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IgA gammopathy

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...
April 10, 2017: Hematological Oncology
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...
March 9, 2017: Annals of Hematology
Ranjit Nair, Shereen Gheith
No abstract text is available yet for this article.
March 9, 2017: Blood
Abhik Banerjee, Kshama Pimpalgaonkar, Alap Lukiyas Christy
Multiple myeloma is a debilitating malignancy arising from plasma cells. These malignant plasma cells called myeloma cells proliferate and infiltrate the bone marrow. The disease is characterized by the presence of a monoclonal protein in plasma and/or the urine. In this report, we present a case of biclonal multiple myeloma which showed two M bands on serum protein electrophoresis. The patient had elevated serum IgA and IgG levels. To reveal the nature of M bands or clonality, serum Immunofixation study was performed which revealed IgA with Lambda and IgG with Kappa light chains...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
Emily D Rout, Alba Maria M Shank, Angharad H K Waite, Andrea Siegel, Anne C Avery, Paul R Avery
A 5-year-old male neutered Bernese Mountain Dog was presented for cutaneous plasmacytoma, which was treated by surgical excision. Four months later, the dog developed multiple skin masses, hyphema, pericardial and mild bicavitary effusions, myocardial masses, and marked plasmacytosis in the peripheral blood. Circulating plasma cells expressed CD34 and MHC class II by flow cytometry. Immunocytochemistry demonstrated that these cells were strongly positive for multiple myeloma oncogene 1/interferon regulatory factor 4 (MUM-1) and weakly to moderately positive for Pax5...
February 10, 2017: Veterinary Clinical Pathology
Marguerite Vignon, Camille Cohen, Stanislas Faguer, Laure-Hélène Noel, Celine Guilbeau, Marion Rabant, Sarah Higgins, Aurélie Hummel, Alexandre Hertig, Hélène Francois, Moglie Lequintrec, Eve Vilaine, Bertrand Knebelmann, Jacques Pourrat, Dominique Chauveau, Jean-Michel Goujon, Vincent Javaugue, Guy Touchard, Khalil El Karoui, Frank Bridoux
Monoclonal gammopathy of renal significance (MGRS) regroups renal disorders caused by a monoclonal immunoglobulin without overt hematological malignancy. MGRS includes tubular disorders, glomerular disorders with organized deposits, and glomerular disorders with non-organized deposits, such as proliferative glomerulonephritis with monoclonal IgG deposits. Since glomerular involvement related to monotypic IgA deposits is poorly described we performed retrospective analysis and defined clinico-biological characteristics, renal pathology, and outcome in 19 referred patients...
March 2017: Kidney International
Yukinori Harada, Kei Sakai, Shiho Asaka, Kazutaka Nakayama
Few cases of IgA nephropathy with angioimmunoblastic T-cell lymphoma (AITL) have been reported. We herein present the case of a 79-year-old Japanese man with AITL and IgA nephropathy. The patient presented with generalized edema, fatigue, and fever. Laboratory investigations revealed polyclonal gammopathy with a high level of IgA, microscopic hematuria, proteinuria, and some other immunological abnormalities. Computed tomography revealed generalized lymphadenopathy. A diagnosis of AITL and IgA nephropathy was made based on inguinal lymph node and renal biopsies...
2017: Internal Medicine
C Lartigau-Roussin, M H Paclet, H Audin, B Toussaint, T Henni, L Raffray
INTRODUCTION: Macro-AST is recognized as a classical aetiology of isolated and persistent increase of serum aspartate aminotransferase (AST) levels. Macro-AST are high molecular weight complexes associating AST and a macromolecule, often an immunoglobulin. Although those macroenzymes of unknown pathogenesis are usually non-pathogenic, association with several diseases, including autoimmune diseases and liver diseases has been described. CASE REPORT: We report here the case of a 45-year-old patient with previously normal liver enzymes in whom an AST elevation and an IgA monoclonal gammopathy were discovered concomitantly...
October 13, 2016: La Revue de Médecine Interne
Natalia Ermak, Thao Nguyen-Khoa, Marie-Alexandra Alyanakian
The diagnostics and follow-up of monoclonal gammopathies such as multiple myeloma require precise analysis of the monoclonal component as well as the other immunoglobulins isotypes, which might be limited by the sensitivity of standard laboratory methods. New serum biomarkers were developed for routine practice in the last decades, such as the free light chain assays and more recently the heavy/light chain assay<strike>s</strike>. Studies have shown that serum free light chain measurement was useful in the identification and follow-up of pauci or nonsecretory myeloma, free light-chain multiple myeloma and AL amyloidosis...
October 1, 2016: Annales de Biologie Clinique
Shintaro Hayashi, Shun Nagamine, Kouki Makioka, Susumu Kusunoki, Koichi Okamoto
A 71-year-old woman with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with IgA-λ monoclonal gammopathy of undetermined significance (MGUS) showed the acute development of tetraplegia, respiratory failure, and a marked fluctuation of the blood pressure. Intravenous (IV) high-dose steroid therapy (methylprednisolone: 1 g/day × 3 days), followed by oral prednisolone (PSL) (40 mg/day), and IV immunoglobulin (IVIg, 0.4 g/kg/day × 5 days) administrations resulted in the amelioration of these symptoms...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
Guru Prasad Patnala, Anila P Sunandini, Rama Rayavarapu, Padmasri Somala Yandapalli
Erythema elevatum diutinum (EED) is a rare form of vasculitis characterized clinically by red-violet brown papules, plaques, and nodules mainly involving the extensor surfaces; histologically by leukocytoclastic vasculitis in early lesions, and fibrosis and cholesterolosis in late lesions. EED has been associated with many systemic disorders including infections, autoimmune disorders, and both benign and malignant hematological disorders. As it is a rare form of vasculitis and only 250 cases reported till date, we report a case of EED in association with IgA monoclonal gammopathy with partial response to dapsone treatment...
July 2016: Indian Dermatology Online Journal
P A von dem Borne, M F Jonkman, R van Doorn
Subcorneal pustular dermatosis (SPD), or Sneddon-Wilkinson disease, is a rare pustular skin disease that follows a chronic relapsing course. A well-known association exists between SPD and IgA monoclonal gammopathy of undetermined significance (MGUS), which exists in up to 40% of cases. SPD has also been observed in patients with IgA myeloma. In SPD, direct and indirect immunofluorescence studies do not reveal in vivo bound IgA to the epithelial cell surface, in contrast to IgA pemphigus, which has similar clinicopathological features...
August 12, 2016: British Journal of Dermatology
Seema Rao, Rakhee Kar, Hara Prasad Pati, Renu Saxena
Scleredema of Buschke is a rare fibromucinous connective tissue disorder of unknown etiology. It is often associated with a benign monoclonal gammopathy and rarely with myelomatosis. We report a case of scleredema-associated IgA myeloma with myelofibrosis in a 24-year-old male patient. Scleredema generally affects young adults and onset of associated monoclonal gammopathy is at a younger age than when not associated with scleredema. However, presentation at a much younger age (24 years in our case) is very unusual...
December 5, 2008: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
İbrahim Tek, Dilsa Mızrak, Güngör Utkan, Selami Koçak Toprak, Hüseyin Tutkak, Abdullah Büyükçelik, Bulent Yalçın, Hakan Akbulut, Fikri İçli
No abstract text is available yet for this article.
June 5, 2010: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
N W C J van de Donk, T Mutis, P J Poddighe, H M Lokhorst, S Zweegman
Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common premalignant disorders. IgG and IgA MGUS are precursor conditions of multiple myeloma (MM), whereas light-chain MGUS is a precursor condition of light-chain MM. Smoldering MM (SMM) is a precursor condition with higher tumor burden and higher risk of progression to symptomatic MM compared to MGUS. Assessment of the risk of progression of patients with asymptomatic monoclonal gammopathies is based on various factors including clonal burden, as well as biological characteristics, such as cytogenetic abnormalities and light-chain production...
May 2016: International Journal of Laboratory Hematology
Laura Magnano, Carlos Fernández de Larrea, Montserrat Elena, María Teresa Cibeira, Natalia Tovar, Juan I Aróstegui, Fabiola Pedrosa, Laura Rosiñol, Xavier Filella, Jordi Yagüe, Joan Bladé
BACKGROUND: Asymptomatic monoclonal gammopathies, such as monoclonal gammopathy of undetermined significance (MGUS) and smoldering myeloma (SMM), are clinical conditions that usually precede symptomatic multiple myeloma. Therefore, risk stratification is crucial owing to the heterogeneous progression rate among these patients. In previous years, suppression of the uninvolved chain of specific heavy/light chain (HLC) pairs in serum has been identified as a new risk factor in MGUS. The aim of the present study was to investigate the prognostic effect of involved and uninvolved HLC pairs and HLC ratios on progression in a series of patients with MGUS and SMM...
June 2016: Clinical Lymphoma, Myeloma & Leukemia
Mohamad Zaidan, Florent Plasse, Marion Rabant, Vincent Javaugue, Bertrand Knebelmann, Marie-Alexandra Alyanakian, Dominique Joly, Dominique Nochy, Frank Bridoux
Cryoglobulins are circulating immunoglobulins that precipitate with cold temperature and dissolve with rewarming. Type 1 cryoglobulinemia is composed of a single monoclonal immunoglobulin and is associated with renal involvement in up to 40% of cases. Type 1 cryoglobulinemia is related to an underlying B-cell haematological malignancy in 60% of patients. In the remaining cases, in the absence of criteria for malignancy, the diagnosis of monoclonal gammopathy of renal significance should be established. The clinical and biological setting and histological features of type 1 cryoglobulinemia are globally similar to those of mixed cryoglobulinemia...
April 2016: Néphrologie & Thérapeutique
J H Wang, W W Zhou, B X Liu, D L Man, Z D Yang, F R Liu, H Shang
The aim of the present study is to examine the expression level of peripheral mir-21 in multiple myeloma (MM) patients and to determine its clinical significance. MM patients (30), monoclonal gammopathy of undetermined significance (MGUS) patients (14), and normal controls (20) were recruited to determine the serum level of β2-MG, IgA and IgM, IgG, λ, κ, TP, ALB, Hb, LDH, and Ca(2+). Gene expression of mir-21 was quantified by SYBR green real-time fluorescent quantitative PCR. We found that the expression level of serum mir-21 in the MM group was significantly higher than the MGUS group and the NC group (P < 0...
January 22, 2016: Genetics and Molecular Research: GMR
Srinath Sundararajan, Abhijeet Kumar, Neha Korde, Amit Agarwal
Smoldering multiple myeloma (SMM) is a pre-malignant condition with an inherent risk for progression to multiple myeloma (MM). The 2014 IMWG guidelines define smoldering multiple myeloma as a monoclonal gammopathy disorder with serum monoclonal protein (IgG or IgA) ≥30 g/L or urinary monoclonal protein ≥500 mg per 24 h and/or clonal bone marrow plasma cells 10-60 % without any myeloma-defining events or amyloidosis. The risk for progression of SMM to MM vary based on clinical, laboratory, imaging, and molecular characteristics...
April 2016: Current Hematologic Malignancy Reports
Chih-Chun Chang, Ming-Jang Su, Shu-Jene Lee, Yu-Hui Tsai, Lin-Yin Kuo, I-Hsin Lin, Hui-Ling Huang, Tzung-Hai Yen, Fang-Yeh Chu
BACKGROUND: Whether ambient exposure to environmental pollutants leads to hematopoietic malignancies such as multiple myeloma (MM) remains to be ascertained. Therefore, we aimed to investigate the immunotyping distribution of serum monoclonal paraprotein and the environmental influence on MM and monoclonal gammopathy of uncertain significance (MGUS) in the Taiwanese population. MATERIALS AND METHODS: Serum protein electrophoresis with immunosubtraction by the capillary zone electrophoresis method was performed as primary screening for MM and MGUS...
2016: Asian Pacific Journal of Cancer Prevention: APJCP
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