Clinicopathological features and outcomes of coexistent light chain cast nephropathy and light chain deposition disease in patients with newly diagnosed multiple myeloma

2021 ◽  
pp. jclinpath-2021-207449
Author(s):  
Zi-Shan Lin ◽  
Xu Zhang ◽  
Dan-Yang Li ◽  
Xiao-Juan Yu ◽  
Ai-Bo Qin ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Overall Survival ◽  
Light Chain ◽  
Renal Outcome ◽  
Newly Diagnosed ◽  
Renal Survival ◽  
Light Chain Deposition Disease ◽  
Cast Nephropathy ◽  
Deposition Disease ◽  
Light Chain Deposition

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.

scholarly journals P0291MYELOMA CAST NEPHROPATHY, LIGHT CHAIN DEPOSITION DISEASE, AND BOTH IN PATIENTS WITH NEWLY DIAGNOSED MULTIPLE MYELOMA

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Zishan Lin ◽  
Xiaojuan Yu ◽  
Suxia Wang ◽  
Fude Zhou ◽  
Minghui Zhao
Keyword(s):  
Multiple Myeloma ◽  
Overall Survival ◽  
Clinical Characteristics ◽  
Newly Diagnosed ◽  
Renal Survival ◽  
Light Chain Deposition Disease ◽  
Cast Nephropathy ◽  
Significant Difference ◽  
Peking University ◽  
Light Chain Deposition

Abstract Background and Aims The pathological patterns of myeloma-related kidney disease are various, in which myeloma cast nephropathy (MCN) and light chain deposition disease (LCDD) are two of the main patterns. Of note, in patients with multiple myeloma (MM), these two patterns of pathology can occur at the same time. However, there are limited data available showing the difference of clinical characteristics and outcomes among newly diagnosed MM patients with these three patterns of pathology. In this study, we retrospectively reviewed 42 newly diagnosed MM patients with renal biopsy-proven pure MCN, pure LCDD or MCN with coexistent LCDD in Peking University First Hospital, to describe the clinical characteristics, treatment, and outcomes, in order to help define the spectrum of prognostic profiles. Method All the native renal biopsies between September 1999 and October 2019 at Peking University First Hospital were retrospectively restudied. A total of 42 newly diagnosed MM patients with pure MCN (n=24), pure LCDD (n=8) and MCN with coexistent LCDD (n=10) were enrolled. The clinical features, as well as prognostic risk factors for renal survival and overall survival were retrospectively investigated. Results The 42 parents consisted of 26 men and 16 women with a mean age of 53.2±10.9 years. There was no significant difference in age and gender among the 3 groups. Patients with pure LCDD had significantly higher hemoglobin levels, a higher percentage of albumin in urine protein, a lower incidence of progressing to ESRD and more likely to present with AKD than patients with pure MCN or with MCN+LCDD. Patients with pure LCDD had significantly higher serum creatinine levels, a lower eGFR, a lower incidence of death and more likely to present with nephrotic syndrome than patients with pure MCN, but no different than patients with MCN+LCDD. No significant difference between pure MCN and MCN+LCDD. Median renal survival in LCDD group (not reached) was significantly higher compared to MCN (11 months, p = 0.002) group and MCN+LCDD group (1months, p = 0.012, Figure 1). Median overall survival in LCDD group (not reached) was significantly higher compared to MCN (39 months, p = 0.014) group, but had no difference with MCN+LCDD group (not reached, p = 0.199, Figure 2). According to the multivariate analysis, the independent predictors of renal survival were hemoglobin (HR: 0.957, 95% CI: 0.922-0.994; p = 0.022) and eGFR (HR: 0.940, 95% CI: 0.880-0.995; p = 0.034), the independent predictors of overall survival were hemoglobin (HR: 0.943, 95% CI: 0.895-0.993; p = 0.025) and pure MCN (HR: 7.597, 95% CI: 1.171-49.272; p = 0.034). Conclusion Patients with MCN+LCDD presented similar in clinical characteristics to patients with pure MCN and both suffer from more serious and more urgent renal damage than patients with pure LCDD. However, the overall survival in patients with MCN+LCDD was similar to patients with pure LCDD while the renal survival was worse. Hemoglobin and eGFR were independent predictors of renal survival and hemoglobin and pure MCN were independent predictors of overall survival.

Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review

2016 ◽  
Vol 25 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Benoit Brilland ◽  
Johnny Sayegh ◽  
Anne Croue ◽  
Frank Bridoux ◽  
Jean-François Subra ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Relevant Literature ◽  
Gamma Glutamyl Transferase ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Uncommon Presentation ◽  
Light Chain Deposition ◽  
Glutamyl Transferase

Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition. Intrahepatic cholestasis was attributed to LCDD on the basis of the liver histology and exclusion of possible diagnoses. Chemotherapy was maintained and resulted in progressive resolution of cholestasis. We report here an uncommon presentation of LCDD, with prominent liver involvement that fully recovered with bortezomib-based chemotherapy, and briefly review the relevant literature. Abbreviations: AKI: Acute kidney injury; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; CMV: Cytomegalovirus; EBV: Epstein–Barr virus; GGT: gamma-glutamyl transferase; HSV: Herpes simplex virus; LC: light chain; LCDD: Light chain deposition disease; MIDD: Monoclonal immunoglobulin deposition disease; MM: Multiple myeloma.

scholarly journals Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

2017 ◽  
Vol 92 (8) ◽  
pp. 739-745 ◽  
Author(s):  
Meera Mohan ◽  
Amy Buros ◽  
Pankaj Mathur ◽  
Neriman Gokden ◽  
Manisha Singh ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Prognostic Factors ◽  
Light Chain ◽  
Clinical Characteristics ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

scholarly journals Liver Failure Caused by Light Chain Deposition Disease Associated with Multiple Myeloma

2012 ◽  
Vol 51 (7) ◽  
pp. 773-776 ◽  
Author(s):  
Armando Mena-Durán ◽  
Elia Muñoz Vicente ◽  
Gonzalo Pareja Llorens ◽  
José Sanchis Cervera
Keyword(s):  
Multiple Myeloma ◽  
Liver Failure ◽  
Light Chain ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition
Sign in / Sign up

Export Citation Format

Share Document