and a 2-year overall survival rate of 10% for HIV-negative PBL.

Clinical characteristics and survival analysis of eight cases HIV-negative plasmablastic lymphoma

目的提高对人免疫缺陷病毒（HIV）阴性浆母细胞淋巴瘤的认识。方法回顾性分析北京协和医院1997年1月至2015年5月确诊的8例HIV阴性浆母细胞淋巴瘤患者的临床资料，分析其临床特征及转归。结果8例HIV阴性浆母细胞淋巴瘤中男3例，女5例，中位年龄60（43~80）岁，其中4例存在导致免疫功能低下的疾病或状态。8例患者均有结外受累，2例Ann Arbor分期为Ⅰ~Ⅱ期，6例为Ⅳ期，其中5例有骨髓受累。所有患者均弥漫表达CD38和CD138，B细胞标志包括PAX-5及Bcl-6少见。5例患者进行EBV-DNA检测，均为阴性。接受化疗并规律随访的7例患者中位随访36（11~57）个月，中位无进展生存时间为15（6~52）个月，中位总生存时间为36（2~52）个月；其中4例采用了硼替佐米联合化疗，3例有效，但疗效难以维持，分别于治疗后2、9、21个月疾病进展。2例Ⅰ~Ⅱ期患者均治疗有效，未出现疾病进展，持续存活；5例Ⅳ期患者化疗后虽然有效，但疗效难以维持，中位总生存时间仅12（6~52）个月，中位无进展生存时间仅10（2~21）个月。结论该组HIV阴性浆母细胞淋巴瘤患者以中老年为主，临床呈现高侵袭性，均出现结外（尤其是骨髓）受累，其免疫表型与浆细胞瘤较为接近，分期较晚的患者预后不良。

Clinical characteristics of HIV-negative plasmablastic lymphoma

Clinical characteristics of HIV-negative plasmablastic lymphoma

Clinical features and outcomes: analysis of 9 cases of HIV-negtive plasmablastic lymphoma

目的评价人免疫缺陷病毒（HIV）阴性的浆母细胞淋巴瘤（PBL）的临床特征。方法回顾性分析中国医学科学院肿瘤医院2006年1月至2016年1月收治的具有完整临床资料的9例HIV阴性PBL患者临床资料。结果9例HIV阴性PBL患者中，男6例，女3例，中位年龄为56 (30~77）岁，均不存在免疫抑制相关的基础疾病。7例原发于结外，其中1例（例4）口腔受侵。Ann Arbor分期：Ⅱ期2例，Ⅳ期7例；9例患者Ki-67指数为60%~90%，≥80％者7例；4例患者进行EB病毒编码的小RNA（EBER）检测，2例EBER阳性。9例患者均接受化疗，8例一线采用CHOP（环磷酰胺＋表阿霉素＋长春新碱＋泼尼松）样方案，仅5例获得缓解（完全缓解1例，部分缓解4例）。其中3例老年患者采用CHOP联合沙利度胺方案，2例获得部分缓解。1例3个化疗方案失败的患者（例7）四线接受沙利度胺＋足叶乙甙治疗，疗效评价为疾病稳定，并维持10个月。1例早期患者（例4）无病生存61.9个月，其余8例一线治疗后均出现复发或进展，其中6例因疾病进展在诊断后2年内死亡。结论本组HIV阴性PBL患者均无明确的免疫抑制因素。原发结外病变常见，但仅1例口腔受侵。多数患者分期晚、治疗效果差。沙利度胺用于PBL的治疗值得进一步研究。

Bortezomib, Ifosfamide, Carboplatin, and Etoposide in a Patient with HIV-Negative Relapsed Plasmablastic Lymphoma.

Plasmablastic lymphoma (PBL) is a rare subtype of diffuse large B cell lymphoma (DLBCL), often associated with HIV infection. We present a case of a 53-year-old HIV-negative man with untreated hepatitis C viral infection who presented with abdominal pain and lymphadenopathy. Lymph node and bone marrow biopsies were consistent with plasmablastic lymphoma. He had partial response (PR) to 6 cycles of EPOCH but disease progressed seven weeks later. Repeat biopsy was consistent with plasmablastic lymphoma. Three cycles of bortezomib, ifosfamide, carboplatin, and etoposide (B-ICE) chemotherapy resulted in a partial response (PR). Five months later, he presented with widespread lymphadenopathy and tumor lysis syndrome with circulating blasts. Flow cytometry revealed a different population of lymphoma cells, this time positive for CD5, CD19, CD20, and CD22, with dim expression of CD45 and CD38. The patient died on the first day of ESHAP chemotherapy. There are no treatment recommendations or standard of care for plasmablastic lymphoma. A literature search yielded 10 cases in which bortezomib was administered in either HIV-positive or HIV-negative PBL. Six reported a partial response, 3 reported a complete response, and 1 was a near-complete response. Bortezomib, in combination with chemotherapy, may be an effective treatment option in PBL as reported here.

Clinicopathologic analysis of HIV-negative plasmablastic lymphoma

To study the clinical, pathologic, immunophenotype, molecular characteristics and prognosis of HIV-negative plasmablastic lymphoma (PBL). Twelve cases of HIV-negative PBLs diagnosed between 2005 and 2014 in Guangdong General Hospital were identified according to WHO classification of tumors of haematopoietic and lymphoid tissues (2008). The clinicopathologic features and outcome were analyzed and the relevant literatures were reviewed. The patients were predominantly male (11/12) with a median age of 55.5 years. The tumor cells showed the characteristic combination of immunoblastic/plasmablastic morphology, plasma cell phenotype and high proliferation, no expression of mature B cell markers. 7/10 of the cases were EBER positive. Two cases were positive for C-myc translocation. Four of twelve patients were died. PBL is a rare, aggressive B-cell lymphoma. HIV-negative PBL has lower rate of oral involvement and EBER expression than HIV-positive patients, the differential diagnosis is very challenging, and the prognosis is worse.

Human immunodeficiency virus-negative plasmablastic lymphoma: a comprehensive analysis of 114 cases.

Human immunodeficiency virus-negative plasmablastic lymphoma (PBL) is an extremely rare entity. Its clinicopathological features, optimal treatment strategy and prognostic factors remain obsure. An extensive search was performed in the English language literature within the Pubmed database using the key words: ‘plasmablastic lymphoma and human immunodeficiency virus-negative or immunocompetent’. Data from 114 patients from 52 articles were analyzed. The mean patient age at diagnosis was 58.90 years (range, 2–86). HIV-negative PBL showed a predilection for elderly individuals (patients older than 60 years, 56.14%) and affected more males than females (M:F, 2.29:1). Ann Arbor stage IV patients accounted for 39.22% while bone marrow involvement was less frequent (12.79%). The Ki-67 index was high with a mean expression of 83%. Epstein-Barr virus (EBV) infection was common being positive in 58.70% of the patients while herpesvirus-8 (HHV-8) infection was rare being positive in only 7.55% of the patients. Immunosuppression was noted in 28.16% of patients. The median overall survival (OS) was 19 months. The 1- and 2-year survival rates were 52.3 and 45.3%, respectively. Age, gender and primary site showed no strong relationship with OS while Immunosuppression, Ann Arbor stage IV and EBV negativity were able to predict a poorer OS. Either complete remission (CR) or partial remission (PR) was superior to the refractory group in OS (P<0.0001 and P=0.0066, respectively). For stage I patients, the application of radiotherapy did not improve the OS. In conclusion, HIV-negative PBL is a distinct entity likely occurring in elderly and immunosuppressed individuals. Immunosuppression status, Ann Arbor stage IV, EBV negativity and refractory to treatment are poor prognostic factors of OS in HIV-negative PBL.

Plasmablastic Lymphoma in the Testis and Duodenum: A Case Report and Literature Review

Plasmablastic lymphoma (PBL) is a rare and highly aggressive subtype of non-Hodgkin’s lymphoma (NHL) associated with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV). There are over 300 reported cases of PBL, of which over 120 are reported in HIV-positive patients. Of HIV-negative patients, patients were either immunocompetent or immunosuppressed transplant recipients. Initial reports highlighted cases arising in the oral cavity of HIV patients and this remains the most prevalent site of disease. However, other sites such as the gastrointestinal tract and skin are the next most common sites. Only three cases have involved the testes, a sanctuary site. Of gastrointestinal sites, there are only a handful of cases with gastric involvement. In the pre-human antiretroviral therapy (HAART) era, median overall survival was dismal. However, prognosis has improved since their advent. There is no significant difference in the survival of HIV-positive and HIV-negative PBL patients. It should be emphasized that due to the scarcity of cases, there are no established standards of care or prospective therapeutic trials for the management and treatment of PBL. Chemotherapy remains the standard approach, though selecting a regimen is still controversial and while response has been good, overall survival remains poor. Since 1995, autologous hematopoietic cell transplantation (AHCT) has been the standard of care for relapsed chemosensitive HIV-negative NHL. In the era of HAART, this has become feasible for HIV patients. A few studies have suggested that use of AHCT in the first line setting and relapsed or refractory disease may confer better outcomes. Herein we describe a unique case of involvement in the testis and duodenum, and there are no previous reports of a patient presenting with both gastrointestinal and urogenital involvement. J Hematol. 2015;4(3):196-201 doi: http://dx.doi.org/10.14740/jh217w

Human immunodeficiency virus-negative plasmablastic lymphoma in the neck: a rare case report and literature review

Plasmablastic lymphoma (PBL) is an aggressive neoplasm exclusively occurring in AIDS patients. Recently, increasing cases of human immunodeficiency virus (HIV)-negative PBL have been reported. No standard therapy protocol is currently available since there is a great difference between PBL with and without HIV infection. Here, we present a rather rare case of HIV-negative PBL in the neck that dramatically responded to radiotherapy alone. Our case highlights the possibility of PBL in the neck and helps to expand our understanding of this separate lymphoma. The related literature review summarized the clinicopathological features and treatment status of HIV-negative PBL.

Plasmablastic lymphoma of the maxillary sinus in an HIV-negative patient: a case report and literature review

Plasmablastic lymphoma (PBL) is a rare and aggressive variant of diffuse large B cell lymphoma. The prognosis of PBL patients is poor. The majority of patients succumb to a fulminant disease course, with most dying in the first year after diagnosis. The small number of HIV-negative PBL cases reported in the literature to date is composed of single case reports and small case series. Consequently, the natural history of the disease in HIV-negative individuals and the optimum treatment are not well characterized. Intensive induction chemotherapy has been associated with marked improved overall survival. However the optimal regimen has not been defined. We describe the third case of PBL of the maxillary sinus which occurred in a 24-year old HIV-negative man. We outline the clinicopathological features and report success using a hyper-CVAD regimen with 6 cycles and consolidation radiation therapy yielding a complete remission of four years.

Plasmablastic lymphoma of the elderly: a clinicopathological comparison with age‐related Epstein–Barr virus‐associated B cell lymphoproliferative disorder

Plasmablastic lymphoma (PBL) is an aggressive lymphoma with a terminally differentiated B cell phenotype; half of patients with this disease have Epstein-Barr virus (EBV) infection. The majority of PBL cases are associated with human immunodeficiency virus (HIV) infection, while the remaining HIV-negative cases were accompanied by other immunodeficiency conditions or immunosenescence in the elderly. To characterize HIV-negative PBL of the elderly (PBL-E), we compared the clinicopathological characteristics of 10 cases of PBL-E and 124 cases with age-related EBV-associated B cell lymphoproliferative disorder (AR-EBVLPD). The 10 PBL-E (eight men, two women; median age: 68 years) were associated with a more indolent clinical behaviour and a better overall survival than AR-EBVLPD. Extranodal involvement was higher in PBL-E (50%) than AR-EBVLPD; notably, the nasal cavity was affected most frequently in PBL-E (60%). Immunoglobulin heavy chain/(IGH)/MYC translocation was detected in half of the PBL-E cases. PBL-E shares some clinical features with AR-EBVLPD, such as HIV negativity, old age, and EBV infection, no known immunosuppressive condition but there are some differences such as a higher ratio of extranodal involvement and better prognosis. PBL-E is a newly recognized condition and should be distinguished from HIV-positive PBL, sharing features with AR-EBVLPD in particular, immunosenescence of the elderly.

Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma: A single institutional experience and literature review

Plasmablastic lymphoma (PBL) is a rare aggressive B-cell lymphoproliferative disorder. HIV-negative PBL has not been extensively reported. Nine HIV-negative PBL patients evaluated at Moffitt Cancer Center were studied. Eight patients had extranodal diseases. All patients were treated with CHOP or hyper-CVAD. Responses were observed in 8 cases (7 complete, 1 partial responses). Four patients underwent consolidation with autologous hematopoietic stem cell transplant (HSCT) in first complete remission (CR1). At median follow-up of 23.9 months, 7 patients were alive and 5 were disease-free. Aggressive induction chemotherapy and consolidation with autologous HSCT in CR1 might be considered for patients with HIV-negative PBL.

HIV-Negative Plasmablastic Lymphoma: Not in the Mouth

Plasmablastic lymphoma (PBL) is an aggressive variant of non-Hodgkin lymphoma initially reported in the oral cavity of HIV-positive individuals. Since its original description, several cases have been reported in patients who do not have HIV infection. However, despite its recognition as a distinct subtype of diffuse large B-cell lymphoma several years ago, comprehensive reviews of this entity are lacking. A MEDLINE search through June 2010 was performed to identify cases with a pathologic diagnosis of HIV-negative PBL based on morphology and minimal immunohistochemical criteria. Our study included a total of 76 cases. The median age was 57 years (range, 1 to 90 years) with a male-to-female ratio of 1.7. Seventy-four percent of cases did not have an apparent association with immunosuppression, 18% had a concurrent lymphoproliferative or autoimmune disorder and 9% developed PBL after solid organ transplantation. Oral involvement was observed in 21%, advanced stage in 60%, Epstein-Barr virus-encoded RNA expression was positive in 45% and Ki-67 expression of greater than or equal to 80% in 61% of the cases. Chemotherapy was documented in 43 patients, from which 43% received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimens. The median and the 2-year overall survival for the whole group were 9 months and 10%, respectively. Patients who had HIV-negative PBL have distinct clinicopathological characteristics, such as short overall survival and lower rates of oral involvement and Epstein-Barr virus-encoded RNA expression than the previously reported in HIV-positive patients.

Plasmablastic lymphoma of the cecum: Report of a case with cytologic findings

Plasmablastic lymphoma (PBL) is a rare lymphoma that is characterized by a diffuse proliferation of large neoplastic cells resembling B immunoblasts, but shows the immunophenotype of plasma cells. PBL is most commonly seen in the oral cavity of human immunodeficiency virus (HIV)-positive patients. Epstein-Barr virus (EBV) may be closely related the pathogenesis of PBL. We report a case of HIV-negative PBL in a 75-year-old man without EBV infection. Histologic examination of the cecal tumor following right hemicolectomy and cytologic examination of ascitic fluid were performed. Cytologic specimens were hypercellular and composed of single cells and loosely formed clusters. Large tumor cells showed plasmacytoid features with basophilic cytoplasm, large nuclei, prominent nucleoli, and focal perinuclear halos. Abnormal mitotic figures were easily identified. On immunohistologic study, the tumor cells were positive for CD138 (plasma cell marker) and kappa, but negative for CD45, CD3, CD20, CD79a, CD56, and cyclin D1. The proliferation index (Ki-67) was high. This is a very rare case of PBL without HIV and EBV infection, involving the cecum.

Bortezomib-Induced Tumor Lysis Syndrome in a Patient With HIV-Negative Plasmablastic Lymphoma

Plasmablastic lymphoma (PBL) is an aggressive lymphoma classified by the World Health Organization as a subtype of diffuse large B-cell lymphoma that shares many morphologic and immunophenotypic features with multiple myeloma. It is extremely rare in immunocompetent patients. Because of the small number of patients reported, this rare lymphoma remains a poorly characterized and understood entity with presently no standard recommendations regarding the optimal treatment. Herein, we report a dramatic clinical response coupled with tumor lysis syndrome to a bortezomib-based treatment in an HIV-negative patient with refractory plasmablastic lymphoma.