Review
Immunology
Artem Oganesyan, Andrew Gregory, Florent Malard, Nerses Ghahramanyan, Mohamad Mohty, Dickran Kazandjian, Arsene Mekinian, Yervand Hakobyan
Summary: MGCS is a new clinical entity associated with MGUS, involving a variety of pathological conditions. Aside from the kidney, the peripheral nervous system, skin, and eye are the main organ systems affected by monoclonal gammopathy. The optimal management of these conditions is currently unknown.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Urology & Nephrology
Lihong Bu, Vincent Javaugue, Sophie Chauvet, Jerold Napier, Surendra Dasari, Jason D. Theis, Julie A. Vrana, Ellen D. McPhail, Samih H. Nasr
Summary: This case report presents a patient with high-risk K light chain multiple myeloma who developed immunotactoid glomerulopathy and myeloma cast nephropathy. The glomerular microtubular deposits stained only for K light chain and C3. Proteomic analysis confirmed the presence of K light chain constant domain and a single VL variability subgroup in both glomeruli and casts. Despite clone-directed chemotherapy, the patient remained dependent on dialysis treatment. The pathogenesis of this light chain-only variant of immunotactoid glomerulopathy may involve activation of the alternative pathway of complement by a nephrotoxic K light chain.
AMERICAN JOURNAL OF KIDNEY DISEASES
(2023)
Article
Medicine, General & Internal
Sheng Nie, Mengyi Wang, Qijun Wan, Yaozhong Kong, Jun Ou, Nan Jia, Xiaodong Zhang, Fan Luo, Xiaoting Liu, Lin Wang, Yue Cao, Ruixuan Chen, Mingpeng Zhao, David Yiu Leung Chan, Guobao Wang
Summary: Performing kidney biopsies in patients with MG and kidney injury can increase the likelihood of correct diagnosis, yet a significant proportion of these patients have lesions unrelated to MG.
FRONTIERS IN MEDICINE
(2021)
Review
Medicine, General & Internal
Hua Huang, Shen-Xian Qian
Summary: Lichen myxedematosus (LM) combined with monoclonal gammopathy of undetermined significance (MGUS) can be diagnosed as scleromyxedema. However, we report a case of generalized papules combined with MGUS that was eventually diagnosed as atypical or intermediate forms of LM because it only involved the skin and the pathological type was not consistent with scleromyxedema. There have been few reports on atypical or intermediate forms of LM, making the course of the disease unpredictable.
FRONTIERS IN MEDICINE
(2023)
Article
Oncology
Dickran Kazandjian, Elizabeth Hill, Alexander Dew, Candis Morrison, Joseph Roswarski, Neha Korde, Michael Emanuel, Ani Petrosyan, Manisha Bhutani, Katherine R. Calvo, Alina Dulau-Florea, Mary Kwok, Min-Jung Lee, Sunmin Lee, Liza Lindenberg, Sham Mailankody, Elisabet Manasanch, Irina Maric, Esther Mena, Nisha Patel, Nishant Tageja, Jane B. Trepel, Baris Turkbey, Hao-Wei Wang, Weixin Wang, Constance Yuan, Yong Zhang, Raul Braylan, Peter Choyke, Maryalice Stetler-Stevenson, Seth M. Steinberg, William D. Figg, Mark Roschewski, Ola Landgren
Summary: Treatment with novel triplet regimens such as KRd and lenalidomide maintenance therapy for high-risk smoldering myeloma may significantly delay the progression to symptomatic multiple myeloma, altering the natural history of the disease. Further randomized clinical trials are needed to confirm the favorable benefit-to-risk profile observed in this phase 2 trial.
Review
Oncology
Louis-Pierre Girard, Cinnie Yentia Soekojo, Melissa Ooi, Wee Joo Chng, Sanjay de Mel
Summary: Immunoglobulin M monoclonal gammopathy of undetermined significance (MGUS) is a distinct form of MGUS with unique clinical manifestations and progression events. It is commonly associated with Waldenstrom macroglobulinaemia and light chain amyloidosis. Additionally, it is often related to autoimmune phenomena and leads to specific disorders. The diagnostic and treatment approaches for these disorders differ from other types of MGUS.
FRONTIERS IN ONCOLOGY
(2022)
Article
Hematology
Carolina Schinke, Alexandra M. Poos, Michael Bauer, Lukas John, Sarah Johnson, Shayu Deshpande, Luis Carrillo, Daisy Alapat, Leo Rasche, Sharmilan Thanendrarajan, Maurizio Zangari, Samer Al Hadidi, Frits van Rhee, Faith Davies, Marc S. Raab, Gareth Morgan, Niels Weinhold
Summary: This study used single-cell RNA sequencing to analyze bone marrow cells at different stages of multiple myeloma (MM) and found changes in immune cell populations during disease progression. These changes included a decrease in memory and naive CD4 T cells, an increase in CD8+ effector T cells and T-regulatory cells, as well as an enrichment of nonclonal memory B cells and CD14, CD16 monocytes. These findings provide important information for understanding the immune changes in MM and for patient stratification and early therapeutic intervention.
Article
Medicine, General & Internal
Marta Morawska, Jadwiga Dwilewicz-Trojaczek, Tomasz Stompor, Piotr Ligocki, Marek Stopinski, Michal Sutkowski, Norbert Grzasko, Anna Kordecka, Mariusz Kordecki, Artur Jurczyszyn, Dominik Dytfeld, Tomasz Wrobel, Krzysztof Jamroziak, Agnieszka Druzd-Sitek, Adam Walter-Croneck, Krzysztof Giannopoulos
Summary: This study developed a 3-step screening protocol for monoclonal gammopathies and multiple myeloma, which proved to be efficient in diagnosis. The protocol can help standardize the understanding of clinical manifestation and diagnostic methods among primary care physicians.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Transplantation
Frank Bridoux, Vincent Javaugue, Samih H. Nasr, Nelson Leung
Summary: Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is a rare renal disease that predominantly affects elderly individuals but can also impact younger patients. Diagnosis relies on immunopathological examination showing granular deposits within the glomeruli. Patients typically experience renal dysfunction and have a poor prognosis, though clone-targeted chemotherapy shows promise in improving renal outcomes.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2021)
Review
Hematology
Jahanzaib Khwaja, Simon J. Salter, Shirley D'Sa
Summary: Cryoglobulinemia is a unique condition characterized by the precipitation of immunoglobulins at low temperatures. Accurate laboratory testing is crucial for diagnosis and monitoring of this disease. Clinical presentations can vary and may involve multiple organ systems. Prompt management is required for severe cases. Further research is needed to determine optimal treatment strategies.
Article
Urology & Nephrology
Cihan Heybeli, Andrew Bentall, Jiqiu Wen, Mariam Priya Alexander, Francis K. Buadi, Fernando G. Cosio, Patrick G. Dean, Angela Dispenzieri, David Dingli, Mireille El Ters, Morie A. Gertz, Amer Hatem, Prashant Kapoor, Hasan Khamash, Taxiarchis Kourelis, Shaji Kumar, Elizabeth C. Lorenz, Martin Mai, Eli Muchtar, David L. Murray, Mikel Prieto, Carrie A. Schinstock, Mark D. Stegall, Rahma Warsame, Nelson Leung
Summary: In patients with immunoglobulin light-chain amyloidosis undergoing kidney transplantation, achieving a very good partial response or complete response before or after the surgery seems to lead to acceptable outcomes.
KIDNEY INTERNATIONAL
(2021)
Article
Medical Laboratory Technology
Erica M. Fatica, Mark Martinez, Paula M. Ladwig, Josiah D. Murray, Mindy C. Kohlhagen, Robert A. Kyle, Taxiarchis Kourelis, John A. Lust, Melissa R. Snyder, Angela Dispenzieri, David L. Murray, Maria A. Willrich
Summary: This study successfully differentiated whether two bands of the same isotype represented monomers and dimers of a single M-protein, an M-protein plus a therapeutic monoclonal antibody, an M-protein with light chain glycosylation, or two distinct biclonal M-proteins using Mass-Fix technology. The results demonstrate the utility of mass spectrometry in accurately diagnosing M-proteins in patients with PCDs.
CLINICAL BIOCHEMISTRY
(2021)
Article
Medicine, General & Internal
Kitti Kormanyos, Klaudia Kovacs, Orsolya Nemeth, Gabor Toth, Gabor Laszlo Sandor, Anita Csorba, Cecilia Nora Czako, Laszlo Modis, Achim Langenbucher, Zoltan Zsolt Nagy, Gergely Varga, Laszlo Gopcsa, Gabor Mikala, Nora Szentmary
Summary: The study analyzed corneal stromal properties in subjects with monoclonal gammopathy using Pentacam and in vivo confocal cornea microscopy. The results showed that corneal light scattering and keratocyte hyperreflectivity were significantly higher in subjects with monoclonal gammopathy compared to the control group.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Medicine, General & Internal
Rupali S. Avasare, Nicole K. Andeen, Andrea Havasi, Jonathan J. Hogan
Summary: Dysproteinemic kidney diseases are kidney disorders caused by lymphoproliferative disorders, resulting in kidney damage from nephrotoxic monoclonal immunoglobulins. There have been advancements in understanding and treating these diseases in the past decade, although challenges remain due to their rarity and the rapidly evolving field of hematology.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Hematology
Simon Stern, Satarupa Chaudhuri, Mark Drayson, Sarah Henshaw, Kamaraj Karunanithi, Fenella Willis
Summary: This Good Practice Paper provides recommendations for the diagnosis, risk stratification and management of MGUS. It also discusses the entity of MGCS and how it should be managed. Additionally, the potential for targeted population screening for MGUS is explored.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Review
Allergy
Laure Frumholtz, Sara Laurent-Roussel, Dan Lipsker, Benjamin Terrier
Summary: Cutaneous vasculitis is an inflammatory disease affecting the dermal blood vessel walls, which can reflect different forms of systemic vasculitis with diverse clinical manifestations depending on the location and size of the vessels involved.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2021)
Article
Dermatology
Renaud Felten, Dan Lipsker, Jean Sibilia, Francois Chasset, Laurent Arnaud
Summary: The term lupus has been used since the Middle Ages to refer to several diseases characterized by ulcerous lesions. In the 19th century, a distinction between different types of lupus emerged, and the systemic nature of the disease was recognized. Modern treatments such as glucocorticoids, hydroxychloroquine, and immunosuppressive agents emerged in the second half of the 20th century. In the 21st century, there has been a deeper understanding of the disease's pathogenesis and the development of biologic and targeted treatments.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Letter
Dermatology
Antoine Braud, Antoine Mahe, Catherine Michel, Bernard Cribier, Dan Lipsker, Cedric Lenormand
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2021)
Letter
Dermatology
V. Reymann, D. Bessis, B. Bergeret, D. Lipsker, A. Du-Thanh, N. Terrail, M. Dandurand, O. Dereure
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2021)
Letter
Dermatology
B. Bergeret, L. -P. Secco, V. Pallure, C. Daien, Y. -M. Pers, J. Gottlieb, S. Barete, C. Girard, D. Lipsker, D. Bessis
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2021)
Article
Dermatology
Cedric Lenormand, Gaelle Marzolf, Dan Lipsker
Summary: Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome is a condition characterized by a slowly expanding red or brown patch overlying a solitary plasmacytoma of the bone. Early recognition is crucial as it may progress to the more serious POEMS syndrome. The syndrome has two forms, classic and morphea-like variants, with treatment depending on the final hematologic diagnosis and requiring radiation of the plasmacytoma.
CLINICS IN DERMATOLOGY
(2021)
Letter
Dermatology
Gaelle Marzolf, Cedric Lenormand, Catherine Michel, Bernard Cribier, Dan Lipsker
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2021)
Review
Dermatology
C. Lenormand, D. Lipsker
Summary: This article reviews the various skin manifestations in patients with lupus erythematosus, discussing their diagnostic, pathogenic and prognostic relevance, as well as their impact on therapeutic choices. Specific skin lesions of LE, such as tumid lupus and lupus profundus, are characterized by autoimmune pathomechanisms and allow for simple diagnosis through clinicopathological correlation. Recognition of certain dermatological signs, like reticulated erythema and splinter hemorrhages, is important for identifying lupus patients at increased cardiovascular risk and with a worse overall prognosis. Neutrophilic cutaneous lupus erythematosus, including entities like neutrophilic urticarial dermatosis, suggests a role of autoinflammatory mechanisms in certain lupus manifestations. Treatment options, such as antimalarials and dapsone, are discussed for different skin manifestations.
ANNALES DE DERMATOLOGIE ET DE VENEREOLOGIE
(2021)
Letter
Dermatology
Didier Bessis, Jean-David Bouaziz, Francois Chasset
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Pierre-Alexis Autrusseau, Roberto Luigi Cazzato, Guillaume Koch, Nitin Ramamurthy, Pierre Auloge, Pierre De Marini, Dan Lipsker, Afshin Gangi, Julien Garnon
Summary: This retrospective study assessed the technical feasibility, safety, and oncologic outcomes of PCA for locoregional and distant lymph node metastases. The results showed that PCA is feasible, safe, and offers promising local tumor control at midterm follow-up.
JOURNAL OF VASCULAR AND INTERVENTIONAL RADIOLOGY
(2021)
Letter
Dermatology
Dan Lipsker
Article
Hematology
Raphael E. Szalat, Joshua Gustine, J. Mark Sloan, Camille Edwards, Vaishali Sanchorawala
Summary: Daratumumab as a single agent or in combination with chemotherapies shows impressive efficacy in AL amyloidosis patients, but predictive factors associated with outcomes remain unclear. Achieving at least VGPR and presence of 1q21 gain are independently associated with better progression free survival and overall survival. Patients receiving more than 12 cycles of daratumumab therapy have significantly longer survival outcomes.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Allergy
Miriam Gross, Carsten Speckmann, Annette May, Tania Gajardo-Carrasco, Katharina Wustrau, Sarah Lena Maier, Marcus Panning, Daniela Huzly, Abbas Agaimy, Yenan T. Bryceson, Sharon Choo, C. W. Chow, Gregor Dueckers, Anders Fasth, Sylvie Fraitag, Katja Graewe, Sabine Haxelmans, Dirk Holzinger, Ole Hudowenz, Judith M. Huebschen, Claudia Khurana, Korbinian Kienle, Roman Klifa, Klaus Korn, Heinz Kutzner, Tim Laemmermann, Svea Ledig, Dan Lipsker, Marie Meeths, Nora Naumann-Bartsch, Jelena Rascon, Anne Schaenzer, Maximilian Seidl, Bianca Tesi, Christelle Vauloup-Fellous, Beate Vollmer-Kary, Klaus Warnatz, Claudia Wehr, Benedicte Neven, Pablo Vargas, Fernando E. Sepulveda, Kai Lehmberg, Annette Schmitt-Graeff, Stephan Ehl
Summary: This study investigated the defective effector mechanism allowing rubella vaccine virus persistence in granulomas by studying patients with cytotoxicity defects and granuloma. The results suggest a critical role of T cells in rubella elimination.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Francois Maurier, Maud Michaud, Roxane Reviron, Dan Lipsker
Summary: This is a case of scar-like skin lesions that appeared after reduction mammoplasty. The patient also developed symptoms of neuromyotonia, causing muscle contractions and abnormal movements. The diagnosis of neuromyotonia was based on clinical and electromyographic findings, while the skin lesions were diagnosed as morphea. Treatment with antiepileptic drugs and corticosteroids was effective.
Article
Ophthalmology
Mathieu Wurtz, Elisa Ruhland, XuanLi Liu, Izzie-Jacques Namer, Viola Mazzoleni, Dan Lipsker, Daniel Keller, Gilles Prevost, David Gaucher
Summary: The study confirmed that PVL activates oxidative pathways and alters neurotransmitter retinal concentrations and release, supporting the hypothesis that PVL could induce a neurogenic inflammation in the retina.
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
(2021)
