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https://read.qxmd.com/read/37005096/-rare-manifestations-of-monoclonal-gammopathies-about-two-clinical-cases-and-literature-review
#1
JOURNAL ARTICLE
Q Perlot, C Hermans, M-C Vekemans
INTRODUCTION: Monoclonal gammopathies are common over the age of 50. Patients are usually asymptomatic. However, some patients present with secondary clinical manifestations, which are now grouped under the entity « Monoclonal Gammopathy of Clinical Significance » (MGCS). CASE REPORT: Here, we report two rare cases of MGCS: an acquired von Willebrand syndrome (AvWS) and an acquired angioedema (AAE). CONCLUSION: The discovery of a decrease in von Willebrand activity (vWF:RCo) or angioedema in a patient over 50 years of age, in the absence of a family history, should prompt a search for a hemopathy and in particular, a monoclonal gammopathy...
March 31, 2023: La Revue de Médecine Interne
https://read.qxmd.com/read/36803980/-latest-updates-on-immunotactoid-glomerulopathy-and-fibrillary-glomerulonephritis
#2
REVIEW
Marie-Camille Lafargue, Camille Cohen
Various hematologic malignancies can lead to renal complications. The most common of these hemopathies to affect the kidney is multiple myeloma, however an increasing number of kidney diseases are associated with other monoclonal gammopathies. It is recognized that clones in small abundance can be responsible for severe organ damage, thus the concept of monoclonal gammopathy of renal significance (MGRS) has emerged. Although the hemopathy in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma, the diagnosis of a renal complication changes the therapeutic management...
February 15, 2023: Bulletin du Cancer
https://read.qxmd.com/read/35937020/early-onset-of-scleromyxedema-arndt-gottron-associated-with-a-monoclonal-gammapathy-successful-treatment-with-intravenous-immunoglobulins
#3
Asma Kefi, Fatima Jaziri, Khaoula Ben Abdelghani, Sami Turki
Arndt-Gottron (S-AG) syndrome or scleromyxedema is a scarce disease characterized by a generalized papular and sclerodermoid eruption and systemic manifestations that can lead to significant morbid-mortality. Interesting, S-AG can be associated with a paraprotein. We report an exceptional scleromyxedema case associated with a benign monoclonal gammapathy in an old woman.
August 2022: Clinical Case Reports
https://read.qxmd.com/read/35870985/-cardiac-amyloidosis-state-of-art-in-2022
#4
JOURNAL ARTICLE
S Oghina, M A Delbarre, E Poullot, K Belhadj, P Fanen, T Damy
The 3 main types of cardiac amyloidosis are linked to two protein precursors: AL amyloidosis secondary to free light chain deposits in the context of monoclonal gammopathy (mainly of undetermined significance or myeloma) and transthyretin amyloidosis (ATTR), comprising wild-type transthyretin amyloidosis (ATTRwt for wild type) and hereditary transthyretin amyloidosis (ATTRv for variant). These diseases are underdiagnosed and highly prevalent in common cardiac phenotypes in recent studies (heart failure with preserved ejection fraction, severe aortic stenosis, hypertrophic cardiomyopathy)...
July 20, 2022: La Revue de Médecine Interne
https://read.qxmd.com/read/35371382/-descriptive-cross-sectional-study-assessing-the-clinical-and-paraclinic-profiles-of-monoclonal-gammopathies-in-an-agricultural-region-of-souss-massa-morocco
#5
JOURNAL ARTICLE
Aissam El Maataoui, Aadil Taoufiq, Salma Fares, Kaouthar Sokori
Introduction: given the lack of information about monoclonal gammopathies, our primary study outcome was to describe the epidemiological, clinical and biochemical profiles of monoclonal gammopathies in the Souss-Massa region, in southern Morocco. Methods: we conducted a retrospective study, by selecting only complete medical records. We used records of patients diagnosed with monoclonal gammopathy at the local oncology center during a period of over 10 years. Results: one hundred and seventeen patients were included in the study, with a high male predominance (65%) and a male/female sex-ratio of 1...
2022: Pan African Medical Journal
https://read.qxmd.com/read/35134549/erythroblastic-synartesis-associated-with-lymphoproliferative-disorder-there-can-be-more-than-meets-the-eye
#6
REVIEW
Camille Mettler, Cassandre Petit, Vincent Ernest, Bouchra Asli, Marie-Thérèse Daniel, Stéphanie Mathis, Jean Marc Zini, Benoit Faucher, Mikael Ebbo, Paul Legendre, Marion Malphettes
Erythroblastic synartesis is a rare cause of acquired dyserythropoiesis. Only 9 cases have been previously reported. We hereby report 3 cases of patients diagnosed with erythroblastic synartesis associated with monoclonal immunoglobulin and an overt malignant lymphoid disorder. A different B-cell clone may produce the monoclonal immunoglobulin, forming a biclonal disorder. In light of these data and literature review, treatment targeting the paraprotein seems to be efficient to control synartesis and correct anemia...
March 2022: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://read.qxmd.com/read/34824836/consider-systemic-capillary-leak-syndrome-in-monoclonal-gammopathy-with-shock
#7
Ounci-Essad, Amine Bouchlarhem, Oussama Lamzouri, Ghizlane El Aidouni, Leila Haddar, Hamza Mimouni, Houssam Bkiyar, Brahim Housni
INTRODUCTION AND IMPORTANCE: The cappilary leacking syndrome is a very rare disease that can be idiopathic (clarkson syndrome) or secondary to other pathologys. CASE PRESENTATION: We report a case of 37-year-old women who was admitted in the emergency room for a hemodynamic shock of neither cardiac nor septic cause, and the patient wasn't presenting any bleeding. The investigations showed that the diagnosis was a Clarkson syndrome crisis and the patient was having supportive treatment containing fluid therapy, vasoactive drugs, and ECMO...
December 2021: Annals of Medicine and Surgery
https://read.qxmd.com/read/34632574/further-characterization-of-clinical-and-laboratory-features-in-vexas-syndrome-large-scale-analysis-of-a-multicentre-case-series-of-116-french-patients
#8
MULTICENTER STUDY
S Georgin-Lavialle, B Terrier, A F Guedon, M Heiblig, T Comont, E Lazaro, V Lacombe, L Terriou, S Ardois, J-D Bouaziz, A Mathian, G Le Guenno, A Aouba, R Outh, A Meyer, M Roux-Sauvat, M Ebbo, L P Zhao, A Bigot, Y Jamilloux, V Guillotin, E Flamarion, P Henneton, G Vial, V Jachiet, J Rossignol, S Vinzio, T Weitten, J Vinit, C Deligny, S Humbert, M Samson, N Magy-Bertrand, T Moulinet, R Bourguiba, T Hanslik, C Bachmeyer, M Sebert, M Kostine, B Bienvenu, P Biscay, E Liozon, L Sailler, F Chasset, A Audemard-Verger, E Duroyon, G Sarrabay, F Borlot, C Dieval, T Cluzeau, P Marianetti, H Lobbes, G Boursier, M Gerfaud-Valentin, J Jeannel, A Servettaz, S Audia, M Larue, B Henriot, B Faucher, J Graveleau, B de Sainte Marie, J Galland, L Bouillet, C Arnaud, L Ades, F Carrat, P Hirsch, P Fenaux, O Fain, P Sujobert, O Kosmider, A Mekinian
BACKGROUND: A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome'). OBJECTIVES: To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome. METHODS: One hundred and sixteen patients with VEXAS syndrome were referred to a French multicentre registry between November 2020 and May 2021...
March 2022: British Journal of Dermatology
https://read.qxmd.com/read/33678446/-poems-syndrome-diagnosis-stratification-treatments
#9
JOURNAL ARTICLE
A Talbot, A Jaccard, B Arnulf
POEMS syndrome is a rare form of B-cell dyscrasia with multiple clinical signs including the acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It is a paraneoplastic syndrome due to an underlying plasma cell disorder belonging to the monoclonal gammopathies of clinical significance (MGCS). The major criteria for this syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), and the presence of Castleman's disease...
May 2021: La Revue de Médecine Interne
https://read.qxmd.com/read/33346488/-monoclonal-gammapathy-of-renal-significance-mgrs-at-the-current-state-terminology-diagnosis-and-treatment
#10
JOURNAL ARTICLE
L V Lysenko Kozlovskaya, V V Rameev, T V Androsova
In this article we discussed the current state of monoclonal gammapathy of renal significance (Monoclonal Gammopathy of Renal Significance MGRS) and revealed problems of B-cell clone secreting nephrotoxic monoclonal immunoglobulin identification. We followed 276 patients with monoclonal gammapathy including patients with non-amyloid nephropathy. The majority of patients had systemic AL-amyloidosis. We established better survival of the treated patients with systemic AL-amyloidosis in comparison with retrospective untreated cohort...
July 9, 2020: Terapevticheskiĭ Arkhiv
https://read.qxmd.com/read/33030148/type-i-cryoglobulinaemia-associated-to-monoclonal-gammapathy-of-undetermined-significance
#11
JOURNAL ARTICLE
Juan Manuel Duarte, Paloma Ocampo, Silvia Graciela Ramos, Orlando Gabriel Carballo, Ricardo E Barcia, Cecilia Elena Arévalo
Cryoglobulins are immunoglobulins that undergo reversible precipitation at cold temperatures. Monoclonal type-I cryoglobulinaemia is the least frequent and is associated to hematological diseases such as multiple myeloma, Waldenström's macroglobulinaemia, chronic lymphocytic leukaemia and lymphoma. We describe the case of a 60-year-old female patient, who suffered from burning pain in her feet for ten months before her admission. The patient presented intermittent distal cyanosis that progressed to digital ischaemia...
2020: Prague Medical Report
https://read.qxmd.com/read/32268672/-clinicopathological-analysis-of-patients-presenting-monoclonal-gammapathy-and-renal-damage
#12
JOURNAL ARTICLE
P Zhang, W Wang, Q Yao, Y Jin, Y R Zou, G S Li
No abstract text is available yet for this article.
April 8, 2020: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://read.qxmd.com/read/32092901/imaging-of-monoclonal-gammapathy-of-undetermined-significance-and-smoldering-multiple-myeloma
#13
REVIEW
Bastien Jamet, Clément Bailly, Thomas Carlier, Cyrille Touzeau, Anne-Victoire Michaud, Mickael Bourgeois, Philippe Moreau, Caroline Bodet-Milin, Françoise Kraeber-Bodere
Multiple myeloma (MM) is always preceded by an initial monoclonal gammopathy of undetermined significance (MGUS) that then develops into asymptomatic or smoldering multiple myeloma (SMM), which constitutes an intermediate clinical stage between MGUS and MM. According to a recent study, risk factors for faster MGUS to MM progression include an M protein of 1.5 g/dL or more and an abnormal free light chain ratio in patients with non-IgM MGUS. Therefore, the International Myeloma Working Group (IMWG) decided to recommend whole-body computed tomography (WBCT) for patients with high-risk MGUS in order to exclude early bone destruction...
February 19, 2020: Cancers
https://read.qxmd.com/read/32066411/persistent-elevation-of-aspartate-aminotransferase-in-a-child-after-incomplete-kawasaki-disease-a-case-report-and-literature-review
#14
REVIEW
Pejman Rohani, Farid Imanzadeh, Aliakbar Sayyari, Maryam Kazemi Aghdam, Reza Shiari
BACKGROUND: Interpretation of abnormalities in liver function tests, especially in asymptomatic children, is a common problem faced by clinicians. Isolated elevation of aspartate aminotransferase may further puzzle physicians. Macro-aspartate aminotransferase (AST) results from complexes AST produces with other plasma components, such as immunoglobulin. To our knowledge, this is the first report on a case of macro-AST-associated incomplete Kawasaki disease (KD). It is to make physicians aware of this benign condition and help to prevent extensive, unnecessary investigations and invasive workups...
February 17, 2020: BMC Pediatrics
https://read.qxmd.com/read/31967972/poems-syndrome-a-rare-cause-of-adrenal-insufficiency-in-a-young-male
#15
JOURNAL ARTICLE
Joanna Prokop, João Estorninho, Sara Marote, Teresa Sabino, Aida Botelho de Sousa, Eduardo Silva, Ana Agapito
SUMMARY: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. We describe a case of a 33-year-old patient, in whom the presenting symptoms were mandibular mass, chronic sensory-motor peripheral polyneuropathy and adrenal insufficiency...
September 17, 2019: Endocrinology, Diabetes & Metabolism Case Reports
https://read.qxmd.com/read/31746572/-management-of-peripheral-neuropathy-in-general-internal-medicine
#16
REVIEW
Raphaël Bernard-Valnet, Thierry Kuntzer, Julien Vaucher
Peripheral neuropathies are frequent in clinical practice and portend severe functional limitations or neuropathic pain. History taking and clinical examination represent key features to trigger investigations and uncover the underlying affection. For general internal medicine practitioners, initial investigation should include search for alcohol consumption, diabetes mellitus, vitamin B12 deficiency and monoclonal gammapathy. Nerve conduction studies should be limited to patients with atypical presentations and/or without a defined etiology...
November 20, 2019: Revue Médicale Suisse
https://read.qxmd.com/read/31671389/-hypervitaminosis-b12-our-experience-and-a-review
#17
REVIEW
Abrar-Ahmad Zulfiqar, Emmanuel Andres, Noel Lorenzo Villalba
High serum levels of vitamin B12 or cobalamin, also called hypervitaminemia B12, is a frequently underestimated biological abnormality. According to the literature, some of the entities related to this finding are solid neoplasia (primary or metastatic) and acute or chronic hematological diseases. Other causes include liver disorders, monoclonal gammapathy of undetermined significance, renal failure and, less frequently, excess of vitamin B12 intake, inflammatory or autoimmune diseases, and transient hematological disorders (neutrophilia and secondary eosinophilia)...
2019: Medicina
https://read.qxmd.com/read/30869332/-conduct-in-case-of-monoclonal-gammapathy
#18
JOURNAL ARTICLE
Olivier Decaux
Conduct in case of monoclonal gammapathy ? Monoclonal gammopathies are frequent in general population (about 3 % beyond 50 years) and their prevalence increases with age. They may be associated with malignant haemopathy (multiple myeloma for IgG and IgA, Waldenström disease for IgM). In absence of malignant haemopathy, the diagnosis is that of monoclonal gammopathy of indeterminate significance (MGUS). MGUS accounts for the majority (over 60 %) cases of monoclonal gammopathy. Only patients with multiple myeloma or Waldenström disease require treatment...
September 2018: La Revue du Praticien
https://read.qxmd.com/read/30701922/immunoglobulinopathies-in-patients-with-angioimmunoblastic-t-cell-lymphoma
#19
JOURNAL ARTICLE
N G Chernova, N P Soboleva, S A Mariina, Y V Sidorova, M N Sinitsyna, V N Dvirnyk, D S Badmazhapova, Y E Vinogradova, E E Zvonkov, V G Savchenko
AIM: The aim of the study was to characterize quantitative and qualitative immunoglobulinopathies in patients with AITL at the onset of the disease. MATERIALS AND METHODS: 55 patients with newly diagnosed AITL were enrolled in the study, the male/female ratio was 30/25; median age was 61 (29-81) years. Diagnosis was based on standard WHO criteria. Immunochemical studies of blood serum included serum protein electrophoresis/immunofixation, nephelometric quantification of total immunoglobulins, serum free light chain assay...
August 17, 2018: Terapevticheskiĭ Arkhiv
https://read.qxmd.com/read/30078781/-lymphoplasmacytic-lymphoma-or-multiple-myeloma-immunoglobulins-may-lead-to-misdiagnosis
#20
JOURNAL ARTICLE
Jérôme Debus, Pierre Lemaire, Mustafa Alani, Jonathan Farhi, Johann Rose, Vincent Cussac, Sabine Defasque, Nathalie Denizon, Habib Ghnaya, Anne Besançon-Bergelin, Kamel Laribi, Fabienne Pineau-Vincent
We report here a case of lymphoplasmacytic lymphoma with IgA paraproteinemia and a case of concomitant Waldenström macroglobulinemia and monoclonal gammapathy of unknown significance. These rare cases show that the isotype of a monoclonal immunoglobulin does not allow to foresee every time the underlying pathology. Clinical data and medical imaging are essential. From a biological point of view, additional analysis such as immunophenotyping, cytogenetics and molecular biology are required in addition to the cytological features in order to make an accurate differential diagnosis between lymphoid and plasma cell malignancy...
August 1, 2018: Annales de Biologie Clinique
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