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Lymphoma CNS

Bei Hu, Ryan Jacobs, Nilanjan Ghosh
PURPOSE OF THE REVIEW: The ligation of PD-1 with PD-L1 activates a critical immune checkpoint leading to T cell dysfunction, exhaustion, and tolerance. Anti-PD-1 or anti-PD-L1 monoclonal antibodies can reverse the immune checkpoint, releasing the brake on T cell responses. We provide a comprehensive review of the literature on the activity of checkpoint inhibitors in lymphoma. RECENT FINDINGS: We discuss the latest findings with checkpoint inhibitors in lymphoma and new promising studies incorporating these agents...
October 18, 2018: Current Hematologic Malignancy Reports
Sophie Hierlmeier, Matthias Eyrich, Matthias Wölfl, Paul-Gerhardt Schlegel, Verena Wiegering
Hematopoietic stem cell transplantation (HSCT) has been an effective method for treating a wide range of malignant or non-malignant disorders. In case of an autologous HSCT, patients receive their own stem cells after myeloablation before extraction. Allogeneic HSCT uses stem cells derived from a donor. Despite being associated with a high risk of early and long-term complications, it is often the last curative option. 229 pediatric patients, who between 1 January 2005 and 31 December 2015 received an HSCT at the University Children's Hospital Wuerzburg, were studied...
2018: PloS One
Lin Zhuang, Jie Lai, Kun Chen, Tianling Ding, Yan Yuan, Yan Ma, Hui Kang, Zhiguang Lin, Ni Fan, Jingjing Ma, Qingchao Zeng, Xiaoping Xu, Qingping Wang, Bobin Chen
The aim of the present study was to prospectively evaluate the recurrence and survival outcome of primary central nervous system lymphoma (PCNSL) with intraocular involvement. For this purpose, a prospective cohort of 103 pathologically confirmed patients with PCNSL was enrolled in this study. Ophthalmologic examinations were performed both at diagnosis and during follow‑up. The patients with PCNSL with suspected intraocular involvement underwent vitrectomy for confirmation. Patients who presented with intraocular involvement either at diagnosis or during disease progression were allocated to the intraocular lymphoma (IOL) group...
October 11, 2018: Oncology Reports
Muharrem Yunce, Nargiz Muganlinskaya, Stephen Selinger
INTRODUCTION: Intravascular large B-cell lymphoma (IVLBCL) is an uncommon disease with a poor prognosis if not diagnosed early. It can present with central nervous system (CNS) manifestations. The diagnosis of IVCBCL is difficult to make given its varied clinical manifestations and the lack of a specific diagnostic modality. CASE PRESENTATION: We report an interesting case of IVLBCL presenting as bilateral strokes. The diagnosis was made by a random skin biopsy, which confirmed IVLBCL...
October 2018: Medicine (Baltimore)
Liye Xu, Jin Cheng Song, Xiu Hua Sun, Zi Fen Gao, Li Lv, Jie Zhu
RATIONALE: Central nervous system (CNS) infiltration of Richter's syndrome (RS) is rare and only a few cases were discussed. Of these published cases, either they were accompanied with lymph node involvement or with a history of chronic lymphocytic leukemia (CLL). To our knowledge, this is the first published case of RS of the brain and meninges diagnosed concurrently with CLL in the absence of any evidence of lymphoma outside of the CNS. PATIENT CONCERNS: A 67-year-old female presented with slurred speech, headache, and left-sided hemiparesis...
October 2018: Medicine (Baltimore)
Pingyuan Wang, Jia Zhou
Great success has been achieved in small molecule drug discovery programs, making extraordinary contributions for human health, especially in targeted therapy. Taking anticancer drug discovery as an example, small molecules traditionally inhibit the target protein enzyme activities and induce cancer cell apoptosis through the target binding. However, the target protein within tumor cells often recovers its activities, leading to acquired drug-resistance through the overexpression of the target protein or the generation of new mutations in the target protein [1]...
October 9, 2018: Current Topics in Medicinal Chemistry
Anahita Nosrati, Ahmad Monabati, Alireza Sadeghipour, Fatemeh Radmanesh, Akbar Safaei, Sajjadeh Movahedinia
Primary central nervous system lymphoma (PCNSL) is a rare specific subtype of non-Hodgkin lymphoma limited to the brain, leptomeninges, spinal cord, or eyes without any systemic presentation and relapse which mostly takes place in CNS. In more than 95% of patients, it is of diffuse large B cell lymphoma (DLBCL) type. Categorizing PCNSL to germinal center cell like or activated B cell like, as we usually do for DLBCL NOS, may not be applicable for predicting outcome. Possible prognostic significance of MYC, BCL2, and/or BCL6 rearrangements may be important given what we know about their impact in systemic DLBCL, but we have limited knowledge about the status of double or triple hit molecular changes in PCNSL...
October 11, 2018: Annals of Hematology
Shaoxuan Hu, Yuqin Song, Xiuhua Sun, Liping Su, Wei Zhang, Jing Jia, Ou Bai, Sheng Yang, Rong Liang, Xiaoling Li, Huilai Zhang, Yuhuan Gao, Weijing Zhang, Xiubin Xiao, Huizheng Bao, Ningju Wang, Hanyun Ren, Xinan Cen, Shun'e Yang, Yu Zhao, Yinan Wang, Yalan Wang, Aichun Liu, Jingwen Wang, Yuankai Shi, Ming Yuan, Yufu Li, Xiaohui He
Primary breast diffuse large B-cell lymphoma(PB-DLBCL) is a rare subtype of DLBCL with limited data on patterns of failure. This multicenter study aimed to define the optimum treatment strategy and patterns of failure for PB-DLBCL patients. We retrospectively reviewed data on 108 PB-DLBCL patients from 21 Chinese medical centers. Only patients with localized disease (involvement of breast and localized lymph nodes) were included. After a median follow-up of 3.2 years, 32% of patients developed progression or relapse...
October 9, 2018: Cancer Science
Sarah J Nagle, Nirav N Shah, Alex Ganetsky, Daniel J Landsburg, Sunita D Nasta, Anthony Mato, Stephen J Schuster, Ran Reshef, Donald E Tsai, Jakub Svoboda
Aim: To describe the long-term outcomes of patients with lymphoma in the CNS treated with rituximab, temozolomide and high-dose methotrexate without consolidation therapy. Patients & methods: A retrospective cohort study of 46 consecutive patients with primary CNS lymphoma (PCNSL, 27 patients) or secondary CNS involvement of diffuse large B-cell lymphoma (DLBCL, 19 patients) who were treated with rituximab on day 1 in combination with high-dose methotrexate (days 1 and 15) and temozolomide (days 1-5) in 28-day cycles without further consolidation...
December 2017: International Journal of Hematologic Oncology
Fotini Debonera, Sunita Nasta, Maria Martinez-Lage, Stephen J Schuster, Donald E Tsai
There is no consensus on the optimal therapy for primary CNS lymphoma. Conventional treatment modalities include chemotherapy and radiation therapy, which carry significant risks of morbidity and mortality. In systemic lymphomas, there are situations where non-Hodgkin lymphomas have resolved spontaneously. We now report the case of a nonimmunocompromised patient with primary CNS lymphoma who underwent a spontaneous remission with a durable response. This case suggests that not all patients with primary CNS lymphomas require aggressive treatment with chemotherapy and radiation therapy...
November 2017: International Journal of Hematologic Oncology
Jessica Davies, Michael Martinec, Mathieu Coudert, Paul Delmar, Gracy Crane
BACKGROUND: The anaplastic lymphoma kinase (ALK) treatment landscape is crowded following recent ALK inhibitor approvals, and updated information on real-world treatment patterns in advanced non-small-cell lung cancer (aNSCLC) with ALK rearrangement (ALK+) is needed. METHODS: This retrospective US cohort study used Flatiron Health's longitudinal electronic health record (EHR)-derived database. Patients (≥18 years old) diagnosed with stage IIIB/IV aNSCLC, with documented ALK rearrangement and ≥2 visits after January 1, 2011 were followed until February 28, 2016...
October 8, 2018: Current Medical Research and Opinion
Soheil Zorofchian, Guangrong Lu, Jay-Jiguang Zhu, Dzifa Y Duose, Justin Windham, Yoshua Esquenazi, Leomar Y Ballester
Primary Central Nervous System Lymphoma (PCNSL) and Metastatic (or Secondary) Central Nervous System Lymphoma (MCNSL) are rare central nervous system (CNS) malignancies that exhibit aggressive clinical behavior and have a poor prognosis. The majority of CNS lymphomas are histologically classified as diffuse large-B cell lymphoma (DLBCL). DLBCL harbors a high frequency of mutations in MYD88 and CD79b . The MYD88 p.L265P mutation occurs at high frequency in CNS lymphoma and is extremely rare in non-hematologic malignancies...
2018: Frontiers in Oncology
Sara Degl'Innocenti, Nicola Della Camera, Cristian Falzone, Carlo Cantile
Intravascular lymphoma (IVL) is a rare angiotropic large-cell lymphoma in which neoplastic lymphocytes proliferate within the lumina of small blood vessels in the absence of a primary extravascular mass or leukemia. This study included 10 cases of canine IVL restricted to the CNS. Dogs had an average age of 8 years and neurological signs mainly referred to brain involvement such as depression, seizures, and ambulatory deficits. Gross examination at necropsy showed focal extensive or multiple hemorrhagic areas mainly distributed in the telencephalon and diencephalon...
October 5, 2018: Veterinary Pathology
Julia Vogt, Rabea Wagener, Manuel Montesinos-Rongen, Ole Ammerpohl, Werner Paulus, Martina Deckert, Reiner Siebert
Primary lymphomas of the central nervous system (PCNSL) are diffuse large B-cell lymphomas (DLBCL) confined to the CNS. We here performed array-based DNA methylation analyses of 26 PCNSL and 78 DLBCL and validated our findings in an independent dataset. We identified 2847 CpGs differentially methylated between PCNSL and non-CNS-DLBCL. Neither a supervised analysis using these CpGs nor application of three CpG classifiers selected for class separation unambiguously separated PCNSL from non-CNS-DLBCL. Remarkably, 6/78 non-CNS-DLBCL consistently segregated with PCNSL, which displayed molecular features typical for PCNSL...
October 3, 2018: Genes, Chromosomes & Cancer
Han W Tun, Patrick B Johnston, Lisa M DeAngelis, Pamela J Atherton, Levi D Pederson, Patricia A Koenig, Craig B Reeder, Antonio M Padula Omuro, David Schiff, Brian O'Neill, Jose Pulido, Kurt A Jaeckle, Christian Grommes, Thomas E Witzig
The combination of pomalidomide (POM) and dexamethasone (DEX) was evaluated for relapsed/refractory primary CNS lymphoma (PCNSL) and primary vitreo-retinal lymphoma (PVRL) to determine the maximal tolerated dose (MTD) of POM as the primary objective, and overall response rate (ORR), progression-free survival (PFS), and safety profile as the secondary objectives. Cohorts-of-three study design was used with dose escalation schedule consisting of POM (3 mg, 5 mg, 7 mg, or 10 mg) PO daily for 21 days every 28 days and DEX 40 mg PO every week (#NCT01722305, ClinicalTrials...
September 27, 2018: Blood
Patrick Bärtschi, Enrique Luna, Pablo González-López, Javier Abarca, Joaquín Herrero, Estela Costa, Artemio Paya, Juan Sales, Pedro Moreno
BACKGROUND: Langerhans cell histiocytosis (LCH) is a multi-systemic dendritic cell proliferation relatively uncommon in adults. Central nervous system (CNS) LHC outside the pituitary is even more uncommon. CASE DESCRIPTION: We report a case of a 42-years-old male complaining of a right side hemicranial pain and left arm minor paresis due to a right insular lobe heterogeneous enhancing lesion associated with extensive vasogenic edema. The first diagnostic impression suggested a glioblastoma multiforme or a localized metastasis...
September 24, 2018: World Neurosurgery
Erika Yamada, Eiichi Ishikawa, Rei Watanabe, Hideaki Matsumura, Noriaki Sakamoto, Akiko Ishii, Akira Tamaoka, Keiichiro Hattori, Naoshi Obara, Shigeru Chiba, Kiyotaka Nakamagoe, Masahide Matsuda, Takao Tsurubuchi, Yasushi Tomidokoro, Hiroyoshi Akutsu, Alexander Zaboronok, Makoto Shibuya, Shingo Takano, Akira Matsumura
OBJECTIVE: To evaluate the effectiveness of random skin biopsies for intravascular large B-cell lymphoma (IVLBCL) with (IVLBCL+CNS) or without central nervous system (IVLBCL-CNS) involvement. PATIENTS AND METHODS: Data from 21 patients with suspected IVLBCL (IVLBCL+CNS and 14 IVLBCL-CNS) who underwent single (4 patients), double (1 patient), and random (16 patients) skin biopsies were retrospectively analyzed. RESULTS: 16 patients (including 6 IVLBCL+CNS) were diagnosed with IVLBCL...
September 24, 2018: World Neurosurgery
Lorenz Thurner, Klaus-Dieter Preuss, Moritz Bewarder, Maria Kemele, Natalie Fadle, Evi Regitz, Sarah Altmeyer, Claudia Schormann, Viola Poeschel, Marita Ziepert, Silke Walter, Patrick Roth, Michael Weller, Monika Szczepanowski, Wolfram Klapper, Camelia Monoranu, Andreas Rosenwald, Peter Möller, Sylvia Hartmann, Martin-Leo Hansmann, Andreas Mackensen, Henning Schäfer, Elisabeth Schorb, Gerald Illerhaus, Rolf Buslei, Rainer Maria Bohle, Stephan Stilgenbauer, Yoo-Jin Kim, Michael Pfreundschuh
To address the role of chronic antigenic stimulation in PCNSL we searched for auto-antigens and identified SAMD-14 and neurabin-I as auto-antigenic targets of the B-cell receptors (BCRs) from 8/12 PCNSLs. In the respective cases SAMD14 and neurabin-I were atypically hyper-N-glycosylated (SAMD14 at ASN339 and neurabin-I at ASN1277) explaining their auto-immunogenicity. SAMD14 and neurabin-I induced BCR pathway activation and proliferation of aggressive lymphoma cell lines transfected with SAMD14 and neurabin-I reactive BCRs...
September 24, 2018: Blood
Shuang Ma, Siddhartha Sen Sen, Rachel Jug, Xuefeng Zhang, Wan-Lin Zhang, Shuai Shen, Cheng-Qian Yu, Hong-Tao Xu, Lian-He Yang, Endi Wang
RATIONALE: Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma with a dismal outcome. Most patients relapse in intracranial sites and <5% of patients relapse in extracranial sites. Here, we present the first case of PCNSL with an adrenal relapse. PATIENT CONCERNS: A 72-year-old woman, first presented 7 years ago with complaints of headache and dizziness. DIAGNOSES: Enhanced magnetic resonance imaging revealed the mass within the splenium of the corpus callosum...
September 2018: Medicine (Baltimore)
Lisa Modelevsky, Richard Tizon, Samantha N Reiss, Marcel Smith, Rachel Garonce, Thomas Kaley
AIM: To establish the safety and feasibility of rapidly infusing rituximab over 90 min in patients with primary CNS lymphoma (PCNSL). PATIENTS & METHODS: We retrospectively reviewed all patients with PCNSL who received rapid rituximab infusions (RRI) from January 2016 to January 2017. Primary end point was incidence of infusion reactions. RESULTS & CONCLUSION: 11 patients received a total of 44 RRIs. Rituximab was dosed at 500 or 750 mg/m2 ...
July 1, 2018: CNS Oncology
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