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Paraproteinemias (Monoclonal Gammopathy)

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A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin.

Also Known As:

Monoclonal Gammopathy; Paraproteinemia; Monoclonal Gammapathies; Cell Dyscrasia, Plasma; Cell Dyscrasias, Plasma; Dyscrasia, Plasma Cell; Dyscrasias, Plasma Cell; Gammapathies, Monoclonal; Gammopathies, Monoclonal; Monoclonal Gammapathy; Paraimmunoglobulinemia; Plasma Cell Dyscrasia; Gammapathy, Monoclonal; Gammopathy, Monoclonal; Monoclonal Gammopathies; Paraimmunoglobulinemias; Plasma Cell Dyscrasias

Networked: 2176 relevant articles (47 outcomes, 118 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Multiple Myeloma
2.	Amyloidosis
3.	Polyneuropathies (Polyneuropathy)
4.	Pemphigus (Pemphigus Vulgaris)
5.	Paraproteinemias (Monoclonal Gammopathy)

Experts

1.	Kyle, Robert A: 19 articles (04/2015 - 01/2002)
2.	Gertz, Morie A: 17 articles (10/2015 - 01/2002)
3.	Dispenzieri, Angela: 17 articles (04/2015 - 01/2002)
4.	Leung, Nelson: 11 articles (07/2015 - 08/2008)
5.	Sethi, Sanjeev: 11 articles (07/2015 - 11/2010)
6.	Notermans, N C: 11 articles (02/2010 - 05/2000)
7.	Rajkumar, S Vincent: 10 articles (07/2015 - 01/2002)
8.	Eurelings, M: 10 articles (02/2010 - 01/2001)
9.	Fermand, Jean-Paul: 9 articles (11/2015 - 04/2002)
10.	Treon, Steven P: 9 articles (03/2013 - 04/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Paraproteinemias:

1.	Immunoglobulin A (IgA)IBA 04/01/1998 - "The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity." 12/01/1987 - "The clinical features and results of serological studies of a patient with Sjögren's syndrome, IgA kappa monoclonal gammopathy, and hyperviscosity syndrome are reported. " 09/01/2014 - "[Pseudohypobicarbonatemia in a patient with a kappa IgA monoclonal gammapathy]." 01/01/2014 - "Using immunohistochemistry and mass spectrometry we demonstrate that the disease resulted from an IgA-κ restricted plasma cell dyscrasia. " 10/01/2013 - "Serum protein electrophoresis revealed an IgA-kappa monoclonal gammopathy. "
2.	Melphalan (Alkeran)FDA LinkGeneric 10/01/1996 - "Among the 17 cases of FIO reported to date, four patients exhibited a monoclonal gammopathy (MCG) and one, treated with melphalan, was the first patient to present clinical and histological remission of the bone and plasma cell manifestations. " 01/01/2001 - "In order to assess whether EC overgrowth in MM reflects a spontaneous in vitro angiogenesis, BM mononucleated cells from 13 untreated (UT) MM, 20 treated (11 with melphalan and nine with DAV schedule) MM, eight patients with monoclonal gammopathy of uncertain significance (MGUS) and eight controls were seeded in an unselective medium to assess EC proliferation. " 04/01/2011 - "Only four of 15 patients with neuropathy were able to receive intensive but promising chemotherapy with a large dose of melphalan for plasma cell dyscrasia. " 08/01/2013 - "Immunosuppressant and immunomodulating therapies have been shown to be ineffective but clinical improvement accompanied by disappearance of monoclonal gammopathy and even nemaline bodies was reported following autologous stem cell transplantation and chemotherapy with melphalan. " 03/01/2003 - "High-dose intravenous melphalan and autologous stem cell transplantation induce remission of the plasma cell dyscrasia in a significant proportion of patients with AL amyloidosis. "
3.	Immunoglobulin G (IgG)IBA 05/01/2014 - "IgG monoclonal gammapathy was diagnosed in a systemic study. " 01/01/2008 - "Contribution of electron microscopy to the study of neuropathies associated with an IgG monoclonal paraproteinemia." 06/01/1991 - "Further studies revealed IgG(lambda) monoclonal gammopathy. " 01/01/1989 - "IgG paraproteinemias: a comparative CSF and serum study." 11/01/2015 - "All five patients had hyperglobulinemia due to IgG1 or IgG4/7 monoclonal gammopathy. "
4.	bortezomib (Velcade)FDA Link 11/01/2004 - "Herein, we review the principles underlying the use of gene expression profiling for delineation of the mechanisms of action of bortezomib against plasma cell dyscrasias, as well as for identification of potential predictors of the clinical activity of bortezomib. " 04/01/2013 - "This suggested that plasma cell-rich acute rejection with monoclonal gammopathy in this patient might have been in an early stage of kappa light chain-producing posttransplant lymphoproliferative disease confined to the renal allograft, and that bortezomib may be effective in treating a patient with this condition." 04/01/2013 - "Subsequent treatment with bortezomib improved graft function and caused the monoclonal gammopathy to resolve. " 07/01/2015 - "Bortezomib has been increasingly used especially in renal related indications such as multiple myeloma and monoclonal gammopathy of renal significance. " 02/01/2006 - "Testing is ongoing to determine bortezomib's role in front-line therapy of this plasma cell dyscrasia, as well as in non-Hodgkin's lymphoma, in which encouraging single-agent activity has been seen. "
5.	Immunoglobulin M (IgM)IBA 01/01/2015 - "Complementary study revealed the presence of blood IgM monoclonal gammopathy and a massive bone marrow lymphoplasmacytic infiltration. " 02/01/2011 - "In this study we have investigated 94 patients with IgM monoclonal gammopathies, in which a BM trephine biopsy and morphological and flow cytometry (FCM) evaluation of BM aspirate were available at diagnosis. " 08/01/2006 - "Here, this study describes four of 317 (1.2%) patients with an IgM monoclonal gammopathy in whom the morphologic and clinical features were consistent with a diagnosis of IgM myeloma." 06/01/2001 - "Serous macular detachments in a patient with IgM paraproteinemia: an optical coherence tomography study." 01/23/2001 - "To study the independent contribution of potential prognostic factors to the prediction of outcome of neuropathy associated with IgM monoclonal gammopathy. "
6.	Immunoglobulins (Immunoglobulin)IBA 12/01/1992 - "To study if immunoglobulin (Ig)-secreting cells actively pass the blood-brain barrier (BBB), 15 patients with monoclonal gammopathy underwent bonemarrow (BM) iliac crest aspiration biopsy, peripheral blood (PB) sampling and cerebrospinal fluid (CSF) analysis. " 05/01/1990 - "A study was made of 29 patients with monoclonal gammopathies to detect aberrations in immunoglobulin (Ig) light chain isotype expression in lymphocytes at various levels of B-cell differentiation, namely, circulating surface Ig positive (SIg+) cells, Ig-secreting cells (plaque forming cells, PFC) and mitogen-induced PFC. " 09/01/1987 - "Further studies indicated that the patient had an indolent plasma-cell dyscrasia, resulting in excessive production of a monoclonal immunoglobulin. " 01/01/1984 - "A large number of mature plasma cells was demonstrated in the bone marrow, and immunocytochemical studies revealed a considerable increase in the proportion of plasma cells which were positive for kappa light chains of immunoglobulins, indicating a monoclonal gammopathy. " 06/15/2015 - "Plasma cell proliferative disorders are generally classified as monoclonal gammopathies given most are associated with the excess secretion of a monoclonal immunoglobulin or M-protein. "
7.	Intravenous Immunoglobulins (IVIG)FDA Link 09/01/2014 - "The first case shows a patient without paraproteinemia who had success with ECP and IVIG, and the second is a patient with paraproteinemia treated effectively with IVIG. " 02/01/2002 - "Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. " 12/01/2005 - "Some small studies and reports of clinical cases presented in this article found benefit from IVIg in treating PN associated with diabetes, paraproteinemia, HIV, multisystem rheumatic diseases, and paraneoplastic PN. " 11/01/1994 - "Results obtained from small numbers of patients treated in open studies suggest that CIDP patients with a monoclonal gammopathy may also improve after IVIg treatment. " 01/01/2015 - "To our knowledge, this is the first report in our country of the successful use of IVIG therapy to treat a patient with IgGλ monoclonal gammopathy and related sensory ataxic axonal polyneuropathy."
8.	Amyloid (Amyloid Fibrils)IBA 12/01/2003 - "Despite complete remission of the plasma cell dyscrasia and improvement of glomerular permeability, the amount of glomerular amyloid mass did not regress." 03/01/1986 - "Autopsy studies showed amyloid deposition in the ventricular myocardium and conduction system; there were no deposits in other viscera, and evidence of a plasma cell dyscrasia was lacking. " 05/01/2014 - "No monoclonal gammopathy and/or amyloid deposition were found. " 05/01/2013 - "SPEP/UPEP/IFE detected a monoclonal gammopathy in 76 individuals, 56 with AL and 20 with ATTR amyloid and was overall a poor predictor of AL amyloid in this patient population: specificity (75%; 95% CI, 65-83%) and positive predictive value (PPV 74%; 95% CI, 63-82%). " 05/01/2013 - "Relationship between monoclonal gammopathy and cardiac amyloid type."
9.	Dexamethasone (Maxidex)FDA LinkGeneric 01/01/2007 - "He became completely asymptomatic after specific treatment with dexamethasone of the paraproteinemia associated with MM. We discuss its pathophysiology in this report." 01/01/2000 - "Pulses of dexamethasone (40 mg) were administered and led to a transient decrease of paraproteinemia to a minimum of 31.9 g/l, followed by an increase to 62 g/l. " 07/01/1997 - "Pulsed high-dose dexamethasone treatment of polyneuropathy associated with monoclonal gammopathy." 06/01/2011 - "We describe the first case, to our knowledge, of NXG with associated monoclonal gammopathy treated with thalidomide plus dexamethasone, achieving complete resolution of the skin lesions and sustaining response more than 3 years after treatment." 01/01/2014 - "Phase I/II studies using ixazomib weekly or twice weekly in relapsed/refractory MM patients suggested antitumor activity of the single agent, but more promising results have been obtained with the combination of ixazomib, lenalidomide, and dexamethasone in newly diagnosed MM. Ixazomib has also been used in systemic amyloidosis as a single agent, showing important activity in this difficult-to-treat plasma-cell dyscrasia. "
10.	Prednisone (Sone)FDA LinkGeneric 07/01/1990 - "Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone." 04/01/1998 - "However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. " 06/01/2002 - "Treatment with melphalan and prednisone resulted in great improvement of cutaneous lesions and paraproteinemia remission. " 07/01/2008 - "In this study we report a patient with chronic progressive sensory ataxia, proximal weakness, immunoglobulin M (IgM) monoclonal gammopathy, and elevated protein levels in the cerebrospinal fluid, who showed a good response to prednisone. " 06/01/2002 - "This case report details how melphalan and prednisone can be administered in the successful treatment of necrobiotic xanthogranuloma with lambda paraproteinemia."

Therapies and Procedures

1.	Stem Cell Transplantation 07/01/2002 - "The most interesting therapeutic approach is autologous-blood stem-cell transplantation, which may produce a complete remission of the plasma-cell dyscrasia and a substantial improvement of clinical manifestations related to LC deposits." 11/01/2010 - "Prospective evaluation of epstein-barr virus reactivation after stem cell transplantation: association with monoclonal gammopathy." 09/01/2006 - "Frequent gain of chromosome band 1q21 in plasma-cell dyscrasias detected by fluorescence in situ hybridization: incidence increases from MGUS to relapsed myeloma and is related to prognosis and disease progression following tandem stem-cell transplantation." 04/15/2002 - "We treated 5 patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and multifocal bone lesions or diffuse bone marrow plasmacytic infiltration with high-dose therapy (HDT) and autologous blood stem cell transplantation. " 12/01/2003 - "High-dose chemotherapy followed by autologous blood stem cell transplantation induces remission of plasma cell dyscrasia in patients with AL amyloidosis. "
2.	Drug Therapy (Chemotherapy) 01/01/2008 - "Chemotherapy stabilized or improved renal function in 3 of nine patients (33%) with non-malignant monoclonal gammopathy. " 09/01/2014 - "The main purpose of identification of these earlier phases of the plasma cell dyscrasia was to identify patients who do not warrant treatment with chemotherapy, in whom the risk of treatment outweighs the benefit. " 05/01/2011 - "Chromosome change at 20q11-q12, including del(20q), is sometimes reported in plasma cell dyscrasia, but most cases are found during or after chemotherapy. " 11/01/2002 - "In each group, we studied clinicopathological features, associated monoclonal gammapathy (detected by immunoelectrophoresis and/or immunoblot) or B-cell lymphoproliferative disease, effects of chemotherapy and long-term renal outcome. " 12/01/1995 - "Thus, we suggest that plasma cell dyscrasias should be classified simply into two main groups according to the need of immediate chemotherapy. "
3.	Transplants (Transplant) 03/01/2015 - "Monoclonal gammopathy of renal significance with light-chain deposition disease diagnosed postrenal transplant: a diagnostic and therapeutic challenge." 05/01/2014 - "Three (21%) patients were positive for monoclonal gammopathy and had a faster rate of recurrence and graft loss. " 12/01/2013 - "Recovery of renal function succeeding stem cell transplant: a case of C3 Glomerulonephiritis secondary to monoclonal gammopathy." 08/01/2013 - "Here we report our results and recommendations regarding the management of common issues related to the donor: pre-transplant pregnancy and monoclonal gammopathy. " 03/01/2012 - "Initial survival data of kidney transplant patients with pre-transplant monoclonal gammopathy."
4.	Enzyme Replacement Therapy 10/01/2007 - "Our observation and previous reports suggest that enzyme replacement therapy is more effective in polyclonal hypergammaglobulinaemia than in monoclonal gammopathy." 05/01/1998 - "We report the effects of enzyme replacement therapy in a patient with Gaucher's disease associated with a monoclonal gammopathy. " 01/15/2012 - "Extended remission of B-cell lymphoma with monoclonal gammopathy in a patient with type 1 Gaucher disease treated with enzyme replacement therapy."
5.	Transplantation (Transplant Recipients) 09/01/2015 - "Patients with TEMPI syndrome have responded to therapies used to treat plasma cell dyscrasias and may benefit from autologous HPC transplantation. " 04/01/2013 - "Plasma cell-rich acute rejection with monoclonal gammopathy in a renal transplant recipient." 01/01/2004 - "A long-term follow-up of patients with paraproteinemia after HSC transplantation is needed to confirm this finding in a larger series of patients." 01/01/2004 - "These findings indicate that paraproteinemia after HSC transplantation may be caused by an aberrant immune reconstitution after both allogeneic and autologous HSC transplantation. " 01/01/2004 - "None of the patients showed paraproteinemia before HSC transplantation. "