Case Report: Concurrent primary CNS lymphoma and meningothelial meningioma - nuances of diagnosis and management

Background: The incidence of two distinct primary intracranial pathologies is an exceedingly rare phenomenon. Although meningiomas are well known to coexist with other primary intracranial malignancies there are only nine reported cases of a meningioma occurring simultaneously with primary CNS lymphoma in the literature. We report a case of a woman who sustained multiple injuries due to two distinct intracranial pathologies, however, lateralizing signs were unrecognized for two weeks prior to her final diagnosis. Case Description: A 64-year-old female with history of diabetes mellitus type 2 initially presented to the Emergency Department, two weeks prior, following a mechanical fall at home resulting in a left bimalleolar fracture. CT imaging revealed a right occipital mass with significant vasogenic edema causing 12mm of midline shift. MRI revealed two distinct homogeneously contrast-enhancing lesions: a right occipital mass with dural-based attachment, as well as a homogenously contrast-enhancing lesion adjacent to the right posterolateral ventricle. FLAIR signal changes were also appreciated and were noted to extend across the corpus callosum, raising concerns for a high-grade glial process. She underwent a right occipital craniotomy with gross total resection of the right occipital mass as well as subtotal resection and biopsy of the second lesion. Final pathology of the extra-axial lesion was found to be meningothelial meningioma and the deep lesion was found to be diffuse large B-cell lymphoma. Discussion: We describe a rare instance of simultaneous meningioma and primary CNS lymphoma that was found to be the underlying cause of a traumatic injury several weeks after the incident. We review the current diagnosis and management nuances in the setting of multiple intracranial oncologic processes.

Download Full-text

Case Report: Concurrent primary CNS lymphoma and meningothelial meningioma - nuances of diagnosis and management

F1000Research ◽

10.12688/f1000research.17770.2 ◽

2019 ◽

Vol 8 ◽

pp. 103

Author(s):

Samir Kashyap ◽

Jacob Bernstein ◽

Ira Bowen ◽

Rosalinda Menoni ◽

Dan Miulli

Keyword(s):

Vasogenic Edema ◽

Traumatic Injury ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

Final Diagnosis ◽

Subtotal Resection ◽

Cns Lymphoma ◽

Diagnosis And Management ◽

Deep Lesion ◽

The Right

Download Full-text

First Results of the Acsé Pembrolizumab Phase II in the Primary CNS Lymphoma (PCNSL) Cohort

Blood ◽

10.1182/blood-2020-141773 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 15-16

Author(s):

Khe Hoang-Xuan ◽

Roch Houot ◽

Carole Soussain ◽

Marie Blonski ◽

Anna Schmitt ◽

...

Keyword(s):

Objective Response ◽

Primary Cns Lymphoma ◽

Central Nervous System Lymphoma ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

High Dose ◽

Moderate Activity ◽

Multicentric Study ◽

First Results ◽

Duration Of Response

Background: AcSé Pembrolizumab is a Phase 2, open-label, single-arm, multi-cohort, multicentric study investigating the efficacy and safety of pembrolizumab monotherapy in patients with advanced rare cancers (NCT03012620). Here, we report the first results of Pembrolizumab in the cohort of Primary Central Nervous System Lymphoma (PCNSL). Methods: Main inclusion criteria were: relapsed or refractory PCNSL after one or several lines of treatment including high dose Methotrexate based chemotherapy, pathologically confirmed diffuse large B cell lymphoma, age>18, HIV negative, concurrent steroid medication at a dose no greater than prednisone 20 mg/day or equivalent. Patients received pembrolizumab 200 mg IV as a 30-minute infusion on Day 1 of every 21-day cycles for a maximum of 2 years. The primary endpoint was the confirmed objective response rate according to IPCG at 84 day after the start of treatment. Secondary endpoints included best response (ORR), duration of response, progression-free survival (PFS), overall survival (OS), and safety. Analysis used all enrolled patients. Results: 50 patients suffering from PCNSL, including 9 primary vitreoretinal lymphoma (PVRL) were included from July, 2017 to October, 2019. Median age was 72 years (range: 43 to 83), Median PS (ECOG) was 1 (range 0-1). The median number of cycles was 4 (range 1-35). At 84 days from start of treatment, 6 patients responded (4 CR+2PR). Overall, 3 patients whose response was not assessed were considered as failures, and the rates of ORR (CR+PR), stable disease (SD), progressive disease (PD) were 26% (13/50, 8 CR + 5 PR), 10% (5/50), 58% (29/50), respectively. ORR was 29% (12/41) and 11% (1/9) in primary cerebral lymphoma and PVRL respectively. After a median follow-up of 6.7 months (range 0.2-27.4), median PFS was 2.6 months, with 6-month PFS of 29.8% and 6-month OS of 60.4%. In responders, median duration of response was estimated at 10 months (95%CI, 2.7 to 12.5). Grade III and IV toxicities related to the drug were observed in 4 patients (8%) and one patient (2%) respectively. No related toxic death was reported. Conclusion: Pembrolizumab shows moderate activity in relapsed/ refractory PCNSL with acceptable toxicity, supporting further studies evaluating its use in combination therapies. Disclosures Hoang-Xuan: BTG: Consultancy, Research Funding. Houot:Bristol-Myers Squibb: Honoraria; MSD: Honoraria; Gilead: Honoraria; Kite: Honoraria; Roche: Honoraria; Novartis: Honoraria; Janssen: Honoraria; Celgene: Honoraria. Schmitt:Celgene: Membership on an entity's Board of Directors or advisory committees; Roche, Janssen: Honoraria. Ahle:Roche: Honoraria; Novartis: Honoraria; Biogene: Honoraria; Abbvie: Honoraria; Sanofi: Honoraria. Bories:Abbvie: Consultancy; Celgen: Consultancy; Gilead: Consultancy; BMS: Honoraria; Novartis: Honoraria. Houillier:BTG: Consultancy.

Download Full-text

P.108 Diffuse large B-cell Lymphoma secondary to iatrogenic immunosuppression with unusual MRI findings and literature review

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.209 ◽

2016 ◽

Vol 43 (S2) ◽

pp. S45-S45 ◽

Cited By ~ 1

Author(s):

AH Naeem ◽

MD Staudt ◽

B Wang ◽

D Lee ◽

A Parrent

Keyword(s):

Literature Review ◽

B Cell ◽

Cell Lymphoma ◽

Immunosuppressive Agents ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

Mri Findings ◽

Axial Mass ◽

Large B Cell

Background: Immunosuppressive therapy is a risk factor for lymphoproliferative disorders. We present a case of primary CNS B-cell lymphoma in the setting of iatrogenic immunosuppression from azathioprine usage. A literature review is provided. Methods: Case report Results: 64-year-old male presents with several weeks of cognitive decline, impaired speech, and headache with a history of ulcerative colitis (on azathioprine and 5-ASA) with no radiological evidence of systemic malignancy. MR showed left frontal extra-axial mass (4.0 x 2.4 x 4.0 cm) with heterogeneous enhancement of a solid component with local dural thickening. The enhancing mass had solid and cystic components. Radiological differential included dural metastasis, atypical meningioma or unusual intra-axial mass including GBM with some dural involvement. He underwent surgical resection, which showed a primary CNS lymphoma, diffuse large B-cell, CD 20 + and EBV +. Post-operatively his cognition improved. Azathioprine was stopped and 5-ASA was increased. He proceeded with MPVC (methotrexate, procarbazine, vincristine, and cytarabine) chemotherapy. Conclusions: Our case shows isolated extra-nodal CNS manifestation of lymphoma in the context of immunosuppressive medications with strikingly atypical MR findings leading to a pre-operative diagnostic dilemma. Treatment is challenging and needs to be individually tailored due to a need for stopping immunosuppressive agents in conjunction with CNS lymphoma treatment.

Download Full-text

Central nervous system involvement in patients with diffuse large B cell lymphoma: analysis of the risk factors and prognostic from a single-center retrospective cohort study

10.21203/rs.2.13767/v1 ◽

2019 ◽

Author(s):

Jingjing Ma ◽

Qing Li ◽

Jie Shao ◽

Yan Ma ◽

Zhiguang Lin ◽

...

Keyword(s):

Cell Lymphoma ◽

Elevated Serum ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

Absolute Count ◽

Cns Involvement ◽

Systemic Involvement ◽

Large B Cell Lymphoma ◽

Significant Difference ◽

Deep Lesion

Abstract Purpose The aim of this study was to identify the risk factors for central nervous system (CNS) involvement in systemic diffuse large B-cell lymphoma (DLBCL) patients and to explore prognostic for DLBCL patients with CNS involvement (relapse or progression). Method This was a retrospective cohort study in our hospital. Data were collected from all DLBCL patients diagnosed in our institutes from January, 2013 to June, 2018. Clinical information was collected from medical records. Result The participants included 138 patients with DLBCL. Among them, 38 patients were diagnosed as CNS lymphoma, including 15 patients exhibited CNS involvement while DLBCL were pathologically confirmed, and 23 patients developed CNS lymphoma during or after initial chemotherapy. The median disease-free interval to CNS involvement was 13 months. Multivariate analysis identified elevated serum lactate dehydrogenase(LDH) level [hazard ratio(HR)=4.035; 95% confidence interval(95%CI):1.147~14.195] was independent predictor of CNS involvement. The median progression-free survival (PFS) and overall survival (OS) time of DLBCL patients with CNS involved were 12.5 months and 22 months, respectively. Multivariate prognostic analysis showed that eastern cooperative oncology group (ECOG) score>2(P=0.018; HR=7.333; 95%CI:1.424~42.002), elevated serum LDH level (P=0.046; HR=6.510; 95%CI:1.035~40.949), deep lesion (P=0.005; HR=10.957; 95%CI:2.050~58.569), and CNS with systemic involvement (P=0.023; HR=2.730; 95%CI:1.151~6.479) were independent poor prognostic factors for the patients. The cases with lymphocyte absolute count >0.75×109/L (HR=0.047; 95%CI:0.003~0.732) had better prognosis. The OS of DLBCL patients with secondary CNS lymphoma was inferior to DLBCL patients without CNS involvement. There was no significant difference between the patients with CNS and extra-CNS involvement. There was no significant difference between the patients with CNS involvement and stage III-IV DLBCL cases without CNS lymphoma. Conclusion In conclusion, elevated serum LDH was independent high-risk factor for secondary CNS lymphoma. For patients DLBCL with CNS involvement, ECOG score>2, elevated serum LDH level, deep lesion, lymphocyte absolute count ≤0.75×109/L and CNS with systemic involvement retained a significant association with outcome.

Download Full-text

Del(6)(q22) and BCL6 Rearrangements in Primary CNS Lymphoma Are Indicators of an Aggressive Clinical Course

Journal of Clinical Oncology ◽

10.1200/jco.2008.16.1455 ◽

2008 ◽

Vol 26 (29) ◽

pp. 4814-4819 ◽

Cited By ~ 79

Author(s):

Francois M. Cady ◽

Brian Patrick O'Neill ◽

Mark E. Law ◽

Paul A. Decker ◽

David M. Kurtz ◽

...

Keyword(s):

Survival Data ◽

Proportional Hazards ◽

Deep Structure ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

Aggressive Lymphoma ◽

Cns Lymphoma ◽

High Dose ◽

Putative Tumor Suppressor Gene ◽

Thin Sections

Purpose Primary CNS lymphoma (PCNSL) is an aggressive lymphoma but clinically validated biologic markers that can predict natural history to tailor treatment according to risk are lacking. Several genetic changes including BCL6 rearrangements and deletion of 6q22, containing the putative tumor suppressor gene PTPRK, are potential risk predictors. Herein we determined the prevalence and survival impact of del(6)(q22) and BCL6, immunoglobulin heavy chain (IGH), and MYC gene rearrangements in a large PCNSL cohort treated in a single center. Patients and Methods Interphase fluorescence in situ hybridization was performed using two-color probes for BCL6, MYC, IGH-BCL6, and del(6)(q22) on thin sections of 75 paraffin-embedded samples from 75 HIV-negative, immunocompetent patients newly diagnosed with PCNSL. Survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and proportional hazards regression adjusting for age, deep structure involvement, and high-dose methotrexate (HDMTX) treatment. Results The prevalence of del(6)(q22) and BCL6, IGH, and MYC translocations was 45%,17%, 13%, and 3%, respectively. The presence of del(6)(q22) and/or a BCL6 translocation was associated with inferior overall survival (OS; P = .0097). The presence of either del(6)(q22) alone or a BCL6 translocation alone was also associated with inferior OS (P = .0087). Univariable results held after adjusting for age, deep structure involvement, and HDMTX. Conclusion Del (6)(q22) and BCL6 rearrangements are common in PCNSL and predict for decreased OS independent of deep structure involvement and HDMTX. Unlike systemic diffuse large B-cell lymphoma, del(6)(q22) is common and IGH translocations are infrequent and usually involve BCL6 rather than BCL2, suggesting a distinct pathogenesis.

Download Full-text

Clinicopathologic and Genetic Profile of Intracranial Marginal Zone Lymphoma: A Primary Low-Grade CNS Lymphoma That Mimics Meningioma

Journal of Clinical Oncology ◽

10.1200/jco.2005.17.624 ◽

2005 ◽

Vol 23 (24) ◽

pp. 5718-5727 ◽

Cited By ~ 88

Author(s):

Pang-hsien Tu ◽

Caterina Giannini ◽

Alexander R. Judkins ◽

Jason M. Schwalb ◽

Richard Burack ◽

...

Keyword(s):

Marginal Zone ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

Genetic Profile ◽

Low Grade ◽

Barr Virus ◽

Genetic Features ◽

Trisomy 3 ◽

Epstein Barr

Purpose Although rare overall, marginal zone B-cell lymphoma (MZBCL) is the most common primary low-grade CNS lymphoma reported in the literature. The aim of this study is to elucidate the biology and genetic features of this unusual tumor. Patients and Methods Fifteen CNS MZBCLs were studied clinically, pathologically, and genetically, including fluorescent in situ hybridization analyses with commercially available MALT1 and IgH break-apart and centromere 3, 7, 12, and 18 probes. Results CNS MZBCLs preferentially affect middle-aged women (female-to-male ratio, 4:1), with 93% presenting as dural-based masses mimicking meningioma. Ten patients with 1 to 7.6 years of follow-up after diagnosis showed no evidence of disease after radiation and/or chemotherapy. Like MZBCLs outside of the CNS, they consisted of CD20+, CD3− small B lymphocytes with varying degrees of plasmacytic differentiation and predominantly κ light-chain restriction (78%). Lymphoid follicles with follicular colonization were seen in three patients and deposition of amyloid was noted in samples from two patients, one of which was tumefactive. Neither Bcl-6 protein nor Epstein-Barr virus–encoded RNA was expressed. Trisomy 3 was detected in six of 12 patients, with no rearrangements of MALT1 or IgH and no trisomies of 7, 12, or 18 detected. Conclusion Our data suggest that intracranial MZBCL is an indolent primary CNS lymphoma that typically presents as a meningioma-like dural-based mass. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality that may contribute to the pathogenesis of this CNS lymphoma.

Download Full-text

PC3 - 128 Clinical Profile and Treatment Outcomes of Patients with Primary CNS Lymphoma in a Tertiary Hospital in the Philippines: An Eight-Year Retrospective Review

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.388 ◽

2016 ◽

Vol 43 (S4) ◽

pp. S20-S20

Author(s):

M.C. Concepcion Sales

Keyword(s):

Survival Rate ◽

Combination Chemotherapy ◽

Single Agent ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

The Philippines ◽

Clinical Profile ◽

Treatment Modalities ◽

Cns Lymphoma ◽

Female Ratio

Primary CNS Lymphoma (PCNSL) is an unusual extranodal form of Non-Hodgkin’s lymphoma with a locally aggressive course but a rare tendency to disseminate systemically. It has been documented in that the clinical characteristics and response to treatment among Asians is comparable to the Western population yet no studies done locally are available. Objectives: This study aims to determine the clinico-pathologic profile of patients diagnosed with PCNSL seen at Philippine General Hospital (PGH) from January 2006 to September, 2014 and to evaluate the patients’ response to the following treatment modalities: 1) Combination chemotherapy 2) Chemo-RT 3) Single agent chemotherapy and 4) no specific anti-lymphoma treatment. Methodology: This is a descriptive and retrospective study that included all cases of histologically-proven PCNSL seen at the PGH from January 2006 to September, 2014. The clinical profile, imaging studies and biopsy findings were obtained from the patient records. The survival rates at the end of one and two years of diagnosis were computed. Results and Conclusion. Among patients diagnosed with PCNSL at PGH, there is a higher incidence of PCNSL among males with a male to female ratio of 1.4:1 and have a younger onset with a median age of 50.2 years. Most patients presented with signs of increase ICP and majority had solitary cortical lesions with histopathologic diagnosis of diffuse large B cell lymphoma. Patients who did not undergo any form of treatment had a mean survival of 10 months. Immunocompromised patients had a shorter life-span with a mean survival of 7.5 months. Treatment of combination chemotherapy with HD-MTX and Rituximab had the most favorable outcome followed by HD-MTX only with a 2 year survival rate of 100% and 66% respectively while patients who underwent chemo-RT had a 2 year survival rate of 33% with a high incidence of neurocognitive delay.

Download Full-text

PATH-60. PRIMARY CNS LYMPHOMA PRECEDED BY ACUTE DEMYELINATION

Neuro-Oncology ◽

10.1093/neuonc/noz175.655 ◽

2019 ◽

Vol 21 (Supplement_6) ◽

pp. vi156-vi157

Author(s):

Justin Low ◽

Anne Buckley ◽

Dina Randazzo

Keyword(s):

Oral Prednisone ◽

Primary Cns Lymphoma ◽

Brain Biopsy ◽

B Cell Lymphoma ◽

High Grade Glioma ◽

Acute Disseminated Encephalomyelitis ◽

Cns Lymphoma ◽

Central Demyelination ◽

Initial Biopsy ◽

Radiographic Improvement

Abstract Primary CNS lymphoma commonly presents with nonspecific encephalopathy or focal neurologic deficits. Magnetic resonance imaging typically shows a homogeneously enhancing mass with surrounding edema. The imaging differential diagnosis is broad, and includes high grade glioma and neuroinflammatory conditions. Definitive diagnosis therefore requires biopsy. Here we present a case of primary CNS lymphoma that was diagnosed as an acute demyelinating process on initial biopsy. A 68 year old female presented with gait instability and vertigo. MRI showed right cerebellar and right trigonal enhancing lesions. Biopsy revealed an acute demyelinating inflammatory process and she was diagnosed with acute disseminated encephalomyelitis. She was treated with intravenous methylprednisolone followed by oral prednisone with resulting clinical and radiographic improvement. She was re-admitted to hospital 4 months later with encephalopathy. Imaging showed a new enhancing mass in the pericallosal frontal lobes. Repeat brain biopsy showed diffuse large B-cell lymphoma. This case illustrates a highly unusual situation of biopsy-proven central demyelination preceding a primary CNS lymphoma diagnosis. It raises a number of etiopathological questions concerning the coexistence and potential causal relationships between demyelination and lymphoma. Additionally, it highlights the need for repeat biopsy if clinical and radiographic suspicion for lymphoma persists despite an alternative initial biopsy result.

Download Full-text

No Benefit from Rituximab Containing Regimens in Patients with Primary Extranodal Diffuse Large B-Cell Lymphoma

Blood ◽

10.1182/blood.v112.11.3615.3615 ◽

2008 ◽

Vol 112 (11) ◽

pp. 3615-3615

Author(s):

Gonzalo Gutiérrez-García ◽

Luis Colomo ◽

Neus Villamor ◽

Leonor Arenillas ◽

Antonio Martínez ◽

...

Keyword(s):

B Cell ◽

Cell Lymphoma ◽

International Prognostic Index ◽

Primary Cns Lymphoma ◽

Prognostic Index ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

Large B Cell Lymphoma ◽

The Impact ◽

Large B Cell

Abstract Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous category of lymphoid tumors that comprises different clinical forms not fully recognized in the WHO classification. In this regard, extranodal (EN) DLBCLs have particular clinicobiological features and outcome, sometimes related to the specific site where the lymphoma arises. Nowadays, rituximab plus chemotherapy (CT) is the gold-standard in the treatment of DLBCL. However, the superiority of rituximab-CT (R-CT) over CT alone has not been addressed for all the clinical subsets of the disease and, in fact, the clinical role of the new therapies might be different for primary nodal or EN DLBCLs. The aim of this study was to assess the impact of rituximab in patients suffering from nodal or EN DLBCL. Two-hundred and thirty non-immunocompromised patients (112M/118F; median age, 61 years) diagnosed with CD20+DLBCL in a single institution between 1997 and 2006 (five years before and after establishing R-CT as the standard treatment in DLBCL) and treated with adriamycin-containing regimens were the subject of the present study. The series included 148 primary nodal and 82 EN DLBCL. Patients with primary CNS lymphoma were excluded and lymphomas arising at Waldeyer ring were considered as nodal DLBCL. The main EN sites were GI (n=26), bone (n=13), soft tissue (n=13), lung/pleura (n=9), liver (n=9), and other (n=12). Main clinico-biological and evolutive variables were analyzed. One hundred nineteen patients received only CT and 111 R-CT. Eighty-seven cases with available information were assigned to germinal center B-cell-like (GCB) (41%) or non-GCB (59%) groups according to the Hans method (Blood2004;103:275–82) based on CD10, BCL6 and MUM1 expression. Main initial features, including the primary nodal or EN origin, international prognostic index (IPI), and GCB/non-GCB categories were similar for CT and R-CT groups. No correlation was observed between the GCB/non-GCB groups and the primary site of the tumor, although nodal lymphomas more frequently expressed MUM1 than EN (69% vs. 31%, respectively; p=0.01). CR rate and 5-year overall survival (OS) according to the treatment arm (CT vs. R-CT) is detailed for the whole series and for the nodal and EN groups in the table and OS curves depicted in the figure. In the whole series, variables predicting poor OS in the multivariate analysis were high-risk IPI (RR 2.5; p<0.001), primary nodal involvement (RR 1.6; p=0.04) and no R-CT treatment (RR 1.9; p=0.002). In the nodal group, IPI and no R-CT maintained the prognostic value, whereas in the primary EN only IPI predicted OS. Moreover, no difference in OS was observed according to the nodal or EN origin in those patients receiving R-CT. Biological subtypes GCB vs. non-GCB did not add predictive information neither in the whole series nor in the nodal or EN groups. In conclusion, patients with primary EN DLBCL seem to have little benefit from the use of R-CT. Nevertheless, this intriguing observation should be confirmed in further prospective studies. Complete response CR (%) 5-years OS (%) CT R-CT CT R-CT *p<0.002 R-CT vs. CT All cases (n=230) 59 79* 46 70* Primary nodal (n=148) 54 78* 34 71* Primary extranodal (n=82) 68 78 70 69 Figure Figure

Download Full-text

The Analysis of HIV-Associated Lymphoma in Japan

Blood ◽

10.1182/blood-2018-99-114060 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 5315-5315

Author(s):

Shotaro Hagiwara ◽

Kentaro Yoshinaga ◽

Masayuki Shiseki ◽

Junji Tanaka ◽

Seiji Okada

Keyword(s):

Board Of Directors ◽

Burkitt Lymphoma ◽

Research Funding ◽

Cell Lymphoma ◽

Primary Cns Lymphoma ◽

B Cell Lymphoma ◽

Cns Lymphoma ◽

Advisory Committees ◽

Large B Cell Lymphoma ◽

Large B Cell

Abstract Background. The recent advance of antiretroviral therapy decreased the morbidity of opportunistic infections. However, the incidence of HIV-associated lymphoma remains high. Also, the outcome of the HIV-associated lymphoma is unclear in the era of rituximab. In order to address these clinical questions, we performed a nation-wide epidemiological study. Methods. Patients with HIV-associated lymphoma were extracted from the database of Japanese society of hematology blood disease registry from January 2012 to December 2015. We analyzed the patient's age, sex, subtypes of lymphoma, the international prognostic index (IPI) for diffuse large B cell lymphoma, and overall survival. Results. Eighty-one patients were extracted from the database. Eighty patients were available for the survival analysis. Seventy-six (93.8%) patients of them were male. The median age was 52.5(25-88) year-old. However, there were two peaks of age; the first peak was 38-40-year-old and the second was 59-62-year-old. Sub-types of lymphomas were diffuse large B cell lymphoma (DLBCL)(48.1%), Burkitt lymphoma(19.8%), primary CNS lymphoma(8.6%), plasmablastic lymphoma(7.4%), peripheral T cell lymphoma(3.7%), Hodgkin's lymphoma(3.7%), primary effusion lymphoma(2.5%), MALT lymphoma(1.2%), Follicular lymphoma(1.2%) and Adult T cell lymphoma/leukemia(1.2%). Extra-nodal involvement at the diagnosis was observed in 61.7%. The involved sites were the brain, stomach, small bowel, colon, thyroid and the others. In DLBCL, the patients with IPI high and high-intermediate risk was 51.3%. The median observation period was 26 months. Estimated 3 years overall survival (OS) in all cases was 68.8+/-0.63%. Although there was no statistical significance, however, the 3 years, OS of Burkitt lymphoma tended to be better than that of DLBCL (84.6%+/-10.0 versus 67.7+/-8.8%). Log-rank analysis showed the OS in DLBCL patients with IPI high-intermediate and high risk was significantly worse than the patients with low, and low-intermediate risk (p<0.001). Estimated 3 years OS was 90+/-9.5% vs. 38.0+/-13.0%, respectively. The outcome of patients with primary CNS lymphoma remains poor, estimated 3 years OS was 45.7+/-22.4%. Conclusion. Our study showed diversity in the pathological subtype of HIV lymphoma. In the era of rituximab, the outcome seemed to be improved in patients with DLBCL and Burkitt lymphoma. However, the survival remains short in patients with poor prognostic factors and primary CNS lymphoma. Figure. Figure. Disclosures Hagiwara: Celgene: Membership on an entity's Board of Directors or advisory committees; Bristol Myers Squibb: Membership on an entity's Board of Directors or advisory committees. Shiseki:NOVARTIS Pharma: Honoraria, Research Funding; Bristol-Myers Sqibb: Honoraria; Otsuka: Speakers Bureau. Tanaka:Novartis Pharma: Honoraria; Bristol-Myers Squibb: Honoraria, Research Funding; Otsuka: Honoraria; Pfizer: Honoraria.

Download Full-text