Immunoglobulin Light (Heavy)-Chain Deposition Disease

Ronco, Pierre; Plaisier, Emmanuelle; Mougenot, B; Aucouturier, Pièrre

doi:10.2215/cjn.01730506

Cited by 112 publications

(108 citation statements)

References 48 publications

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“…In most reported cases of gHCDD, there was a deletion of the CH1 domain in the circulated or deposited g heavy chains (1,3,15), which is required for secretion of free heavy chains by plasma cells. The inability to detect a corresponding monoclonal heavy chain component in the serum in three of our patients may relate to its presence at very low titers, below the level of detection by our standard SIFE, or to rapid rates of tissue deposition (13). In addition, because many of the secreted heavy chains are truncated proteins, they may elude detection by standard SPEP/SIFE techniques.…”

Section: Discussionmentioning

confidence: 80%

Renal Monoclonal Immunoglobulin Deposition Disease

Nasr¹,

Valeri²,

Cornell³

et al. 2012

Clinical Journal of the American Society of Nephrology

254

214

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SummaryBackground and objectives To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series.Design, setting, participants, & measurements Characteristics of 64 MIDD patients who were seen at Mayo Clinic are provided.Results Of 64 patients with MIDD, 51 had light chain deposition disease, 7 had heavy chain deposition disease, and 6 had light and heavy chain deposition disease. The mean age at diagnosis was 56 years, and 23 patients (36%) were #50 years of age. Clinical evidence of dysproteinemia was present in 62 patients (97%), including multiple myeloma in 38 (59%). M-spike was detected on serum protein electrophoresis in 47 (73%). Serum free light chain ratio was abnormal in all 51 patients tested. Presentation included renal insufficiency, proteinuria, hematuria, and hypertension. Nodular mesangial sclerosis was seen in 39 patients (61%). During a median of 25 months of follow-up (range, 1-140) in 56 patients, 32 (57%) had stable/improved renal function, 2 (4%) had worsening renal function, and 22 (39%) progressed to ESRD. The mean renal and patient survivals were 64 and 90 months, respectively. The disease recurred in three of four patients who received a kidney transplant.Conclusions Patients with MIDD generally present at a younger age than those with light chain amyloidosis or light chain cast nephropathy. Serum free light chain ratio is abnormal in all MIDD patients, whereas only threequarters have abnormal serum protein electrophoresis. The prognosis for MIDD is improving compared with historical controls, likely reflecting earlier detection and improved therapies.

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Section: Discussionmentioning

confidence: 80%

Renal Monoclonal Immunoglobulin Deposition Disease

Nasr¹,

Valeri²,

Cornell³

et al. 2012

Clinical Journal of the American Society of Nephrology

254

214

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“…18,19 Furthermore, the variable region of the light chain probably determines the specific type of renal damage that a light chain can cause. Both lambda and kappa light chains are nephrotoxic, but lambda light chains are more frequently involved in the formation of amyloid than kappa, 20 and kappa are more frequently involved in other types of renal damage, such as LCDD 21 and acquired adult Fanconi's syndrome. 22 Cast formation is not the only pathophysiologic mechanism in myeloma kidney.…”

Section: Pathogenesismentioning

confidence: 99%

Pathogenesis and treatment of renal failure in multiple myeloma

et al. 2008

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Renal failure is a frequent complication in patients with multiple myeloma (MM) that causes significant morbidity. In the majority of cases, renal impairment is caused by the accumulation and precipitation of light chains, which form casts in the distal tubules, resulting in renal obstruction. In addition, myeloma light chains are also directly toxic on proximal renal tubules, further adding to renal dysfunction. Adequate hydration, correction of hypercalcemia and hyperuricemia and antimyeloma therapy should be initiated promptly. Recovery of renal function has been reported in a significant proportion of patients treated with conventional chemotherapy, especially when high-dose dexamethasone is also used. Severe renal impairment and large amount of proteinuria are associated with a lower probability of renal recovery. Novel agents, such as thalidomide, bortezomib and lenalidomide, have significant activity in pretreated and untreated MM patients. Although there is limited experience with thalidomide and lenalidomide in patients with renal failure, data suggest that bortezomib may be beneficial in this population. Clinical studies that have included newly diagnosed and refractory patients indicate that bortezomib-based regimens may result in rapid reversal of renal failure in up to 50% of patients and that full doses of bortezomib can be administered without additional toxicity.

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“…In LCDD, the pattern of glomerular immunofluorescence shows a marked heterogeneity. In contrast, tubular deposits of light chains are clearly visible and are seen in all cases [21]. We could easily have diagnosed this patient with LCDD at renal biopsy if light-chain staining had been performed, despite absent glomerular findings typical of LCDD on light microscopy.…”

Section: Discussionmentioning

confidence: 83%