A non-invasive differential diagnostic model for light chain cast nephropathy in newly diagnosed multiple myeloma patients with renal involvement: a multicenter study

Author(s):  
Zi-Shan Lin ◽  
Ai-Bo Qin ◽  
Su-Xia Wang ◽  
Xiao-Juan Yu ◽  
Bao Dong ◽  
...  
2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  

Abstract Background . Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods . We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results. In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 58 (29.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain were more frequent in patients with renal involvement (P < 0.05).9 patients with renal involvement were performed renal biopsy, and cast nephropathy, renal amyloidosis were proved in 5 and 4 patients, respectively. Conclusions. Renal involvement was common at MM diagnosis and had diverse clinical manifestations. The most common clinical manifestations include renal failure, isolated albuminuria and nephrotic syndrome. Nephrologists should rule out MM in patients presenting with renal involvement.


2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  

Abstract Background Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 53 (27.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain disease were more frequent in patients with renal involvement (P < 0.05). 9 patients with renal involvement were performed renal biopsy, and cast nephropathy and renal amyloidosis were proved in 5(55.6%) and 4(44.4%) patients, respectively. Conclusions Renal involvement was common at MM diagnosis and had diverse clinical manifestations. Nephrologists should rule out MM in patients presenting with renal involvement.


2021 ◽  
pp. jclinpath-2021-207449
Author(s):  
Zi-Shan Lin ◽  
Xu Zhang ◽  
Dan-Yang Li ◽  
Xiao-Juan Yu ◽  
Ai-Bo Qin ◽  
...  

AimsA varying proportion of patients with multiple myeloma suffer from more than one type of kidney disease simultaneously, of which the most common pattern is coexistent light chain cast nephropathy and light chain deposition disease (LCCN+LCDD). We investigated clinicopathological characteristics and outcomes of LCCN+LCDD in comparison with pure LCCN and pure LCDD.MethodsWe retrospectively analysed 45 newly diagnosed multiple myeloma patients with pure LCCN (n=26), LCCN +LCDD (n=9) and pure LCDD (n=10) between 2000 and 2019 at Peking University First Hospital.ResultsPathologically, patients with LCCN+LCDD were more likely to have λ light chain isotype and presented atypical features of LCDD including less nodular glomerulosclerosis and less deposit distribution than patients with pure LCDD. In clinical characteristics, patients with LCCN +LCDD and patients with pure LCCN shared similar features. The death-censored renal survival in patients with LCCN +LCDD was similar to patients with pure LCCN but worse than patients with pure LCDD, but the overall survival was much better than patients with LCCN alone and similar to patients with pure LCDD. For patients with pure LCCN, the independent predictor of death-censored renal survival was lactate dehydrogenase, and the independent predictors of overall survival were the mean number of casts and serum albumin.ConclusionsPatients with LCCN+LCDD had similar renal outcome compared with patients with pure LCCN but the overall survival is much better. Thus, for patients with LCCN, especially those with λ restriction, pathologists should carefully evaluate the kidney specimens to exclude the possibility of combined LCDD.


2010 ◽  
Vol 28 (33) ◽  
pp. 4976-4984 ◽  
Author(s):  
Meletios A. Dimopoulos ◽  
Evangelos Terpos ◽  
Asher Chanan-Khan ◽  
Nelson Leung ◽  
Heinz Ludwig ◽  
...  

Renal impairment is a common complication of multiple myeloma (MM). The estimated glomerular filtration rate (eGFR) using the Modification of Diet in Renal Disease formula is the recommended method for the assessment of renal function in patients with MM with stabilized serum creatinine. In acute renal injury, the RIFLE (risk, injury, failure, loss and end-stage kidney disease) and Acute Renal Injury Network criteria seem to be appropriate to define the severity of renal impairment. Novel criteria based on eGFR measurements are recommended for the definition of the reversibility of renal impairment. Rapid intervention to reverse renal dysfunction is critical for the management of these patients, especially for those with light chain cast nephropathy. Bortezomib with high-dose dexamethasone is considered as the treatment of choice for such patients. There is limited experience with thalidomide in patients with myeloma with renal impairment. Thus, thalidomide can be carefully administered, mainly in the context of well-designed clinical trials, to evaluate if it can improve the rapidity and probability of response that is produced by the combination with bortezomib and high-dose dexamethasone. Lenalidomide is effective in this setting and can reverse renal insufficiency in a significant subset of patients, when it is given at reduced doses, according to renal function. The role of plasma exchange in patients with suspected light chain cast nephropathy and renal impairment is controversial. High-dose melphalan (140 mg/m2) and autologous stem-cell transplantation should be limited to younger patients with chemosensitive disease.


2019 ◽  
Vol 3 (5) ◽  
pp. 744-750 ◽  
Author(s):  
Nidhi Tandon ◽  
Surbhi Sidana ◽  
S. Vincent Rajkumar ◽  
Morie A. Gertz ◽  
Francis K. Buadi ◽  
...  

Abstract We evaluated the impact of achieving a rapid response in 840 newly diagnosed multiple myeloma patients from 2004 to 2015. Rates of very good partial response (VGPR) or better were 29% (240/840) after 2 cycles of treatment, 42% (350/840) after 4 cycles of treatment, and 66% (552/840) as best response. Early responders after 2 cycles of treatment had higher rates of light chain disease, anemia, renal failure, International Staging System (ISS) stage III disease, and high-risk cytogenetics, especially t(4;14), and were more likely to have received triplet therapy and undergo transplant. Median progression-free survival (PFS) and overall survival (OS) were not different among patients with ≥VGPR and &lt;VGPR after 2 cycles (PFS, 28 vs 30 months, P = .6; OS, 78 vs 96 months, P = .1) and 4 cycles (PFS, 31 vs 29 months; OS, 89 vs 91 months, P = .9), although both were improved, with ≥VGPR as best response (PFS, 33 vs 22 months, P &lt; .001; OS, 102 vs 77 months, P = .003). On multivariate analysis stratified by transplant status, achievement of ≥VGPR after 2 cycles was not associated with improved PFS (hazard ratio [95% confidence interval]; transplant cohort, 1.1 [0.7-1.6]; nontransplant cohort, 1.2 [0.8-1.7]) or OS (transplant cohort, 1.6 [0.9-2.9]; nontransplant cohort, 1.5 [1.0-2.4]). Covariates in the model included high-risk cytogenetics, ISS stage III, triplet therapy, creatinine ≥2 mg/dL, light chain disease, and age. Although patients with high-risk disease are more likely to achieve early response, a rapid achievement of a deep response by itself does not affect long-term outcomes.


2008 ◽  
Vol 2 (1) ◽  
Author(s):  
Kolitha Basnayake ◽  
Colin Hutchison ◽  
Dia Kamel ◽  
Michael Sheaff ◽  
Neil Ashman ◽  
...  

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 5036-5036 ◽  
Author(s):  
Beihui Huang ◽  
Juan Li ◽  
Junru Liu ◽  
Dong Zheng ◽  
Mei Chen ◽  
...  

Abstract Abstract 5036 Objective: To assess the efficacy and tolerability of bortezomib with dexamethasone for patients with primary systemic light chain (AL) amyloidosis or multiple myeloma-associated AL amyloidosis. Methods: Twelve newly diagnosed patients with primary systemic AL amyloidosis and six patient with multiple myeloma-associated AL amyloidosis were treated with a combination of bortezomib (1. 3 mg/m2 d1, 4, 8, 11) and dexamethasone (20 mg d1–4). Results: Sixteen patients was evaluable. 12/16 had a hematologic response and 6/16 (37. 5%) a hematologic complete response. Median cycles to response was 1 cycle and median cycles to best response was 2 cycles. In patients with primary AL amyloidosis, 8/10 (80. 0%) had a hematologic response and 5/10 (50. 0%) a hematologic complete response. In patients with myeloma-associated AL amyloidosis, 7/10 (70. 0%) had a hematologic response and 1/6 (16. 7%) a hematologic complete response. Twelve patients (75. 0%) had a response in at least one affected organ, in which 7 in patients with primary AL amyloidosis and 5 in myeloma-associated AL amyloidosis. Person correlation between hematologic response and organ response was 0. 667 (p=0. 005). Fatigue, diarrhea and infection were the most frequent side effects. Three patients developed herpes zoster and had to stop chemotherapy. Conclusions: VD produces rapid and high hematological responses in the majority of patients with newly diagnosed AL regardless of primary or associated with myeloma. It is well tolerated with few side effects. This treatment may be a valid option as first-line treatment for newly diagnosed patients with primary systemic AL amyloidosis and multiple myeloma-associated AL amyloidosis. Disclosures: No relevant conflicts of interest to declare.


Sign in / Sign up

Export Citation Format

Share Document