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Sarah Löw, Tracy T Batchelor
Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma limited to the brain, spinal cord, leptomeninges, and eyes. The majority of patients are immunocompetent, with a median age of 65 years at diagnosis. Historically, whole-brain radiation therapy (WBRT) was the first and sole treatment for PCNSL. Today, due to the recognized neurotoxicity of WBRT, this modality is usually avoided in the treatment. Most chemotherapy regimens are based on high-dose methotrexate plus the anti-CD20 monoclonal antibody rituximab, leading to high response rates, but 5-year survival is still poor at approximately 30% compared with other extranodal lymphomas...
February 2018: Seminars in Neurology
Lesley Uttley, Iñigo Bermejo, Shijie Ren, Marrissa Martyn-St James, Ruth Wong, David L Scott, Adam Young, Matt Stevenson
As part of its Single Technology Appraisal process, the National Institute for Health and Care Excellence (NICE) invited the manufacturer (Pfizer) of tofacitinib (TOF; Xeljanz® ) to submit evidence of the drug's clinical and cost-effectiveness in the treatment of rheumatoid arthritis (RA) after the failure of conventional disease-modifying antirheumatic drugs (cDMARDs). The School of Health and Related Research Technology Appraisal Group at the University of Sheffield was commissioned to act as the independent Evidence Review Group (ERG)...
March 15, 2018: PharmacoEconomics
Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Reiko Yamada, Takashi Sakuno, Hiroyuki Inoue, Hiroshi Miura, Toshifumi Takeuchi, Yasunori Shiono, Hiroaki Okuse, Misaki Nakamura, Masaki Katsurahara, Yasuhiko Hamada, Kyosuke Tanaka, Noriyuki Horiki, Yoshiyuki Takei
A 49-year-old man was admitted to our hospital with pancreatitis. He was diagnosed with systemic lupus erythematosus at 34 years of age and was being treated with oral tacrolimus (3 mg/day) and predonine (10 mg/day) for the past 15 months. The computed tomography (CT) scan showed the mass lesion had invaded the pancreatic head via thickening of the duodenal wall. Upper gastrointestinal endoscopy showed the all-round ulcerative lesion from the superior duodenal angle to the descending portion. Histological examination confirmed the diagnosis of diffuse large B cell lymphoma (DLBCL)...
March 15, 2018: Clinical Journal of Gastroenterology
Pierre Feugier, Thérèse Aurran, Béatrice Mahé, Remi Letestu, Florence Nguyen-Khac, Bruno Cazin, Olivier Tournilhac, Hervé Maisonneuve, Olivier Casasnovas, Alain Delmer, Véronique Leblond, Bruno Royer, Bernadette Corront, Sylvie Chevret, Roselyne Delépine, Sandrine Vaudaux, Eric Van den Neste, Marie Christine Béné, Florence Cymbalista, Damien Roosweil, Stéphane Leprêtre
March 15, 2018: Haematologica
Alexandre Karras
Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease...
March 12, 2018: Néphrologie & Thérapeutique
Reibán-Espinoza Esteban, Bourlon Christianne, Aguayo Alvaro, Demichelis-Gómez Roberta
INTRODUCTION: The expression of the CD20 on adult B-cell acute lymphoblastic leukemia (ALL-B) has generally been associated with a poor prognosis, and several studies have explored the incorporation of rituximab into the therapeutic regimen for adult ALL-B patients, with a positive effect on event-free survival (EFS). PATIENTS AND METHODS: We analyzed the prognostic value of CD20 expression and the effect of rituximab for the treatment of Hispanic adult ALL-B patients...
February 26, 2018: Clinical Lymphoma, Myeloma & Leukemia
Thomas Erblich, Silvia Montoto
Advanced follicular lymphoma (FL) remains an incurable disease, at least with conventional therapy. Despite the long remission and disease-free periods that can be currently achieved with treatment, the disease will ultimately relapse in most of the patients. Areas covered: This article reviews the current spectrum of therapies for patients with relapsed FL in the rituximab area. It will revisit current therapies, approaches to therapeutic decision making and novel agents under investigation. Expert Commentary: At present, rituximab or second generation anti-CD20 antibodies either as single agent or in combination with chemotherapy, anti-CD20 maintenance therapy and stem cell transplant remain effective treatment options for relapsed patients...
March 15, 2018: Expert Review of Hematology
C G Bien
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy...
March 13, 2018: Der Nervenarzt
Clive M Michelo, Bram van Cranenbroek, Peran Touw, Frans H J Claas, Arnold van der Meer, Irma Joosten
Background: Antibody-mediated rejection in solid organ transplantation is an important immunological barrier to successful long-term graft survival. Next to complement activation, natural killer (NK) cells have been implicated in the process. Human cytomegalovirus (CMV), independently associated with decreased graft survival, has a strong imprint on the immune response. Here, we assessed the effect of CMV status on alloreactive NK cell reactivity. Methods: We compared antibody-mediated NK cell cytolytic activity (CD107a expression) and IFNγ production between healthy CMV-seropositive (n = 8) and CMV-seronegative (n = 11) individuals, in cocultures of NK cells with anti-HLA class I or rituximab (control) antibody-coated Raji cells...
December 2017: Transplantation Direct
Sandesh Parajuli, Didier A Mandelbrot, Brenda Muth, Maha Mohamed, Neetika Garg, Fahad Aziz, Robert R Redfield, Weixiong Zhong, Brad C Astor, Arjang Djamali
Background: There is limited information on treatment strategies and monitoring strategies for late antibody-mediated rejection (ABMR) after kidney transplantation. Methods: In this observational and nonrandomized study, we compared 78 patients diagnosed with late ABMR (>3 months after transplant) who were treated with standard of care steroids/IVIG (n = 38) ± rituximab (n = 40) at our center between March 1, 2013 and December 31, 2016. All patients had follow-up biopsy and donor-specific antibodies (DSA) monitoring within 3 to 12 weeks...
December 2017: Transplantation Direct
Guillaume Moulis, Grégory Pugnet, Nathalie Costedoat-Chalumeau, Alexis Mathian, Gaëlle Leroux, Jonathan Boutémy, Olivier Espitia, Laurence Bouillet, Sabine Berthier, Jean-Baptiste Gaultier, Pierre-Yves Jeandel, Amadou Konaté, Arsène Mékinan, Elisabeth Solau-Gervais, Benjamin Terrier, Daniel Wendling, Fanny Andry, Camille Garnier, Pascal Cathébras, Laurent Arnaud, Aurore Palmaro, Patrice Cacoub, Zahir Amoura, Jean-Charles Piette, Philippe Arlet, Maryse Lapeyre-Mestre, Laurent Sailler
OBJECTIVES: To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP). METHODS: We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months. Other outcomes were adverse drug reactions (ADRs), persistence of biologics and factors associated with a response...
March 13, 2018: Annals of the Rheumatic Diseases
Khawar Abbas, Muhammed Mubarak, Mirza Naqi Zafar, Wajiha Musharraf, Mehjabeen Imam, Tahir Aziz, Adibul Hassan Rizvi
OBJECTIVES: Plasma cell-rich acute rejection is an aggressive form of acute rejection that occurs late after transplant and is usually resistant to standard antirejection therapy. This study reports the safety, efficacy, and outcomes of plasma cell-rich acute rejection after treatment with bortezomib, a proteasome inhibitor, in 10 patients after a first living-related renal transplant. MATERIALS AND METHODS: Plasma cell-rich acute rejection was diagnosed using the 2007 Banff classification...
March 9, 2018: Experimental and Clinical Transplantation
Ahmed I Akl, Hussein A Sheashaa, Mona Abdel Rahim, Muhammed A El Hadedy, Ayman F Refaie
Transplant is the optimal therapy for patients with end-stage renal disease. Acute cellular rejection refractory to treatment remains a major risk factor for graft loss and poor outcomes. In this study, we describe a 39-year-old man who received a living-related kidney transplant. Two days after transplant, the patient displayed acute deterioration of graft function. Conventional anti-rejection therapy was initiated, but graft function did not improve. Biopsy revealed acute cellular rejection (grade IIA), and C4d and HLA antibodies were negative...
March 9, 2018: Experimental and Clinical Transplantation
Roberta Gualtierotti, Maria Orietta Borghi, Maria Gerosa, Tommaso Schioppo, Paola Larghi, Jens Geginat, Pier Luigi Meroni
OBJECTIVES: B cells play an important role in the initiation and progression of systemic lupus erythematosus (SLE). Accordingly, B cell-targeted therapy has been suggested as a new rational approach for treating lupus. Belimumab, a human monoclonal antibody directed against B lymphocyte stimulator (BLyS), was reported as the first biological treatment effective in reducing mild-to-moderate SLE disease activity by using different scoring systems and endpoints. Conversely clinical trials with rituximab, a chimeric monoclonal antibody directed against the CD20 expressed by B cells, have failed to achieve primary endpoints in spite of a number of reports showing its beneficial effects...
February 27, 2018: Clinical and Experimental Rheumatology
Xiaojun Huang, Lugui Qiu, Jie Jin, Daobin Zhou, Xiequn Chen, Ming Hou, Jianda Hu, Yu Hu, Xiaoyan Ke, Junmin Li, Yingmin Liang, Ting Liu, Yue Lv, Hanyun Ren, Aining Sun, Jianmin Wang, Chunting Zhao, Mariya Salman, Steven Sun, Angela Howes, Jingzhao Wang, Peng Wu, Jianyong Li
In the Asia-Pacific region, treatment options are limited for patients with relapsed/refractory chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). Rituximab is widely used in this setting when purine analog-based therapies are not appropriate. We evaluated the efficacy and safety of ibrutinib compared with rituximab in a randomized, open-label phase 3 study in predominantly Asian patients with relapsed/refractory CLL/SLL. Patients (N = 160) were randomly assigned 2:1 to receive 420 mg ibrutinib (n = 106) until disease progression (PD) or unacceptable toxicity or up to six cycles of rituximab (n = 54)...
March 13, 2018: Cancer Medicine
Alexandra Smith, Eve Roman, Simon Appleton, Debra Howell, Rod Johnson, Cathy Burton, Russell Patmore
The treatment landscape for mantle cell lymphoma (MCL) has changed dramatically in recent years, with findings from clinical trials reporting improvements in survival. Data on the general patient population are, however, sparse; and it is unclear whether the effects observed in clinical trials have translated into the real-world setting. To investigate this, we examined first-line and relapsed/refractory (RR) disease management in 335 MCL patients diagnosed between 2004 and 2015 in an established population-based patient cohort, along with data on demographic, diagnostic and prognostic factors...
March 13, 2018: British Journal of Haematology
Li Liu, Xuyan Zhang, Sizhou Feng
Epstein-Barr virus related post-transplant lymphoproliferative disorders (EBV-PTLDs) are rare but potentially fatal complications of allogeneic hematopoietic stem cell transplantation (allo-HSCT), characterized by uncontrolled proliferation of EBV-infected lymphocytes. The most frequent risk factors include T cell depletion of graft, HLA mismatch, severe graft versus host disease (GVHD), EBV sero-mismatch (recipient-/donor+) and so on. EBV-PTLDs commonly manifest as fever and lymphadenopathy and may rapidly progress to multi-organ failure and even death...
March 9, 2018: Biology of Blood and Marrow Transplantation
Eli M Miloslavsky, John L Niles, Zachary S Wallace, Frank B Cortazar, Ana Fernandes, Karen Laliberte, John H Stone
OBJECTIVE: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV. METHODS: Patients with active AAV received an 8-week prednisone taper and RTX 375mg/m2 weekly for 4 weeks...
February 3, 2018: Seminars in Arthritis and Rheumatism
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