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IgA gammopathy

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https://www.readbyqxmd.com/read/29766549/leukocytoclastic-vasculitis-associated-with-immunoglobulin-a-lambda-monoclonal-gammopathy-of-undetermined-significance-a-case-report-and-review-of-previously-reported-cases
#1
Hiroshi Umemura, Osamu Yamasaki, Keiji Iwatsuki
Leukocytoclastic vasculitis is often associated with immunoglobulin (Ig)A deposition on the vascular walls. IgA-associated leukocytoclastic vasculitis comprises various underlying diseases. Hematological disorders that can be minor triggers include multiple myeloma and monoclonal gammopathy of undetermined significance. Here, we present the case of a 78-year-old woman with leukocytoclastic vasculitis associated with monoclonal gammopathy of undetermined significance of the IgA lambda chain. Oral steroid administration initially showed remission of vasculitis; however, the condition recurred after four attempts of treatment withdrawal...
May 15, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29721239/meningitis-spondylodiscitis-pneumonia-and-septic-shock-with-streptococcus-pneumoniae-in-a-previously-healthy-woman-with-isolated-igg2-igg3-iga-deficiency-and-monoclonal-gammopathy-of-undetermined-significance
#2
Shahin Gaini, David Gudnason, Bjarni Á Steig, Jenny Jónsdóttir Nielsen
A 66 years old Caucasian woman with pneumococcal meningitis was treated and discharged after an uncomplicated course. Five months later she was readmitted with fever and right side abdominal pain and diagnosed with pneumococcal spondylodiscitis. One year later she was treated for a severe chest X-ray confirmed left lobar pneumonia. Two years later she was diagnosed with a pneumococcal pneumonia in her left lung with septic shock. An immune deficiency screen revealed slightly reduced IgA levels, low IgG2 levels, low IgG3 levels and high IgG1 levels...
March 29, 2018: Infectious Disease Reports
https://www.readbyqxmd.com/read/29686791/lung-consolidation-as-a-rare-presentation-of-lymphoplasmacytic-lymphoma-with-extramedullary-waldenstr%C3%A3-m-s-macroglobulinemia
#3
Omar Abdulfattah, Ebad Ur Rahman, Bikash Bhattarai, Sumit Dahal, Zainab Alnafoosi, David Trauber, Danilo Enriquez, Frances Schmidt
Objectives : Lymphoplasmacytic lymphoma (LPL) is a mature B cell lymphoma that usually involves the bone marrow, spleen and lymph nodes. Extramedullary involvement, including the lung, is rarely reported. Case description : A 73-year-old female initially presented to our hospital complaining of productive cough of white-colour sputum for three weeks duration. She reported unintentional weight loss of ten pounds over the last five months. There was no history of haemoptysis, fever, night sweats, chills, recent infections or hospitalization...
2018: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29656787/fifty-year-incidence-of-waldenstr%C3%A3-m-macroglobulinemia-in-olmsted-county-minnesota-from-1961-through-2010-a-population-based-study-with-complete-case-capture-and-hematopathologic-review
#4
Robert A Kyle, Dirk R Larson, Ellen D McPhail, Terry M Therneau, Angela Dispenzieri, Shaji Kumar, Prashant Kapoor, James R Cerhan, S Vincent Rajkumar
OBJECTIVE: To determine the incidence of Waldenström macroglobulinemia (WM) in a strictly defined geographic area over a 50-year period. PATIENTS AND METHODS: All residents of Olmsted County with a diagnosis of WM, consisting of a monoclonal IgM protein of any size and/or 10% or more lymphoplasmacytic infiltration of the bone marrow along with anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly requiring therapy, were identified from January 1, 1961, to December 31, 2010...
April 12, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29577510/successful-treatment-with-steroid-and-cyclosporine-a-in-a-patient-with-immunoglobulin-a-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-deposits
#5
Keisuke Sato, Shiho Makabe, Yuko Iwabuchi, Kaori Kojima, Masayo Sato, Hiroshi Kataoka, Takahito Moriyama, Sekiko Taneda, Ken Tsuchiya, Kosaku Nitta, Toshio Mochizuki
We report a case of glomerulonephritis with monoclonal immunoglobulin (Ig) A deposits as a form of monoclonal gammopathy of renal significance (MGRS) caused by monoclonal immunoglobulins without blood disorders in a 41-year-old woman. She developed lower leg oedema and was hospitalized because of nephrotic syndrome. Serum and urine were negative for M protein, and the free light chain κ/λ ratio was within the normal range. Renal histopathological findings included mesangial proliferation, endocapillary cell proliferation, and a double-contour appearance of the capillary walls...
March 25, 2018: Nephrology
https://www.readbyqxmd.com/read/29532268/monoclonal-gammopathy-in-rheumatic-diseases
#6
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29447637/crohn-disease-associated-neutrophilic-urticarial-dermatosis-report-and-literature-review-of-neutrophilic-urticarial-dermatosis
#7
REVIEW
Zhe Hou, Brian R Hinds, Philip R Cohen
Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized...
November 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29397379/analytical-validation-of-the-hevylite-assays-for-m-protein-quantification
#8
Joannes F M Jacobs, Inez-Anne Haagen, Astrid Lodder, Cieleke van der Kroft, Corrie M de Kat Angelino, Sandra Croockewit, Ed Nieuwenhuys, Kyra A Gelderman
BACKGROUND: The heavy/light chain (HLC) immunoassay quantifies the different heavy chain/light chain combinations of each immunoglobulin (Ig) class. This makes the HLC assay suited to quantify monoclonal immunoglobulins (M-protein) and for monitoring of patients with monoclonal gammopathies. This method is particularly advantageous for those samples in which electrophoretic quantification of the M-protein is not possible. METHODS: In this study we tested the analytical performance of the HLC assay in 166 routine clinical samples and in 27 samples derived from the Dutch external quality assessment (EQA) for M-protein diagnostics (74 participating laboratories)...
February 5, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29326807/heavy-chain-diseases-and-myeloma-associated-fanconi-syndrome-an-update
#9
REVIEW
Roberto Ria, Franco Dammacco, Angelo Vacca
The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the production of a monoclonal immunoglobulin heavy chain without an associated light chain. There are three types of HCD, defined by the class of immunoglobulin heavy chain produced: IgA (α-HCD), IgG (γ-HCD), and IgM (μ-HCD). Alpha-HCD is the most common and usually occurs as intestinal malabsorption in a young adult from a country of the Mediterranean area. Gamma- and μ-HCDs are rarer and associated with a B-cell non-Hodgkin lymphoma that produces an abnormal Ig heavy chain...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29324245/heavy-light-chain-assay-as-a-biomarker-for-diagnosis-and-follow-up-of-multiple-myeloma
#10
Hyojin Chae, Eunhee Han, Jaeeun Yoo, Jaewoong Lee, Jeong Joong Lee, Kyoungho Cha, Myungshin Kim, Yonggoo Kim, Sung-Eun Lee, Chang-Ki Min
BACKGROUND: The heavy-light chain (HLC) assay enables the accurate measurement of each isotype-specific heavy and light chain (i.e., IgGĸ, IgGλ, IgAĸ, and IgAλ) and the derivation of an HLC-pair ratio. However, to date, only limited data have validated the usefulness of serial HLC measurements in the routine follow-up of intact immunoglobulin multiple myeloma (MM) patients. METHODS: A total of 36 diagnostic and 671 post-treatment sera from 115 IgG and 61 IgA MM patients were assessed with capillary zone electrophoresis, immunosubtraction electrophoresis, total immunoglobulin measurement, free light chain, and HLC assay...
April 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29323083/monoclonal-gammopathy-with-double-m-bands-an-atypical-presentation-on-serum-protein-electrophoresis-simulating-biclonal-gammopathy
#11
Kaustubh Bora, Umesh Das, Bhupen Barman, Alice Abraham Ruram
Monoclonal gammopathies, such as multiple myeloma, typically exhibit high levels of a monoclonal immunoglobulin (M-protein), produced by a clone of abnormally proliferating B-lymphocytes and/or plasma cells. The M-protein can be evaluated by serum protein electrophoresis (SPEP), which yields a single discrete band (M-band), usually in the γ-globulin region. Rarely, two M-bands appear simultaneously at different positions during SPEP - a condition known as biclonal gammopathy, which is a result of clonal expansion of two different neoplastic cell lines...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29146225/response-comparison-of-multiple-myeloma-and-monoclonal-gammopathy-of-undetermined-significance-to-the-same-anti-myeloma-therapy-a-retrospective-cohort-study
#12
John P Campbell, Jennifer L J Heaney, Sankalp Pandya, Zaheer Afzal, Martin Kaiser, Roger Owen, J Anthony Child, David A Cairns, Walter Gregory, Gareth J Morgan, Graham H Jackson, Chris M Bunce, Mark T Drayson
BACKGROUND: Multiple myeloma is consistently preceded by monoclonal gammopathy of undetermined significance (MGUS), which is usually only treated by a form of anti-multiple myeloma therapy if it is causing substantial disease through deposition of secreted M proteins. However, studies comparing how MGUS and multiple myeloma plasma cell clones respond to these therapies are scarce. Biclonal gammopathy multiple myeloma is characterised by the coexistence of an active multiple myeloma clone and a benign MGUS clone, and thus provides a unique model to assess the responses of separate clones to the same anti-multiple myeloma therapy, in the same patient, at the same time...
December 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#13
LETTER
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
No abstract text is available yet for this article.
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#14
REVIEW
Eduardo Nobile-Orazio, Mariangela Bianco, Andrea Nozza
Purpose of review Several advances have been made on the pathogenesis and therapy of neuropathies associated with paraproteinemia (monoclonal gammopathy). It is important for the neurologist to understand the pathogenetic relevance of this association especially when the hematological disease does not require per se any therapy. Recent findings Treatment of the neuropathy in patients with malignant paraproteinemia is mainly addressed by the hematologist while the neurologist is mainly involved in the initial diagnosis and in deciding whether the neuropathy is caused by the disease or by the chemotherapy used for the disease...
October 16, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28893029/poems-syndrome-with-biclonal-gammopathy-a-rare-association
#15
Dibyendu De, Sandeepan Halder, Suvro Sankha Datta
Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Here, we are describing the cases of a 60-year-old lady, presented with bilateral pedal oedema and pericardial effusion and peripheral neuropathy. She also had hepatosplenomegaly, hyperpigmented rash and hypothyroidism and hyperparathyroidism...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28780374/maternal-t-and-b-cell-engraftment-in-two-cases-of-x-linked-severe-combined-immunodeficiency-with-igg1-gammopathy
#16
Tsubasa Okano, Takuro Nishikawa, Eri Watanabe, Takashi Watanabe, Takehiro Takashima, Tzu-Wen Yeh, Motoi Yamashita, Mari Tanaka-Kubota, Satoshi Miyamoto, Noriko Mitsuiki, Masatoshi Takagi, Yoshifumi Kawano, Yoshiki Mochizuki, Kohsuke Imai, Hirokazu Kanegane, Tomohiro Morio
X-linked severe combined immunodeficiency (X-SCID), caused by defects in the common gamma chain, is typically characterized by T and NK cell defects with the presence of B cells. T cell dysfunction and impaired class-switch recombination of B cells mean that patients typically have defects in class-switched immunoglobulins (IgG, IgA, and IgE) with detectable IgM. Here, we describe two patients with X-SCID with IgG1 gammopathy, in whom we identified maternal T and B cell engraftment. Exclusively, maternal B cells were found among the IgD- CD27+ class-switched memory B cells, whereas the patients' B cells remained naïve...
October 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28775105/poems-syndrome-in-a-patient-with-rheumatoid-arthritis
#17
Takahiko Akagi, Tomoyuki Mukai, Shoko Kodama, Yoshitaka Morita
Multiple myeloma has been reported to be associated with rheumatoid arthritis (RA). POEMS syndrome is a rare variant of multiple myeloma and is characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report the case of a 67-year-old patient with RA who developed numbness and tingling in both legs due to polyneuropathy. CT showed a massive right pleural effusion and a sclerotic lesion in the right ninth rib. Histopathological examination of the rib revealed IgA lambda-type plasmacytoma...
August 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28602036/circulating-adiponectin-levels-differ-between-patients-with-multiple-myeloma-and-its-precursor-disease
#18
Jonathan N Hofmann, Sham Mailankody, Neha Korde, Ye Wang, Nishant Tageja, Rene Costello, Adriana Zingone, Malin Hultcrantz, Michael N Pollak, Mark P Purdue, Ola Landgren
OBJECTIVE: An increased risk of multiple myeloma (MM) has been observed among individuals with low prediagnostic circulating levels of adiponectin, a metabolic hormone that is typically underexpressed among those with overweight or obesity. To assess whether adiponectin may influence myeloma development or progression to frank MM, circulating adiponectin levels were compared across patients with different stages of MM and its precursor, monoclonal gammopathy of undetermined significance (MGUS)...
August 2017: Obesity
https://www.readbyqxmd.com/read/28397326/diagnosis-and-management-of-neuropathies-associated-with-plasma-cell-dyscrasias
#19
REVIEW
Evan Rosenbaum, Douglas Marks, Shahzad Raza
Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Fortunately, with increasing understanding into the biologic mechanisms of underlying hematologic diseases, additional biomarkers have recently been developed, thus improving our diagnostic capacity...
February 2018: Hematological Oncology
https://www.readbyqxmd.com/read/28280994/detection-of-myd88-l265p-and-whim-like-cxcr4-mutation-in-patients-with-igm-monoclonal-gammopathy-related-disease
#20
Xin-Xin Cao, Qi Meng, Hao Cai, Tian-Hua He, Cong-Li Zhang, Wei Su, Jian Sun, Yue Li, Wei Xu, Dao-Bin Zhou, Jian Li
A broad spectrum of diseases are associated with IgM monoclonal gammopathy, including Waldenstrom macroglobulinemia (WM), various types of B cell non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), and monoclonal gammopathy of undetermined significance (MGUS); these are called IgM monoclonal gammopathy related diseases (IgM-RD). We investigated MYD88 L265P and WHIM-like CXCR4 mutations in various IgM-RD. Patients with serum immunofixation electrophoresis confirmed IgM monoclonal gammopathy who had enough material for DNA extraction and presented between January 2008 and October 2016 at Peking Union Medical College Hospital were enrolled in this cohort...
June 2017: Annals of Hematology
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