Monoclonal Gammapathies Of Renal Significance

Various hematologic malignancies can lead to renal complications. The most common of these hemopathies to affect the kidney is multiple myeloma, however an increasing number of kidney diseases are associated with other monoclonal gammopathies. It is recognized that clones in small abundance can be responsible for severe organ damage, thus the concept of monoclonal gammopathy of renal significance (MGRS) has emerged. Although the hemopathy in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma, the diagnosis of a renal complication changes the therapeutic management...

In this article we discussed the current state of monoclonal gammapathy of renal significance (Monoclonal Gammopathy of Renal Significance MGRS) and revealed problems of B-cell clone secreting nephrotoxic monoclonal immunoglobulin identification. We followed 276 patients with monoclonal gammapathy including patients with non-amyloid nephropathy. The majority of patients had systemic AL-amyloidosis. We established better survival of the treated patients with systemic AL-amyloidosis in comparison with retrospective untreated cohort...

High serum levels of vitamin B12 or cobalamin, also called hypervitaminemia B12, is a frequently underestimated biological abnormality. According to the literature, some of the entities related to this finding are solid neoplasia (primary or metastatic) and acute or chronic hematological diseases. Other causes include liver disorders, monoclonal gammapathy of undetermined significance, renal failure and, less frequently, excess of vitamin B12 intake, inflammatory or autoimmune diseases, and transient hematological disorders (neutrophilia and secondary eosinophilia)...

Liver disease is the major complication of chronic HCV infection. However, extrahepatic complications are common (50-75%), including mixed cryoglobulinaemia and B-cell lymphomas. Given that chronic hepatitis C has become curable using expensive oral direct-acting antivirals (DAAs), it seems worth revisiting the whole spectrum and burden of disease in HCV carriers.Herein, we update the most clinically significant medical complications associated with chronic hepatitis C and the evidence of benefits that would derive from a wide use of curative DAA therapies...

Proteinuria is an expected complication in transplant patients treated with mammalian target of rapamycin inhibitors (mTOR-i). However, clinical suspicion should always be supported by histological evidence in order to investigate potential alternate diagnoses such as acute or chronic rejection, interstitial fibrosis and tubular atrophy, or recurrent or de novo glomerulopathy. In this case we report the unexpected diagnosis of amyloidosis in a renal-transplant patient with pre-transplant monoclonal gammapathy of undetermined significance who developed proteinuria after conversion from tacrolimus to everolimus...

INTRODUCTION: Cryoglobulins are immunoglobulins that persist in the serum, precipitate at the cold temperature and resolubilise when rewarmed. There are three categories, the type I, II and III, with neurological symptoms. Those are dominated by peripheral neuropathy, the central nervous system is generally saved. We reported the case of a cryoglobulinemia of the type I having generated a ischemic cerebrovascular accident (CVA), a seldom described situation. CASE REPORT: A 62 years old man, without noteworthy past...

This work aims to define the interest and the limits of free light chain (FLC) determination in serum and urine for the investigation of monoclonal gammopathies. Based on the study of nine typical cases extracted from laboratory practice, the authors demonstrate the interest of this determination for the diagnosis and the monitoring of FLC and non secretory myelomas. This test is also useful for the evaluation of response to chemotherapy and the early detection of relapses in intact immunoglobulin multiple myelomas...

From July 1992 to December 2000, 288 cases of monoclonal gammapathy (MG) were collected at the university hospital of Sfax. The middle age of the patients at the time of the diagnosis was 62 years and 7 months with extremes to 18 months and 99 years and median to 64 years. One hundred and eighty-two patients were men and 106 women. Among the 270 observations for which aetiology has been established, 73 were classified MG of undetermined significance (MGUS), 160 myeloma (or plasmocytoma) and 37 other malignant MG (Waldenstrom's macroglobulinemia: 13, lymphoma: 9, alpha heavy chains disease: 6, primary amyloidosis: 5, chronic lymphocytic leukaemia: 4)...

BACKGROUND: Retrospective study about results of treatment of patients (pts) with primary (AL) and secondary (AA) amyloidosis is given. 31 pts with systemic forms of amyloidosis have been treated and followed-up in our department since 1993. METHODS AND RESULTS: 6 men and 11 women were in the AL group with the mean age of 59 years. Multiple myeloma was diagnosed in 9 pts, monoclonal gammapathy of undetermined significance (MGUS) was found in 8 pts. The kidneys were affected in all pts, heart in 59% of pts, liver, joints and skin in 26% of pts and polyneuropathy was detected only in 1 pt...

BACKGROUND: The clinical relevance of distinguishing two types of glomerulonephritis (GN) with non-amyloid organized immunoglobulin (Ig) deposits-fibrillary GN (FGN) and immunotactoid (microtubular) GN (IT/MTGN)-on the basis of ultrastructural organization, is debated. METHODS: Twenty-three patients with organized glomerular Ig deposits were classified into two groups based on the fibrillar or microtubular ultrastructural appearance of the deposits. Kidney biopsy samples were studied by immunofluorescence microscopy, using anti-light chain conjugates (all cases) and anti-IgG subclass conjugates (13 patients)...

A permanent and quantitatively abnormal proteinuria (> 150 mg/ 24 h) found at the laboratory should be followed by an identification and quantification of all proteins present. Specific immunochemistry techniques (immunoturbidimetric or immuno-nephelometric) are almost exclusively used for measuring albumin (or microalbuminuria) in nephropathic diabetic patients. Measurement of monoclonal free light chains is not recommended due to poor accuracy. Determination of other small proteins (molecular mass, MM < 40 kDa), transferrine, immunoglobulins G (assessment of selectivity) is far less frequent and Tamm-Horsfall protein is measured in specialized laboratories...

BACKGROUND: Multiple myeloma (MM) is mostly fatal neoplastic disorder, however, the median survival of 30-40 months does not adequately express the wide spectrum courses of the disease. Therefore better prognostic factors are needed. METHODS AND RESULTS: We analyzed the value of both standard clinical features and cytokines for differential diagnosis, assessment of myeloma activity, prediction of overall survival and duration of remission in a group of 124 patients with MM and in 156 with various monoclonal gammapathies, respectively...

Patients with a monoclonal gammapathy of undetermined significance (MGUS) are usually submitted to a periodical clinical follow-up, but it is not known if this surveillance can ameliorate the prognosis of a plasma cell malignancy that will be eventually detected. We compared the clinical and laboratory characteristics at onset, the response to chemotherapy and the survival, of 21 cases of newly diagnosed multiple myeloma (MM) arising from the malignant transformation of MGUS and 41 cases without a previous history of MGUS, recruited to the same first-line treatments over a 3-years period...

BACKGROUND: Bence-Jones nephropathy, the most serious form of which is renal failure, is one of the frequent complications in multiple myeloma (MM). Precise pathogenetic mechanism of renal injury remains unclear. Experimental study points to the possible role of some cytokines in the development of this type of nephropathy. We have investigated the levels of interleukin-6 (IL-6), tumour nekrosis factor alpha (TNF-alpha) and their soluble receptors in patients with plasmocytoma. METHODS AND RESULTS: The group comprised 49 patients with plasmocytoma, mostly with MM...

We refer in the present article, the first case found in our laboratory of Monoclonal gammapathy of the IgD type. A 47-year-old man presented at the emergency department with a history of malaise, lethargy, tiredness, thirstiness and obscure depositions. Clinical examination revealed a normocytic anaemia. The plasma urea was 423 mg/dl and the plasma creatinine was 15, 3 mg/dl. He was admitted to hospital with a diagnosis of acute renal failure. The later electrophoresis in serum revealed a little monoclonal band that was identified as IgD-lambda type by immunofixation electrophoresis...

Data from the french national registry show that about 20% of plasma exchange are performed to alleviate clinical manifestations related to a monoclonal Ig. Plasma removal has to be only considered as a part of therapy, the disease being controlled by cytotoxic drugs. However, owing to its delayed effect plasma exchange are useful to preserve the functional or vital prognosis. Technical aspects such as plasma volume removed or frequency of procedures will be scheduled according to monoclonal Ig specification: repartition between intra and extravascularly pools-allotypes-efficiency of chemotherapy...

Sera from 192 consecutive HIV negative renal transplant patients with more than 6 months follow-up were investigated for monoclonal or oligoclonal immunoglobulins (mIg) by immunoelectrophoresis or immunofixation. Gammapathy was present in 25 patients (13 percent). Eleven patients had only one monoclonal band, whereas 14 had two or more bands. Sixty percent were IgG K, 29 percent IgG lambda and 11 percent IgM lambda or K. Ninety percent of these mIg did not exceed 2 g/l; mIg appeared within 2-27 months following the transplantation (mean time-lag 8 +/- 6...

145 Determinations of beta2-microglobulin (beta2m) have been realized in 111 patients with a monoclonal gammapathy. The level of beta2m is significantly higher (p = 0,0001) in the group with monoclonal gammapathy (M = 4,75 mg/l +/- 5,62) than in normal subjects (M 1,38 mg/l +/- 0,38). In a group with monoclonal gammapathy with renal failure, the level of beta2m is higher (M = 7,07 mg/l +/- 7,76) than in a group with renal failure alone (M = 3,45 mg/l +/- 1,91). The difference is significant (p = 0,005). The level of beta2m is higher in IgA monoclonal gammapathies (M = 5,61 mg/l) than in IgG (M = 4,18 mg/l) or IgM (M = 4,37 mg/l) gammapathies, but the differences of the means between the three groups are not significant...