keyword
https://read.qxmd.com/read/36845094/case-report-vesiculobullous-eruption-with-an-atypical-iga-deposition-pattern-in-a-patient-with-multiple-myeloma-a-case-report-and-literature-review
#21
Tong Li, Hongjie Liu, Wei Li
Cutaneous vesiculobullous eruptions associated with multiple myeloma (MM) are rare. Although the development of blisters is mostly driven by amyloid deposits of paraproteins in the skin, autoimmunity may play a role. In this study, we report an unusual case of an MM patient with blisters who presented with both flaccid and tense vesicles and bullae. Direct immunofluorescence revealed IgA autoantibody deposits in the basement membrane zone (BMZ) and intercellular space of the epidermis, which displayed an atypical autoantibody deposition pattern...
2023: Frontiers in Immunology
https://read.qxmd.com/read/36799924/biclonal-gammopathies-in-south-tunisia-clinical-and-biological-characteristics
#22
JOURNAL ARTICLE
Ameni Jerbi, Hend Hachicha, Aida Charfi, Faten Kallel, Sawsan Feki, Mourad Ben Ayed, Faten Ayadi, Rim Akrout, Faten Frikha, Ali Amouri, Khaoula Kammoun, Moez Mdhaffar, Mohamed Ben Hmida, Nabil Tahri, Zouheir Bahloul, Sofien Baklouti, Moez Elloumi, Hatem Masmoudi
OBJECTIVE: Biclonal gammopathies (BGs) are rare situations characterized by the production of 2 monoclonal proteins. There are no available data on BGs in North Africa. We aimed to estimate the prevalence of BGs in our population and describe their clinical and laboratory features. METHODS: We conducted a 31-year retrospective study including patients with persistent double monoclonal bands based on the results of immunofixation/immunoelectrophoresis. RESULTS: A total of 35 patients with available clinical data (sex ratio, M/F = 1...
February 17, 2023: Laboratory Medicine
https://read.qxmd.com/read/36746240/fdg-pet-ct-and-new-radiopharmaceuticals-in-patients-with-multiple-myeloma
#23
JOURNAL ARTICLE
María Isabel Morales Lozano, María José García-Velloso
Multiple myeloma is a monoclonal gammopathy, a clonal proliferative disorder of plasma cells that produces a protein called M or myeloma protein in the bone marrow, usually IgG or IgA. It accounts for 1% in the general cancer statistics and represents 10% of all hematologic tumours, with a cumulative incidence in Spain of about 5/100,000/year. The incidence increases with age, so that 50% of cases are diagnosed in patients over 75 years of age, being infrequent in the population under 40 years of age. This publication details the indications of FDG PET/CT for the staging and response assessment in patients with MM, accepted by the international working group on myeloma, as well as new molecular imaging radiopharmaceuticals with potential value for personalised medicine...
2023: Revista española de medicina nuclear e imagen molecular
https://read.qxmd.com/read/36735715/rare-pattern-of-myelodysplastic-syndrome-mds-with-serum-monoclonal-immunoglobulin-case-report
#24
JOURNAL ARTICLE
Yating Lin, Jun He, Fan Li, Xuezhong Gu
BACKGROUND: Myelodysplastic syndromes (MDS) are clonal hematopoietic stem cell disorders characterized by bone marrow dysplasia, ineffective hematopoiesis, and cytopenias. Monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM) patients have a high risk of secondary MDS or acute myeloid leukemia (AML) compared to healthy persons, and chemotherapy or transplantation may result in secondary treatment-related MDS. METHODS: A patient was diagnosed with both MDS and MGUS, which was treated using thalidomide, dexamethasone, and danazol...
February 3, 2023: Alternative Therapies in Health and Medicine
https://read.qxmd.com/read/36451151/fibronectin-glomerulopathy-with-monoclonal-gammopathy-responding-to-bortezomib-plus-dexamethasone-a-case-report
#25
JOURNAL ARTICLE
Xiaoli Li, Xueting Qi, Zhigang Ma, Wenhui Huang
BACKGROUND: Fibronectin glomerulopathy is a rare, familial glomerular disease characterized by mesangial fibronectin deposition in the glomeruli. It is caused by the genetic defect in fibronectin and does not involve the activation of the immune system. Therefore, glomerular immunoglobulin and complement staining is generally absent or weak. Monoclonal gammopathy (MG) is an increasing cause of renal lesion, featured by light chain (κ or λ) and/or heavy chain restriction in glomeruli...
November 30, 2022: BMC Nephrology
https://read.qxmd.com/read/36403110/systemic-light-chain-amyloidosis-myopathy-responsive-to-daratumumab-monotherapy
#26
JOURNAL ARTICLE
Diana Maria Chitimus, Edouard Berling, Laurent Garderet, Nadia Venturelli, Edoardo Malfatti, François Jérôme Authier, Guillaume Nicolas, Pascal Laforêt, Claire Lefeuvre
BACKGROUND AND PURPOSE: Amyloid myopathy is a rare and severe manifestation of systemic light chain (AL) amyloidosis. Early diagnosis and staging are mandatory for optimal therapy, given the rapid progression of muscle weakness. Despite the efficacy of bortezomib-based treatment regimens, there is a lack of therapeutic alternatives in non-responsive patients. METHOD: The case report of a patient with systemic AL amyloidosis myopathy treated with daratumumab is presented...
November 20, 2022: European Journal of Neurology
https://read.qxmd.com/read/36367665/tubulointerstitial-nephritis-with-iga-kappa-positive-plasma-cells-in-a-patient-with-primary-sj%C3%A3-gren-s-syndrome-and-monoclonal-gammopathy
#27
JOURNAL ARTICLE
Kazuhiro Sonomura, Yuuki Oobayashi, Misa Iimori, Hiroko Yamauchi, Mayuka Nakayama
This is a case report of a 69-year-old Japanese man who has been undergoing treatment for primary Sjögren's syndrome (pSS) since he was 62 years. A renal biopsy, which revealed diffuse and severe mononuclear cell infiltration in the tubulointerstitium, was performed because of progressive renal dysfunction. Immunostaining demonstrated most of the infiltrating cells to be IgA, kappa, CD38, and CD138 positive. Immunofixation blood test revealed IgA kappa-type M protein; however, bone marrow abnormalities or lymph node enlargements on examination or imaging, respectively, were not observed...
November 11, 2022: CEN Case Reports
https://read.qxmd.com/read/36362407/pathology-of-initial-axon-segments-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-and-related-disorders
#28
REVIEW
Edyta Dziadkowiak, Marta Nowakowska-Kotas, Sławomir Budrewicz, Magdalena Koszewicz
The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily...
November 7, 2022: International Journal of Molecular Sciences
https://read.qxmd.com/read/36315502/prevalence-of-corneal-findings-and-their-interrelation-with-hematological-findings-in-monoclonal-gammopathy
#29
JOURNAL ARTICLE
Mohammad Al Hariri, Markus Munder, Walter Lisch, Alexander K Schuster, Eva-Marie Fehr, Björn Jacobi, Alexander Desuki, Andreas Kreft, Adrian Gericke, Norbert Pfeiffer, Joanna Wasielica-Poslednik
PURPOSE: To determine prevalence of paraproteinemic keratopathy (PPK) among patients with monoclonal gammopathy (MG). To evaluate interrelation between corneal and hematological parameters in patients with PPK. METHODS: Fifty-one patients with monoclonal gammopathy of undetermined significance (n = 19), smoldering multiple myeloma (n = 5) or multiple myeloma (n = 27) were prospectively included in this study. Best-corrected visual acuity, slit-lamp biomicroscopy, Scheimpflug tomography, in-vivo confocal laser scanning microscopy, optical coherence tomography and complete hematological workup were assessed...
2022: PloS One
https://read.qxmd.com/read/36189210/site-specific-n-glycosylation-characterization-of-micro-monoclonal-immunoglobulins-based-on-ethcd-scehcd-ms-ms
#30
JOURNAL ARTICLE
Mengqi Luo, Yonghong Mao, Wenjuan Zeng, Shanshan Zheng, Huixian Li, Juanjuan Hu, Xinfang Xie, Yong Zhang
Monoclonal immunoglobulin produced by clonal plasma cells is the main cause in multiple myeloma and monoclonal gammopathy of renal significance. Because of the complicated purification method and the low stoichiometry of purified protein and glycans, site-specific N-glycosylation characterization for monoclonal immunoglobulin is still challenging. To profile the site-specific N-glycosylation of monoclonal immunoglobulins is of great interest. Therefore, in this study, we presented an integrated workflow for micro monoclonal IgA and IgG purification from patients with multiple myeloma in the HYDRASYS system, in-agarose-gel digestion, LC-MS/MS analysis without intact N-glycopeptide enrichment, and compared the identification performance of different mass spectrometry dissociation methods (EThcD-sceHCD, sceHCD, EThcD and sceHCD-pd-ETD)...
2022: Frontiers in Immunology
https://read.qxmd.com/read/36159343/a-rare-case-of-laryngeal-non-igm-lymphoplasmacytic-lymphoma
#31
Michelle K Hong, Albert Y Han, Travis L Shiba
Laryngeal lymphoplasmacytic lymphoma has been previously reported only a handful of times in the literature. It can be difficult to diagnose without significant histologic workup and proper methodology. Here, we demonstrate the first known case of laryngeal lymphoplasmacytic lymphoma with non-immunoglobulin M (IgM) features. In this case report, a 79-year-old female with seropositive rheumatoid arthritis presented with five months of dysphonia and dyspnea on exertion. Lab studies revealed high levels of serum IgA and IgG...
September 2022: Curēus
https://read.qxmd.com/read/35966255/a-novel-approach-for-more-precise-quantification-of-m-protein-using-variables-derived-from-immunosubtraction-electropherogram-and-associated-biochemistry-analytes
#32
JOURNAL ARTICLE
Dragana Šegulja, Danica Matišić, Karmela Barišić, Dunja Rogić
Introduction: Due to limitations in currently used methodologies, the widely acknowledged approach for quantifying M-protein (MP) is not available. If employed as a source of quantitative data, the immunosubtraction electropherogram (IS-EPG), a qualitative analysis of MP, has the potential to overcome known analytical issues. The aim of this study is to explore measured and derived variables obtained from immunosubtraction electropherogram as a tool for quantifying MP and to compare the derived results to currently available methods...
October 1, 2022: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://read.qxmd.com/read/35883213/detection-and-characterization-of-paraproteinemia-in-canine-chronic-b-cell-lymphocytic-leukemia-using-routine-and-free-light-chain-immunofixation
#33
JOURNAL ARTICLE
R Adam Harris, Erik B Gary, Emily D Rout, Anne C Avery, A Russell Moore
BACKGROUND: Hyperglobulinemia is reported in 26% of canine chronic B-cell lymphocytic leukemia (B-CLL) cases. However, few cases have been characterized by protein electrophoresis and immunofixation (IF), and the incidence of a monoclonal protein (M-protein) is unknown using these techniques. OBJECTIVE: To characterize and determine the proportion of canine B-CLL cases with an M-protein using plasma protein electrophoresis (PPE), routine and free light chain (fLC) IF, and to assess if productive B-CLL cases express MUM1/IRF4 by cell tube block (CTB)...
December 2022: Veterinary Clinical Pathology
https://read.qxmd.com/read/35879978/heavy-chain-light-chain-antibody-immunofluorescence-to-identify-monoclonal-plasma-cells-in-a-case-of-plasma-cell-rich-acute-interstitial-nephritis
#34
Niloufarsadat Yarandi, Mariam P Alexander, Samih H Nasr, Nelson Leung
Heavy/light chain (HLC) antibodies can be used to quantify intact HLC pairs. In immunofluorescence studies, they allow differentiation of monoclonal versus polyclonal immunoglobulin deposits in kidney diseases that occur in the setting of monoclonal gammopathy. Here, we present a case of a patient with acute kidney injury with first kidney biopsy suggestive of acute interstitial nephritis with a polymorphous infiltrate of plasma cells. Routine immunofluorescence did not show a monotypic plasma cell infiltrate...
August 2022: Kidney medicine
https://read.qxmd.com/read/35873606/poems-syndrome-clinical-and-laboratory-approach-towards-diagnosis
#35
Mala Mahto, Tarun Kumar, Mukunda Kumar, Gunjan Kumar, Sushil Kumar, Akash Bansal, Ayan Banerjee
Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem paraneoplastic disorder. Here we describe a case of a 50 year old post-menopausal female who presented with chief complaints of difficulty in walking, getting up from squatting position and tingling sensation of bilateral lower limbs since 1 month. Additional unusual features in the patient included hepatosplenomegaly, endocrinal involvement in the form of hypothyroidism and elevated estradiol levels for her age...
July 2022: Indian Journal of Clinical Biochemistry: IJCB
https://read.qxmd.com/read/35871597/acquired-von-willebrand-syndrome-in-a-patient-with-multiple-comorbidities-including-malt-lymphoma-with-iga-monoclonal-gammopathy-and-hyperviscosity-syndrome
#36
JOURNAL ARTICLE
Mai Owari, Kayo Harada-Shirado, Ryuichi Togawa, Masahiko Fukatsu, Yuki Sato, Koichiro Fukuchi, Mamiko Endo, Hiroshi Takahashi, Satoshi Kimura, Tsukasa Osaki, Masayoshi Souri, Akitada Ichinose, Yoko Shibata, Yuko Hashimoto, Takayuki Ikezoe
Acquired von Willebrand syndrome (aVWS) develops with various underlying diseases. We herein report an individual with aVWS associated with mucosa-associated lymphoid tissue lymphoma in the lungs complicated by hyperviscosity syndrome, Sjögren's syndrome, and hypothyroidism. This patient developed life-threatening hemorrhaging during a lung biopsy despite transfusion of concentrate of plasma-derived VWF/factor VIII. The use of rituximab caused remission of the lymphoma and hyperviscosity syndrome in parallel with the resolution of aVWS...
July 22, 2022: Internal Medicine
https://read.qxmd.com/read/35838241/subcorneal-pustular-dermatosis-type-iga-pemphigus-associated-with-multiple-myeloma-a-case-report-and-literature-review
#37
JOURNAL ARTICLE
Hiroshi Koga, Masahiro Tsutsumi, Kwesi Teye, Norito Ishii, Maki Yamaguchi, Koji Nagafuji, Takekuni Nakama
Immunoglobulin A (IgA) pemphigus, also known as intercellular IgA dermatosis, is a rare autoimmune bullous disease presenting with IgA anti-keratinocyte cell surface autoantibodies. Concomitant lymphoproliferative disorders have been reported in IgA pemphigus, including IgA monoclonal gammopathy of undetermined significance and IgA type multiple myeloma (MM). A 35-year-old Japanese woman with a 3-year history of pruritic papulovesicles on her lower legs and trunk was referred to our department. Histopathological examination revealed acantholytic blisters, and results of both direct and indirect immunofluorescence were negative...
July 15, 2022: Journal of Dermatology
https://read.qxmd.com/read/35747029/biclonal-gammopathy-as-a-misleading-indicator-to-diagnose-poems-syndrome-an-autopsy-case-report-and-a-review-of-the-literature
#38
Shizuko Ainai, Ryouhei Komaki, Naokazu Muramae, Rena Uno, Kenta Mori, Kazunori Otsui, Kimikazu Yakushijin, Kazuhiko Sakaguchi
A 76-year-old man presented with a four-month history of progressive bilateral lower limb muscle weakness and dysesthesia. The patient had extravascular volume overload, and laboratory findings confirmed hypothyroidism, renal dysfunction, and chronic inflammation. Serum protein and immunofixation electrophoresis revealed biclonality of immunoglobulin A (IgA)-kappa and IgA-lambda, which was attributed to chronic inflammation. Subsequently, we detected the proliferation of monoclonal plasma cells in the bone marrow, which led to a diagnosis of POEMS syndrome...
May 2022: Curēus
https://read.qxmd.com/read/35713822/iga-vasculitis-with-underlying-monoclonal-iga-gammopathy-innovative-therapeutic-approach-targeting-plasma-cells-a-case-series
#39
JOURNAL ARTICLE
Antoine Hankard, Saskia Ingen-Housz-Oro, Khalil El Karoui, Romain Paule, Bertrand Lioger, Benoit Brihaye, Maxime Battistella, Stéphanie Jobard, Julie Magnant, Elisabeth Diot, Adrien Bigot, Nicole Ferreira-Maldent, Sophie Deriaz, Ann-Rose Cook, Hélène Henique, Francois Maillot, Achille Aouba, Alexandra Audemard-Verger
OBJECTIVE: There is currently no evidence of the possible benefit of plasma cell-targeting therapies (PCTT) in immunoglobulin A (IgA) monoclonal gammopathy (MG) associated with IgA vasculitis (IgAV). We report the outcome of different PCTT regimens in a cohort of MG-IgAV. METHODS: We used a French network to retrospectively describe the outcome of MG-IgAV patients treated with PCTT. RESULTS: Five patients were included (mean age 65 years)...
June 17, 2022: Clinical Rheumatology
https://read.qxmd.com/read/35660484/assessment-of-m-protein-quantification-using-capillary-electrophoresis-and-immunosubtraction-based-integration-in-clinical-samples-with-low-m-protein-concentrations
#40
JOURNAL ARTICLE
Hanwool Cho, Jin Jung, Hyojin Chae, Jeong Joong Lee, Myungshin Kim, Eun-Jee Oh, Yonggoo Kim, Chang-Ki Min
OBJECTIVES: Quantification of monoclonal protein (M-protein) by serum protein electrophoresis (SPE) is indispensable for diagnosing and monitoring monoclonal gammopathies. However, quantification of small and beta migrating M-proteins is challenging because of overlapping non-immunoglobulin and/or polyclonal immunoglobulin protein fractions. We compared a new integration method based on immunosubtraction (IS-CE) using capillary zone electrophoresis (CZE) against the routine method, which includes a combination of perpendicular drop (0...
September 2022: Clinical Biochemistry
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