A case of bilateral renal arterial thrombosis associated with cryocrystalglobulinaemia

Leung, Nelson; Buadi, Francis K.; Song, Kevin; Magil, Alexander B.; Cornell, Lynn D.

doi:10.1093/ndtplus/sfp140

Cited by 24 publications

(36 citation statements)

References 21 publications

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“…Terms, such as "MIDD with MGUS" or "glomerulonephritis with MGUS," have been used in the literature and diagnosis. [13][14][15] Unfortunately, MGUS in this context is misrepresented because in these patients there is significance to the monoclonal gammopathy, and its significance is not "undetermined." Despite their nonmalignant nature, these diseases are associated with a great deal of morbidity and even mortality.…”

Section: Introductionmentioning

confidence: 99%

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Leung

Bridoux

Hutchison

et al. 2012

Blood

464

359

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Multiple myeloma is the most frequent monoclonal gammopathy to involve the kidney; however, a growing number of kidney diseases associated with other monoclonal gammopathies are being recognized. Although many histopathologic patterns exist, they are all distinguished by the monoclonal immunoglobulin (or component) deposits. The hematologic disorder in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma. Unfortunately, due to the limitations of the current diagnostic schema, they are frequently diagnosed as MGUS. Because treatment is not recommended for MGUS, appropriate therapy is commonly withheld. In addition to endstage renal disease, the persistence of the monoclonal gammopathy is associated with high rates of recurrence after kidney transplantation. Preservation and restoration of kidney function are possible with successful treatment targeting the responsible clone. Achievement of hematologic complete response has been shown to prevent recurrence after kidney transplantation. There is a need for a term that properly conveys the pathologic nature of these diseases. 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. (Blood. 2012;120(22):4292-4295)

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Section: Introductionmentioning

confidence: 99%

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Leung

Bridoux

Hutchison

et al. 2012

Blood

464

359

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“…7,8 These two terms, however, have been used interchangeably in some prior publications. 5,7,9 Crystalglobulinemia has been described mainly in patients with MM 2,5,10 but may rarely occur in patients with monoclonal gammopathy of renal significance. 9 The biochemical basis for the phenomenon of crystallization of monoclonal protein is still unknown.…”

Section: Discussionmentioning

confidence: 99%

“…5,7,9 Crystalglobulinemia has been described mainly in patients with MM 2,5,10 but may rarely occur in patients with monoclonal gammopathy of renal significance. 9 The biochemical basis for the phenomenon of crystallization of monoclonal protein is still unknown. Anecdotal evidence suggests that it occurs due to Fc-Fc interactions of IgG-type monoclonal protein, 11 possibly owing to abnormal glycosylation of the light chain portion of monoclonal protein, 4 or through interactions with albumin ( Figure 2).…”

Section: Discussionmentioning

confidence: 99%

Crystalglobulin-Induced Nephropathy

Gupta

Ters

Kashani

et al. 2015

Journal of the American Society of Nephrology

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Crystalline nephropathy refers to renal parenchymal deposition of crystals leading to kidney damage. The most common forms of crystalline nephropathy encountered in renal pathology are nephrocalcinosis and oxalate nephropathy. Less frequent types include urate nephropathy, cystinosis, dihydroxyadeninuria, and druginduced crystalline nephropathy (e.g., caused by indinavir or triamterene). Monoclonal proteins can also deposit in the kidney as crystals and cause tissue damage. This occurs in conditions such as light chain proximal tubulopathy, crystal-storing histiocytosis, and crystalglobulinemia. The latter is a rare complication of multiple myeloma that results from crystallization of monoclonal proteins in the systemic vasculature, leading to vascular injury, thrombosis, and occlusion. In this report, we describe a case of crystalglobulin-induced nephropathy and discuss its pathophysiology and the differential diagnosis of paraprotein-induced crystalline nephropathy.

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“…The deposits are first categorized as organized versus nonorganized (25). The organized deposits are further classified as fibrils (AIg amyloidosis or monoclonal fibrillary GN), microtubules (immunotactoid GN or cryoglobulinemia), or crystals/inclusions (LCPT, crystal-storing histiocytosis, and cryocrystalglobulinemia) (26)(27)(28). Light-chain casts occasionally have a crystalline structure that fractures during processing of the tissue.…”

Section: The Role Of Monoclonal Proteins In Kidney Diseasesmentioning

confidence: 99%

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

Leung

Nasr

2016

CJASN

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Monoclonal gammopathy is increasingly recognized as a cause of kidney injury. These renal conditions behave differently than ones without monoclonal gammopathy and require specific treatment. To avoid misdiagnosis, testing for paraprotein should be performed in addition to vasculitis and autoimmune diseases serologies in adults with unexplained AKI or proteinuria. Because the prevalence of monoclonal gammopathy is much more common than glomerular diseases, the nephrotoxicity of the monoclonal protein must be confirmed before cytotoxic therapy is initiated. This can only be done by a kidney biopsy. After a monoclonal gammopathy of renal significant is verified, the evaluation should then focus on the identification of the pathologic clone, because therapy is clone specific. We present this patient to illustrate the clinical presentation of a patient with renal dysfunction and a monoclonal gammopathy. This patient is also used to discuss the diagnostic process in detail when monoclonal gammopathy-associated renal disease is suspected.

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A case of bilateral renal arterial thrombosis associated with cryocrystalglobulinaemia

Cited by 24 publications

References 21 publications

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Crystalglobulin-Induced Nephropathy

A Patient with Abnormal Kidney Function and a Monoclonal Light Chain in the Urine

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