Pdf monoclonal gammopathy of renal significance mgrs. We report a case of monoclonal gammopathy of renal significance in a 63yearold man who presented with nephroticrange proteinuria and renal insufficiency. Monoclonal gammopathy of undetermined significance mgus dx criteria serum m protein monoclonal gammopathy of undetermined significance mgus facts normal plasma cells a type of white blood cell produce antibodies also known as immunoglobulins which help fight infection. This likely reflects the high frequency of monoclonal gammopathy of undetermined significance in older patients and the frequent use of serum andor urine protein electrophoresis as. Clinical features the results of the study performed in mayo showed that more than half of the patients with monoclonal gammopathy had renal lesions that were directly relat. Monoclonal gammopathy of undetermined significance does not have end organ damage, but a proportion of cases. Jun 01, 2016 laboratory testing in monoclonal gammopathy of renal significance mgrs. His past medical history was positive for htn, dm, and hepatitis c successfully treated with ombitasvirperiteprevirdasabuvir for three months. Development of renal failure without proteinuria in a patient. Neph gr monoclonal gammopathy of renal significance what. What happens with we find renal pathology findings and they confirm a monoclonal strain of b cell clone. The majority of patients with serum andor urine monoclonal gammopathy who undergo renal biopsy have disease unrelated to monoclonal gammopathy deposition.
But few beacon readers are likely to know what mgrs is. Hutchison4, guy touchard1, sanjeev sethi5, jeanpaul fermand6, maria m. It is because of this necessity that we propose the term monoclonal gammopathy of renal significance mgrs to discriminate the pathologic nature of these diseases from the truly benign mgus. Monoclonal gammopathy of undetermined significance mgus definition. The prevalence of monoclonal gammopathy during lupus is 2. A 78yearold man with a 9year history of igg kappa lightchainmgus and normal urine protein excretion developed severe renal failure. It is accepted that mgus should not be treated until progression to multiple myeloma or another lymphoid malignancy.
Testing and management for monoclonal gammopathy of uncertain. The kidney biopsy showed a membranoproliferative glomerulonephritis pattern with extensive crystalloid deposits in the glomerular capillary endothelial cells and very few in the tubular epithelial cells. Now, most beacon readers probably have heard of monoclonal gammopathy of undetermined significance mgus, an early stage of multiple myeloma that remains benign in most people diagnosed with it. Renal lesions in monoclonal gammopathy can be grouped as follows. Monitoring of monoclonal gammopathy of undetermined. Diagnosis of monoclonal gammopathy of renal significance frank bridoux1, nelson leung2,3, colin a. By definition, these disorders do not meet diagnostic criteria for overt, symptomatic multiple myeloma or a lymphoproliferative disorder. A 58yearold man with no significant medical history is found to have an elevated total protein concentration 8.
Mgrs, mgus, monoclonal gammopathy of renal significance and monoclonal gammopathy of undetermined. The focus of the article is on the different types of laboratory tests that are involved in. This condition is now categorized as monoclonal gammopathy of renal significance mgrs. With the exception of immunoglobulin light chain al amyloidosis, few studies have focused on therapeutic issues. Diagnosis of monoclonal gammopathy of renal significance kidney. In their longterm followup study of patients with monoclonal gammopathy of undetermined significance mgus, kyle et al. Heavy proteinuria is one of its cardinal manifestations. The prevalence of decreased glomerular filtration rate in. These monoclonal proteins can damage tissues and organs, particularly the kidneys. The era has begun posted on june 25, 20 by ajkdblog in commentary 2 comments in an article recently published in the american journal of kidney diseases, zand et al discuss a series of patients with c3 glomerulopathy and monoclonal gammopathy.
Nov 16, 2017 leung n, bridoux f, hutchison ca, nasr sh, cockwell p, fermand jp, dispenzieri a, song kw, kyle ra, on behalf of the international kidney and monoclonal gammopathy research group 2012 monoclonal gammopathy of renal significance. Mgus premyeloma and kidney damage peoplebeatingcancer. Monoclonal gammopathy of renal significance presenting as. Screening for monoclonal immunoglobulin and an appropriate hematologic workup are fundamental and, sometimes a difficult challenge. Monoclonal gammopathy of renal significance triggered by viral e hepatitis. It is also characterized by lack of lytic bone lesions, anemia, hypercalcemia, and renal insufficiency related to the plasma cell proliferative process. Monoclonal gammopathy of renal significance mgrs clinic monoclonal proteins are immunoglobulins made by clonal antibodyproducing cells. The term monoclonal gammopathy of renal significance mgrs was introduced to distinguish monoclonal gammopathies that result in the. Recently, monoclonal gammopathy of renal significance mgrs reclassified all monoclonal m gammopathies that are associated with the development of a kidney disease but do not meet the definition of symptomatic multiple myeloma mm.
Clinical work up and treatment of monoclonal gammopathy of. The term monoclonal gammopathy of renal significance mgrs is a recent concept, introduced in 2012, to distinguish the nephropathic nature of these diseases from the truly benign monoclonal gammopathy of undetermined significance. The term monoclonal gammopathy of renal significance mgrs comprises a group of diseases pathogenetically characterised by proliferation of a bcell or plasma cell clone that synthesises and secretes a monoclonal immunoglobulin or its components light andor heavy chains, that may deposit and cause glomerular, tubular, interstitial andor vascular damage. Monoclonal gammopathy of renal significance mgrs is a new nosological group of entities metaentity defined in 2012, whose pathogenesis depends on monoclonal immunoglobulins ig secreted by lowgrade lymphoproliferative disorders, which belong to mproteinrelated diseases. By definition, patients with mgrs have bcell clones that do not meet the definition of multiple myeloma or lymphoma. Mgus is defined by a monoclonal immunoglobulin mprotein or paraprotein in the serum of up to 30gl in the absence of lytic bone lesions, anaemia, hypercalcaemia and renal insufficiency that is related to the underlying monoclonal plasma cell proliferation. However, monoclonal gammopathy of unknown significance mgus or smoldering. A 75yearold man with chronic renal disease of unknown origin since 2010 experienced rapid worsening of renal.
Laboratory testing in monoclonal gammopathy of renal. Diagnosis of monoclonal gammopathy of renal significance. Pdf multiple myeloma mm is the most frequent monoclonal gammopathy to involve the kidney. Monoclonal gammopathy monoclonal gammopathy of renal significance mgrs represents a group of disorders in which a monoclonal immunoglobulin secreted by a nonmalignant or premalignant b cell or plasma cell clone causes renal damage. As a result, the term monoclonal gammopathy of renal significance mgrs was coined in 2012 to. Al amyloidosis complicating monoclonal gammopathy of undetermined significance mgus has usually a predominant glomerular deposition of lambda light chain. Morbidity associated with monoclonal gammopathy of renal significance is high due to the severe renal lesions and the associated systemic alterations.
By definition, patients with mgrs do not meet the criteria for overt multiple myelomabcell proliferation, and the hematologic disorder is generally consistent with monoclonal. Hospital medicine 2016, march 69, san diego, calif. By definition, patients with mgrs do not meet the criteria for overt multiple. Monoclonal gammopathy of renal significance mgrs is defined by the causal relationship between a small bcell clone and renal disease, usually through deposition of the secreted monoclonal immunoglobulin mig or a fragment thereof. Among 55 patients with monoclonal gammopathy and unrelated renal disease 63. In this setting, structural peculiarities of the mig, particularly the variable domain, and not the rate of production, are the main determinants of renal lesions.
Monoclonal gammopathy of renal significance is a wide group of kidney diseases. Patients with mgus appear to have a higher risk of developing myeloid malignancies such as myelodysplastic syndromes mds and. A new term now referred to mgus disorders with renal biopsy findings as mgrs monoclonal gammopathy of renal significance. Monoclonal gammopathy of renal significance mgrs is a recently conceived description of kidney injury resulting from a nonmalignant plasma cellb cell clone that, by definition, does not fulfill criteria for multiple myeloma or lymphoma. Diagnosis and treatment of monoclonal gammopathy of renal. Monoclonal gammopathy of renal significance mgrs represents a group of disorders in which a monoclonal immunoglobulin secreted by a nonmalignant or premalignant b cell or plasma cell clone causes renal damage. Monoclonal gammopathy of undetermined significance. We discuss the most common diagnostic difficulties and suggest an algorithm for clinical approach. Integrate recent developments in nephrology and hypertension to their current practice.
Moreover, monoclonal gammopathy was discovered in the phase of remission of lupus in our patient. C3 glomerulonephritis associated with monoclonal gammopathy. Monoclonal gammopathy of renal significance what does the future hold. Paraproteinrelated kidney disease represents a complex group of diseases caused by an abnormal paraprotein secreted by a clone of b cells. Evidence already supports monoclonal protein as the direct cause of the kidney disease and not the tumor. By definition, patients with mgrs do not meet the criteria for overt multiple myelomabcell proliferation, and the hematologic disorder is generally consistent with. Atc2010 nejm banff2011 liver transplantation medical student other blogs podocytes pre eclampsia pseudoelectrolytes. Neph gr monoclonal gammopathy of renal significance. By definition, patients with mgrs do not meet the criteria for overt multiple myeloma bcell proliferation, and the hematologic disorder is generally consistent with monoclonal. Monoclonal gammopathy of renal significance mgrs regroups all renal disorders caused by a monoclonal immunoglobulin mig secreted by a nonmalignant bcell clone. It is an article about monoclonal gammopathy of renal significance mgrs.
Pdf diagnosis of monoclonal gammopathy of renal significance. Monoclonal gammopathy of undetermined significance mgus dx criteria serum m protein mgrs clinic. Herrera8, efstathios kastritis9, giampaolo merlini10, murielle roussel11, fernando c. Clinical work up and treatment of monoclonal gammopathy. A 75yearold man with chronic renal disease of unknown origin since 2010 experienced rapid worsening. Each type of plasma cell produces only one type of immunoglobulin ig, the same immunoglobulin protein as the original abnormal cell. Laboratory testing in monoclonal gammopathy of renal significance mgrs. Bortezomib maintenance for the treatment of monoclonal. Explain rationale for drug treatment choices based on advances in clinical trials.
Testing and management for monoclonal gammopathy of. Monoclonal gammopathy of unknown significance mgus is a benign condition that carries a risk of progression to haematological malignancy. The term monoclonal gammopathy of renal significance mgrs comprises a group of diseases pathogenetically characterised by proliferation of a bcell or. How i treat monoclonal gammopathy of renal significance. Development of renal failure without proteinuria in a. Pdf on mar 1, 2018, gabriela spacek da fonseca and others published monoclonal gammopathy of renal significance. There is a need for a term that properly conveys the pathologic nature of these diseases. Nephrotic syndrome, renal insufficiency, and a monoclonal. Monoclonal gammopathies of renal significance revista nefrologia. Monoclonal gammopathy of renal significance triggered by viral e. By definition, patients with mgrs do not meet the criteria for overt multiple myelomabcell proliferation, and the hematologic disorder is generally consistent with monoclonal gammopathy of undetermined significance mgus. The leading differential diagnostic consideration was.
Crystalloid glomerulopathy in monoclonal gammopathy of. Monoclonal gammopathy of renal significance arkana. A new term, monoclonal gammopathy of renal significance mgrs was proposed by leung and colleagues to describe a group of haematological disorders associated with kidney disease that fail to meet the standard definitions for mm or lymphoma. Monoclonal gammopathy of renal significance was proposed by the international kidney and monoclonal gammopathy research group for renal lesions in monoclonal gammopathy in recent years. Monoclonal gammopathy of renal significance wikilite. Crystalloid glomerulopathy in monoclonal gammopathy of renal. The aim of this study is to determine the prevalence of chronic renal failure crf, defined as aglomerular filtration rate gfr below 1. Monoclonal gammopathy with renal significance sysakiewicz. Multiple myeloma, the second most frequent blood cancer, and its precursor, monoclonal gammopathy of uncertain significance, are associated with an increased risk of fragility fractures. Asma khatoon, md and roberto collazomaldonado, md, methodist dallas medical center, dallas, tx.
The disease manifestations range from tubulopathies, such as the fanconi syndrome, to a spectrum of glomerular diseases that can present with varying degrees of proteinuria and renal dysfunction. Making sense of serum protein bands best tests july 2011. Nasr5 on behalf of the international kidney and monoclonal. However, current guidelines fail to offer explicit indications for healthcare professionals in terms of testing and thresholds for onward referral. In such cases, the renal impairment is often linked to the underlying haematological disorder. Monoclonal gammopathy of renal significance mgrs is an entity that was defined in 2012 to describe a spectrum of renal diseases resulting from the nephrotoxic effects of paraproteinemia from nonmalignant clonal b cells or plasma cells, and by definition does not meet criteria for multiple myeloma, chronic lymphocytic leukemia, or malignant lymphoma. Monoclonal gammopathy of renal significance mgrs can present with myriad of morphological features. It applies to cases that do not meet the diagnostic criteria for multiple myeloma and the renal damage secondary to m protein. The term monoclonal gammopathy of renal significance mgrs comprises a group of diseases pathogenetically characterised by proliferation of a bcell or plasma cell clone that synthesises and.
Monoclonal gammopathy of renal significance shm abstracts. Monoclonal gammopathy of undetermined significance nejm. Collazomaldonado, md, facp a 60year old african american man was referred for evaluation of nephrotic syndrome. After these negative studies, renal biopsy was performed to investigate further the cause of the nephrotic syndrome, hematuria, and renal insuf. Monoclonal gammopathy of renal significance mgrs clinic. Accordingly, early diagnosis is fundamental, as is stopping the clonal production of immunoglobulins using specific chemotherapy. Monoclonal gammopathy of renal significance mgrs regroups all renal disorders caused by a monoclonal immunoglobulin mig secreted by. N2 recently, the term monoclonal gammopathy of renal significance mgrs was introduced to distinguish monoclonal gammopathies that result in the development of kidney disease from those that are benign. Monoclonal gammopathy of renal significance can be responsible for the tubular defect present in fanconi syndrome 14.
Pdf diagnosis of monoclonal gammopathy of renal significance efstathios kastritis academia. The list of new myelomarelated research that is at the bottom of every edition of myeloma morning has only one entry today. The nonmalignant nature of these clones has been a source of confusion for clinicians when it comes to treatment. We report a case of membranoproliferative glomerulonephritis caused by monoclonal gammopathy. Recently, growing evidence has started to show that even small monoclonal clones can be responsible for renal impairment. How i treat monoclonal gammopathy of renal significance mgrs. We think the term monoclonal gammopathy of renal significance is most helpful to indicate a causal relationship between the monoclonal gammopathy and the renal damage and because the significance of the monoclonal gammopathy is no longer undetermined. Apr 25, 2016 the topic of that article is monoclonal gammopathy of renal significance mgrs. The scope of kidney affection in monoclonal gammopathies. Is that now mgus really undetermined or insignificant.
19 1258 505 1608 971 672 2 161 1302 208 537 641 522 1593 190 128 988 763 1212 320 189 611 243 11 799 599 856 1320 426 800 1091 181 1179 498 881 279 1141 673 119 1287 1032 388 340 234 1312 798 984