Letter
Clinical Neurology
Tuba Rashid Khan, Vincent Zimmern, Victor Aquino, Cynthia Wang
Summary: This report describes a case of a child with early and severe manifestations of AQP-4 positive NMOSD, who had refractory disease despite aggressive immunotherapy and ultimately underwent AHSCT.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2021)
Article
Oncology
Malte Roerden, Stefan Wirths, Martin Soekler, Wolfgang A. Bethge, Wichard Vogel, Juliane S. Walz
Summary: High-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (Auto-HSCT) is a standard frontline treatment for fit mantle cell lymphoma (MCL) patients. However, lymphoma cell contamination of stem cell grafts may lead to poor outcomes. Analyzing residual MCL cells in autologous grafts can improve risk assessment for MCL patients undergoing Auto-HSCT.
Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica and its spectrum is a relapsing demyelinating disorder of the central nervous system, encompassing six syndromes and associated with aquaporin-4 autoantibodies. Effective treatment is able to prevent relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica, previously known as Devic's disease, is a relapsing demyelinating disorder of the central nervous system that encompasses six syndromes and is associated with aquaporin-4 autoantibodies. Effective treatment is crucial in preventing relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Clinical Neurology
Shabeer Paul, Gouranga Prasad Mondal, Ramesh Bhattacharyya, Kartik Chandra Ghosh, Imtiyaz Ahmad Bhat
Summary: Over the past two decades, the disease concept of NMOSD has significantly changed with the detection of MOG antibody and the understanding of immune astrocytopathy. The revised diagnostic criteria have widened the clinical spectrum of NMOSD, allowing for earlier diagnosis and prompt initiation of effective immunosuppression for better long-term outcomes. Challenges still remain in treating seronegative NMOSD due to limited treatment options.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2021)
Editorial Material
Critical Care Medicine
Matthew Rendo, Teri J. Franks, Jeffrey R. Galvin, Andrew Berglund, Charles Volk, Matthew Peterson
Summary: Light chain deposition disease is a rare condition where light chains are deposited in organs, often caused by unchecked production from a plasma cell clone. Pulmonary involvement is rare, especially in young, otherwise healthy patients. Treatment involving autologous stem cell transplantation has shown success in isolated cases.
Article
Immunology
Liang Wang, Lei Du, Qinying Li, Fang Li, Bei Wang, Yuanqi Zhao, Qiang Meng, Wenyu Li, Juyuan Pan, Junhui Xia, Shitao Wu, Jie Yang, Heng Li, Jianhua Ma, Jingzi ZhangBao, Wenjuan Huang, Xuechun Chang, Hongmei Tan, Jian Yu, Lei Zhou, Chuanzhen Lu, Min Wang, Qiang Dong, Jiahong Lu, Chongbo Zhao, Chao Quan
Summary: This study investigates the predictors of relapse, and severe visual or motor disability in patients with NMOSD and develops corresponding predictive models. Early identification of patients at risk of unfavorable outcomes is crucial for informing treatment decisions.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Clinical Neurology
Hesham Abboud, Andrea Salazar-Camelo, Naveen George, Sarah M. Planchon, Marcelo Matiello, Maureen A. Mealy, Andrew Goodman
Summary: Neuromyelitis optica spectrum disorders are autoimmune inflammatory conditions that primarily affect the optic nerves, spinal cord, and other areas, leading to recurrent attacks of visual, motor, and sensory dysfunction. While progress has been made in prevention and treatment, the residual disability per attack remains high. Treatment options for NMOSD are limited but can be inferred from therapies used in other diseases.
JOURNAL OF NEUROLOGY
(2022)
Article
Cell Biology
Gianluca Bagnato, Antonio Giovanni Versace, Daniela La Rosa, Alberta De Gaetano, Egidio Imbalzano, Marianna Chiappalone, Carmelo Ioppolo, William Neal Roberts, Alessandra Bitto, Natasha Irrera, Alessandro Allegra, Giovanni Pioggia, Sebastiano Gangemi
Summary: Autologous hematopoietic stem cell transplantation has shown improved outcomes in the treatment of severe systemic sclerosis, but there are still challenges such as accurate patient selection and pre-transplant analysis.
Article
Oncology
Mohammad Rafiee, Fatemeh Amiri, Mohammad Hossein Mohammadi, Abbas Hajifathali
Summary: This study investigated the predictive value of circulatory microRNAs (miRs) expression for outcomes of autologous hematopoietic stem cell transplantation (AHSCT). The results showed that the expression of miR-125b was associated with relapse, high lactate dehydrogenase (LDH), and high erythrocyte sedimentation rate (ESR). This finding provides a potential opportunity for prognosis evaluation and new targeted therapy after AHSCT.
Article
Immunology
Hyo Jae Kim, Jong-Eun Park, Wangyong Shin, Dayoung Seo, Seungmi Kim, Hyunji Kim, Jinsung Noh, Yonghee Lee, Hyunjin Kim, Young-Min Lim, Hyori Kim, Eun-Jae Lee
Summary: NMOSD patients exhibit the most pronounced B cell activation among those with CIDDs, characterized by higher levels of isotype class switching, clonality, SHM rates, and shorter CDR3 lengths. These features are distinct from those observed in patients with other CIDDs like MOGAD.
JOURNAL OF NEUROINFLAMMATION
(2023)
Article
Oncology
Juan Chen, Li Liu, Runzhi Ma, Aiming Pang, Donglin Yang, Xin Chen, Jialin Wei, Yi He, Rongli Zhang, Weihua Zhai, Qiaoling Ma, Erlie Jiang, Mingzhe Han, Jiaxi Zhou, Sizhou Feng
Summary: This study evaluated the efficacy of autologous hematopoietic stem cell transplantation (auto-HSCT) in patients with favorable-risk acute myeloid leukemia in first remission. The results showed that auto-HSCT had excellent outcomes in these patients and could be an effective treatment for them.
CANCER CELL INTERNATIONAL
(2022)
Article
Immunology
Simone A. Minnie, Geoffrey R. Hill
Summary: The incidence of multiple myeloma is increasing globally, with substantial morbidity and mortality and remains largely incurable. Autologous stem cell transplantation (ASCT) has been shown to provide a progression free survival benefit in eligible patients beyond novel therapies. ASCT results in immune effects beyond cytoreduction, highlighting the potential for new immunotherapy maintenance strategies to prevent myeloma progression.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Koon-Ho Chan, Chi-Yan Lee
Summary: NMOSD is an autoimmune disorder that predominantly affects females, leading to serious CNS inflammatory disorders. The pathogenesis involves AQP4-IgG autoantibodies targeting aquaporin-4, triggering astrocytopathy and neuroinflammation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Clinical Neurology
Viktoria Papp, Melinda Magyari, Orhan Aktas, Thomas Berger, Simon A. Broadley, Philippe Cabre, Anu Jacob, Jun-ichi Kira, Maria Isabel Leite, Romain Marignier, Katsuichi Miyamoto, Jacqueline Palace, Albert Saiz, Maria Sepulveda, Olafur Sveinsson, Zsolt Illes
Summary: The systematic review of worldwide prevalence, incidence, and demographic characteristics of NMOSD highlighted variations among different geographic areas and ethnicities, with prominent female predominance in adults and peak incidence and prevalence in middle-aged adults. African ethnicity had the highest incidence and prevalence of NMOSD, whereas White ethnicity had the lowest, suggesting the need for further epidemiologic studies to investigate genetic effects and etiologic factors.
Article
Biochemistry & Molecular Biology
Angela Genchi, Elena Brambilla, Francesca Sangalli, Marta Radaelli, Marco Bacigaluppi, Roberto Furlan, Annapaola Andolfo, Denise Drago, Cinzia Magagnotti, Giulia Maria Scotti, Raffaella Greco, Paolo Vezzulli, Linda Ottoboni, Marco Bonopane, Daniela Capilupo, Francesca Ruffini, Daniela Belotti, Benedetta Cabiati, Stefania Cesana, Giada Matera, Letizia Leocani, Vittorio Martinelli, Lucia Moiola, Luca Vago, Paola Panina-Bordignon, Andrea Falini, Fabio Ciceri, Anna Uglietti, Maria Pia Sormani, Giancarlo Comi, Mario Alberto Battaglia, Maria A. A. Rocca, Loredana Storelli, Elisabetta Pagani, Giuseppe Gaipa, Gianvito Martino
Summary: This study conducted a phase 1 clinical trial evaluating the feasibility, safety and tolerability of intrathecally transplanted human fetal neural precursor cells (hfNPCs) in patients with progressive multiple sclerosis (PMS). The results demonstrated that hfNPC therapy is feasible, safe and tolerable in PMS patients.
Article
Clinical Neurology
Giacomo Boffa, Alessio Signori, Luca Massacesi, Alice Mariottini, Elvira Sbragia, Salvatore Cottone, Maria Pia Amato, Claudio Gasperini, Lucia Moiola, Stefano Meletti, Anna Maria Repice, Vincenzo Brescia Morra, Giuseppe Salemi, Francesco Patti, Massimo Filippi, Giovanna De Luca, Giacomo Lus, Mauro Zaffaroni, Patrizia Sola, Antonella Conte, Riccardo Nistri, Umberto Aguglia, Franco Granella, Simonetta Galgani, Luisa Maria Caniatti, Alessandra Lugaresi, Silvia Romano, Pietro Iaffaldano, Eleonora Cocco, Riccardo Saccardi, Emanuele Angelucci, Maria Trojano, Giovanni Luigi Mancardi, Maria Pia Sormani, Matilde Inglese
Summary: This study compared the effects of autologous hematopoietic stem cell transplantation (AHSCT) with other anti-inflammatory disease-modifying therapies (DMTs) on long-term disability worsening in active secondary progressive multiple sclerosis (SPMS). The results showed that AHSCT was associated with a slower disability progression and a higher likelihood of disability improvement compared to standard immunotherapy.
Article
Clinical Neurology
Chiara Starvaggi Cucuzza, Elisa Longinetti, Nicolas Ruffin, Bjoern Evertsson, Ingrid Kockum, Maja Jagodic, Faiez Al Nimer, Thomas Frisell, Fredrik Piehl
Summary: This study aimed to determine the clinical and neuroradiologic disease activity, as well as B-cell repopulation dynamics, after implementing extended rituximab dosing in RRMS. The results showed that extended dosing intervals did not increase the risk of relapse in RRMS, and the dynamics of total B-cell and memory B-cell repopulation varied considerably.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2023)
Article
Clinical Neurology
Sonja Kosek, Barbro Persson, Rui Rodrigues, Clas Malmestrom, Anna Rostedt Punga, Joachim Burman
Summary: This study aimed to estimate the 5-year incidence rate of autoimmune encephalitis (AE) and paraneoplastic neurological syndrome (PNS) in Sweden. The results showed that the incidence rate of AE and PNS doubled from 2015 to 2019.
ACTA NEUROLOGICA SCANDINAVICA
(2023)
Review
Allergy
Alessandro Bruno, Carmelo Gurnari, Tobias Alexander, John A. Snowden, Raffaella Greco
Summary: VEXAS is a novel disease with a variety of clinical features, mainly affecting men and presenting symptoms in the fifth to sixth decade of life. It has attracted medical interest due to its association with multiple medical conditions. However, its recognition in everyday clinical practice is challenging and requires close collaboration between different medical specialists. The treatment and diagnostic guidelines for VEXAS are exploratory and include rheumatological and supportive care treatments. Allogeneic hematopoietic stem cell transplantation may be curative, but its position in the treatment algorithm is yet to be defined.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Raffaella Greco, John A. Snowden, Nina Simone Knelange, Gloria Tridello, Carlotta Cacciatore, Alienor Xhaard, Fabio Ciceri, Matthew Collin, Christelle Ferra, Ann De Becker, Manuela Badoglio, Dina Averbuch, Tobias Alexander, Per Ljungman, Rafael De la Camara
Summary: This multicenter observational study reported the clinical data on COVID-19 in 11 patients affected by severe autoimmune diseases treated with HSCT. All patients had symptoms during the initial phase of the SARS-CoV-2 infection. Four cases developed lower respiratory tract disease. Hospitalization was required in 6 cases, but none needed ICU admission or ventilation/supplemental oxygen. Different interventions were adopted and all patients survived and resolved the infection at the last follow-up.
JOURNAL OF AUTOIMMUNITY
(2023)
Article
Clinical Neurology
David Leppert, Mitsuru Watanabe, Sabine Schaedelin, Fredrik Piehl, Roberto Furlan, Matteo Gastaldi, Jeremy Lambert, Bjorn Evertsson, Katharina Fink, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Jun-ichi Kira, Pascal Benkert, Aleksandra Maceski, Eline Willemse, Johanna Oechtering, Annette Orleth, Stephanie Meier, Jens Kuhle
Summary: This study aimed to determine the differences in granulocyte activation markers (GAM) between neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS), and whether they are associated with neurological impairment. The results showed that GAM and adhesion molecules were higher in acute NMOSD compared to RRMS and correlated with clinical disability scores. The composite of GAM can be used as a reliable biomarker to differentiate NMOSD from MS, including aAQP4(-) NMOSD.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Clinical Neurology
Katharina Fink, Agnes Gorczyca, Peter Alping, Simon Englund, Susan Farmand, Annette M. Langer-Gould, Fredrik Piehl, Kyla McKay, Thomas Frisell, Neda Razaz
Summary: This study aimed to investigate the association between multiple sclerosis (MS) in mothers and the risks of adverse pregnancy and perinatal outcomes. A population-based retrospective cohort study in Sweden showed that mothers with MS had a higher risk of obstetric complications and their newborns had an increased risk of preterm birth and being small for gestational age compared to MS-free mothers. However, exposure to disease-modifying therapy (DMT) during pregnancy did not lead to major adverse outcomes.
MULTIPLE SCLEROSIS JOURNAL
(2023)
Article
Immunology
Chiara Oltolini, Andrea Acerbis, Giorgio Orofino, Sara Racca, Maddalena Noviello, Stefania Dispinseri, Nicola Clementi, Simona Piemontese, Elisabetta Xue, Fabio Giglio, Maria Teresa Lupo Stanghellini, Elisa Diral, Alessandro Bruno, Elena Tassi, Valeria Beretta, Ilaria Marzinotto, Gabriella Scarlatti, Vito Lampasona, Anna Ardemagni, Michela Sampaolo, Chiara Bonini, Consuelo Corti, Jacopo Peccatori, Antonella Castagna, Fabio Ciceri, Raffaella Greco
Summary: This report describes a case of a 34-year-old patient with active SARS-CoV-2 infection and high-risk leukemia who underwent allogeneic hematopoietic stem cell transplantation. Timely anti-SARS-CoV-2 preventive therapies and prompt management of transplant-related complications resulted in a favorable outcome.
FRONTIERS IN IMMUNOLOGY
(2023)
Editorial Material
Oncology
Michele Malagola, Robert Ohgami, Raffaella Greco
FRONTIERS IN ONCOLOGY
(2023)
Article
Clinical Neurology
Elisa Longinetti, Simon Englund, Joachim Burman, Katharina Fink, Anna Fogdell-Hahn, Martin Gunnarsson, Jan Hillert, Annette Magdalene Langer-Gould, Jan Lycke, Petra Nilsson, Jonatan Salzer, Anders Svenningsson, Johan Mellergard, Tomas Olsson, Fredrik Piehl, Thomas Frisell
Summary: This study analyzed a Swedish nationwide observational study on RRMS to identify trajectories of processing speed and physical disability after DMT start. The results showed that patients' processing speed remained stable over time, while those with moderate physical disability experienced deterioration in physical function. However, there was a strong association between processing speed and disability.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Letter
Medicine, Research & Experimental
Elisabetta Xue, Gianluca Scorpio, Annalisa Ruggeri, Daniela Clerici, Francesca Farina, Edoardo Campodonico, Andrea Acerbis, Paolo Fiore, Alessandro Bruno, G. Carrabba, Jacopo Peccatori, Raffaella Greco, Maria Teresa Lupo Stanghellini, Fabio Ciceri, Consuelo Corti
CURRENT RESEARCH IN TRANSLATIONAL MEDICINE
(2023)
Article
Medicine, Research & Experimental
Edoardo Campodonico, Elisabetta Xue, Simona Piemontese, Anna Chiara, Alessandro Bruno, Gianluca Scorpio, Rosamaria Nitti, Daniele Sannipoli, Giorgio Orofino, Paolo Fiore, Maria Chiara Quattrocchi, Elisa Diral, Daniela Clerici, Francesca Farina, Consuelo Corti, Francesca Lunghi, Maria Teresa Lupo-Stanghellini, Nadia Di Muzio, Fabio Ciceri, Raffaella Greco, Jacopo Peccatori
Summary: A retrospective analysis on 14 MF patients found that splenic irradiation before hematopoietic stem cell transplantation can effectively reduce spleen size and alleviate symptoms. This approach is safe and effective in the selected population and may be considered as a treatment option for MF.
CURRENT RESEARCH IN TRANSLATIONAL MEDICINE
(2023)
Article
Clinical Neurology
Faisal Hayat Nazir, Anna Wiberg, Malin Mueller, Sara Mangsbo, Joachim Burman
Summary: Multiple sclerosis is a complex and heterogeneous disease that often starts as a clinically isolated syndrome. Autoantibodies play an important role in its pathogenesis, but their target has been difficult to identify. Cell-based methods have been developed as an alternative strategy for detecting autoantibodies. This study explored differences in antibody binding to oligodendroglial and neuronal cell-lines in serum and CSF samples from multiple sclerosis patients and controls, and found that the binding of immunoglobulin G from CSF to the human oligodendroglioma cell-line was the best discriminator between patients and controls, with a high sensitivity and specificity. The cell-based ELISA showed a high degree of accuracy in discriminating between multiple sclerosis patients and controls, with the disease course being the major determinant for antibody binding.
BRAIN COMMUNICATIONS
(2023)
Article
Clinical Neurology
Bruno Brochet, Alessandra Solari, Jeannette Lechner-Scott, Fredrik Piehl, Dawn Langdon, Raymond Hupperts, Krzysztof Selmaj, Francesco Patti, Luis Brieva, Eva Maria Maida, Nektaria Alexandri, Andrzej Smyk, Axel Nolting, Birgit Keller, Xavier Montalban, Eva Kubala Havrdova
Summary: Cladribine tablet treatment significantly improved the health-related quality of life in people with highly active relapsing multiple sclerosis, with good safety and tolerability.
MULTIPLE SCLEROSIS JOURNAL
(2023)