A case of multiple myeloma associated light chain disease: presenting with severe intrahepatic cholestasis and renal failure

2001 ◽  
Vol 96 (9) ◽  
pp. S223
Author(s):  
D MEYER
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Light Chain Disease

scholarly journals Renal involvement as the first symptom of multiple myeloma

2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Light Chain ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Renal Amyloidosis ◽  
Cast Nephropathy ◽  
Light Chain Disease ◽  
Immunofixation Electrophoresis

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.

scholarly journals Kidney failure as an unusual initial presentation of biclonal gammopathy (IgD multiple myeloma associated with light chain disease): A case report

2015 ◽  
Vol 72 (2) ◽  
pp. 196-199
Author(s):  
Violeta Rabrenovic ◽  
Zoran Mijuskovic ◽  
Slobodan Marjanovic ◽  
Milorad Rabrenovic ◽  
Dragan Jovanovic ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Case Report ◽  
Light Chain ◽  
Bone Biopsy ◽  
Autologous Bone Marrow Transplantation ◽  
Laboratory Analysis ◽  
Team Work ◽  
Light Chain Disease ◽  
Biclonal Gammopathy

Introduction. Immunoglobulin D (IgD) myeloma is a rare disease, about 2% of all myelomas, even rarer when accompanied with another multiple myeloma in biclonal gammopathy. We presented a case of biclonal gammopathy - associated manifestation of IgD myeloma and light chain disease in a patient who initially had renal failure. Case report. 37-year-old male approximately one month before hospitalization began to feel malaise and fatigue along with decreased urination. Laboratory analysis revealed azotemia. A dialysis catheter was placed and hemodialysis started. The patient was then admitted to our hospital for further tests and during admission, objective examination revealed pronounced paleness with hepatosplenomegaly and hypertension (170/95 mmHg). Laboratory analysis showed erythrocyte sedimentation rate 122 mm/h, expressed anemic syndrome (Hb 71 g/L) and renal failure dialysis rank: creatinine 1,408 ?mol/L, urea31.7 mmol/L. There was two M components in serum protein electrophoresis: IgD lambda and free light chain lambda. Proteinuria was nephrotic rank (5.4 g/24 h), whose electrophoresis revealed 2 M components - massive in ? 2 fraction of 71%; 7% in the discrete ? fraction, beta 2M /serum 110 mg/L, in urine 1.8 mg/L - extremely high; IgL kappa / lambda index 1 : 13 (reference value ratio 2 : 1). The findings pointed to double myeloma disease: IgD myeloma and Bence Jones lambda myeloma. Bone biopsy confirmed IgD myeloma lambda 100% infiltration medulla predominantly plasmablasts. The treatment continued with hemodialysis 3 times per week with chemotherapy protocol bortezomib, doxorubicin, dexamethasone. After 4 cycles of chemotherapy, there was a decrease of IgD, ? - light chains, reduction in proteinuria (1.03 g/24 h), so hemodialysis was reduced to once per week. Six months after treatment initiation the patient underwent autologous bone marrow transplantation. In a 2-year follow-up period double myeloma disease showed complete remission. Conclusion. The presented rare form of double myeloma disease with initial renal insufficiency underscores the importance of careful observation and team-work that can alter the course of this serious disease.

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 Outcomes with early response to first-line treatment in patients with newly diagnosed multiple myeloma

2019 ◽  
Vol 3 (5) ◽  
pp. 744-750 ◽  
Author(s):  
Nidhi Tandon ◽  
Surbhi Sidana ◽  
S. Vincent Rajkumar ◽  
Morie A. Gertz ◽  
Francis K. Buadi ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
High Risk ◽  
Light Chain ◽  
Progression Free Survival ◽  
Early Response ◽  
Stage Iii ◽  
Newly Diagnosed ◽  
Best Response ◽  
Light Chain Disease ◽  
The Impact

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.

scholarly journals Double light-chain disease: a case report.

1979 ◽  
Vol 25 (1) ◽  
pp. 190-192 ◽  
Author(s):  
F R Dalal ◽  
S Winsten
Keyword(s):  
Multiple Myeloma ◽  
Case Report ◽  
Column Chromatography ◽  
Light Chain ◽  
Clinical Studies ◽  
Light Chains ◽  
Light Chain Disease

Abstract A patient with massive proteinuria was discovered to have double light-chain disease. Immunological studies demonstrated monoclonal light chains of both the lambda and kappa type in urine. The light chains were separate and distinct and were not found to be a part of any of the whole molecule immunoglobulins such as IgG, IgM, IgA, IgD, or IgE. Uniqueness of the proteins was confirmed by column chromatography. Clinical studies showed that the patient had multiple myeloma.

Improving Outcomes in Patients with Renal Failure Secondary to Multiple Myeloma: Results From ChAR2M2.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1875-1875
Author(s):  
Colin Hutchison ◽  
Parisa Airia ◽  
Mark Cook ◽  
Daniel Grima
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Side Effects ◽  
Light Chain ◽  
De Novo ◽  
Conflicts Of Interest ◽  
Treatment Patterns ◽  
Free Light Chain ◽  
High Rate ◽  
Sustained Reduction

Abstract Abstract 1875 Poster Board I-900 Study purpose: To explore how free light chain (FLC) removal by high cut-off haemodialysis (HCO-HD) has been adopted into clinical practice for the management of renal failure secondary to multiple myeloma. Describing treatment patterns and the laboratory and clinical outcomes associated with its use. Methods: A chart audit of patients treated with FLC removal by HCO-HD, using the Gambro HCO 1100 dialyser, was performed in 16 dialysis centers across 9 countries. Patient demographics, treatment patterns and dialysis side-effects were recorded. In addition, the following outcomes were measured: dialysis independence and reductions in serum FLCs concentrations at 12 and 21 days. Results: Data for 66 patients was entered. Patients had an average age of 65.1 (SD×10.1); 42 of them (63.64%) were male and 24 (36.36%) were female. Sixteen (24%) presented with relapsing myeloma and 50 (76%) had de novo disease. On average, each patient received 13 HCO-HD sessions (SD×8). Forty-one patients became dialysis independent (62.12%), after an average of 12 sessions. Dialysis related side-effects were reported in 6% of all patients. Forty patients (60.61%) were reported to have a sustained reduction in serum FLC concentrations by day 12. By day 21 this had increased to forty-one (62.12%). Among the patients who achieved a sustained reduction in serum FLC concentrations, 28 (70%) had a decline in FLC levels of more than 50% by day 12 and 34 (82.93%) by day 21. Among patients who achieved sustained reduction of more than 50% in serum FLC concentrations by day 12, 75% became dialysis independent. In comparison only 53% of those with a reduction of less than 50% became dialysis independent (p×0.007). Furthermore, among patients who achieved sustained FLC reduction of greater than 75%, 81% became dialysis independent. The rate of dialysis independence was also significantly higher in patients with de novo disease compared with those with relapsing myeloma (64% versus 56%, p×0.04). Conclusion: Free light chain removal by HCO-HD was well tolerated and associated with a very high rate of dialysis independence in patients with renal failure secondary to multiple myeloma. Rates of renal recovery were greater in patients with de novo myeloma and those who achieved an early reduction in serum FLC concentrations. Disclosures: No relevant conflicts of interest to declare.

Combined Bendamustine, Prednisone and Bortezomib (BPV) In Patients With Relapsed Or Refractory Multiple Myeloma and Light Chain Induced Renal Failure

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3203-3203 ◽  
Author(s):  
Wolfram Pönisch ◽  
Barbara Moll ◽  
Dietger Niederwieser
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Renal Dysfunction ◽  
Light Chain ◽  
Severe Side Effect ◽  
Renal Response ◽  
Group A ◽  
Severe Renal Dysfunction ◽  
Grade 3 ◽  
Group B

Abstract Introduction Serious renal failure represents a main complication of Multiple Myeloma (MM). An estimated 25% to 50% of patients are affected during the course of their disease. These patients are at increased risk for infections and have a significantly worse prognosis. Small phase I/II studies suggest that treatment with chemotherapy and/or new substances results in recovery of renal function in more than 25%. The window of opportunity for reversal of renal impairment is rather small making an immediate and highly active treatment strategy mandatory. Bortezomib and bendamustine have turned out to be quickly acting and effective drugs in the treatment of MM. Methods Between March 2005 and March 2013, 36 patients (median age 64; range 32-81 years) with relapsed/refractory MM and light chain induced renal failure (creatinine clearance <60ml/min) were treated with bendamustine 60mg/qm on day 1 and 2, bortezomib 1.3mg/qm on day 1, 4, 8 and 11, and prednisone 100mg on day 1, 2, 4, 8 and 11. Cycles were repeated every 21 days. Patients were divided into two groups: group A (n=20) consisted of patients with moderate or severe renal dysfunction (eGFR 15-59ml/min) and group B (n=16) of patients with renal failure/dialysis (eGFR <15ml/min). Results The median number of the BPV-treatment was 2 (1-7) cycles. 24 patients (67%) responded after at least one cycle of chemotherapy with 3 CR, 3 nCR, 6 VGPR, and 12 PR. Six patients had MR, 2 patients stable disease and 4 patients had a progress. With a median follow up of 22 months of the surviving patients, median PFS and OS for patients with moderate or severe renal dysfunction (group A) were 10 months and 25 months, respectively. Outcome for these patients was significantly better compared to patients with renal failure/dialysis (group B) with a median PFS and OS of 3 months and 7 months, respectively (p<0.02). Eleven patients showed a CRrenal, 5 patients a PRrenal and 15 patients a MRrenal. Median time to first renal response and best renal response were 21 days and 42 days, respectively. The most common severe side effect was grade 3-4 thrombocytopenia in 81% of the patients. Grade 3-4 neutropenia was observed in 50% of the patients. Moderate to severe infections were seen in 13 patients. Summary These results indicate that the combination of bortezomib, bendamustine and prednisone is effective and well tolerated in patients with relapsed/refractory MM and light chain induced renal failure. Disclosures: No relevant conflicts of interest to declare.

Efficacy Of Lenalidomide Treatment In Multiple Myeloma (MM) Patients – a Report of Polish Myeloma Group

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3236-3236
Author(s):  
Lidia Usnarska-Zubkiewicz ◽  
Jakub Debski ◽  
Aleksandra K. Butrym ◽  
Wojciech Legiec ◽  
Marek Hus ◽  
...  
Keyword(s):  
Renal Failure ◽  
Disease Progression ◽  
Light Chain ◽  
Response Rate ◽  
Conflicts Of Interest ◽  
Staging System ◽  
Total N ◽  
Light Chain Disease ◽  
Disease Stabilization ◽  
Beta 2

Abstract The aim of the multi-centre observational study was to evaluate the efficacy of lenalidomide (len) therapy in patients (pts) with resistant or relapsed MM as well in pts who continued treatment with len due to complications from the earlier lines of the therapy. The study involved 306 pts, 153 women and 153 men, aged 26-89 years (mean 60,5); on the onset of lenalidomide therapy 115 pts (38,8 %, Cohort 1, C1) were diagnosed with resistant MM, 135 pts (44,1%, Cohort 2, C2) had relapse of the disease, and 56 (18,3 %, Cohort 3, C3 ) len was instituted as continuation therapy due to polyneuropathy. In Stage I, II and III according to Durie - Salmon staging system there were 36, 88 and 182 pts respectively; 23 pts developed renal failure. 179 pts had IgG myeloma, 78 – IgA, 31pts were diagnosed with light chain disease, 13 – with non-secretory myeloma, 3- IgM, 2 -IgD and 196 with kappa light chain disease. Prior to len therapy the pts received 1-9 treatment lines (mean:3); 83 pts underwent megachemiotherapy. Average time from diagnosis to start of len treatment was 47 months (1-230). In 284 (92,8%) pts, len was administered at the dose of 25mg p.o , on days 1-21, in the remaining 22 (7,2%) pts (14, 3, 5 pts respectively) at the dose of 15mg, 10mg or 5 mg for 21 days, and dexamethasone (dx) at the dose of 20 mg on days 1-4, 8-11. 28 pts received only len at the dose of 25 mg for 21 days; individual pts were administered len with bortezomib or bendamustin and dexamethasone. The cycles were repeated every 28 days. All the pts were administered aspirin 75mg as the prophylaxis of deep vein thrombosis. Therapeutic response was evaluated on the basis of modified criteria of the European Group for Blood and Marrow Transplantation in all pts. At the time of the assessment 32 (10,45% ) pts have completed 1-2 cycles, 48 (15,7%) 3-4 cycles, the others 6 and more. The response rate was highest in pts continuing treatment, significantly higher than in resistant and relapsed myeloma. The response rate in group 1 and 2 was comparable 68,7% i 85,7%.(tabl ) respectively.ResponseResistant MM n=115 (C1);Relapsed MM n=135 (C2);Treatment continuation n=56 (C3);Total (n=306)CR/sCR10 (8,7 %)13 (9,6 %)15 (26,8 %)38VGPR11 (9,6 %)19 (14,1%)19 (33,9 %)49PR58 (50,4%)70 (51,9 %)16 (28,6 %)144SD29 (25,2 %)21 (15,6 %)3 (5,3 %)53PD7 (6,1 %)12 (8,9 %)3 (5,3 %)22Response:79 (68,7%)102 (85,7%)50 (89,2%)No response:36 (31,3%)33 (27,7%)6 (10,%)PC1:C2 =0,2266PC1:C3=0,0033PC2:C3=0,0321 The percentage of all responses was significantly higher in pts without renal insufficiency compared to those with renal failure (224/283 vs. 11/23) p= 0,0006, and in the group of pts whose serum beta-2-microglobulin was <3.5 mg/L compared to those with beta-2-m > 3.5 mg/L 89/112 vs 45/68 p=0,0316. There were no significant differences in response to len, depending on the number of previous lines of treatment 1 vs.> 1, stage ISS, and underwent or no megachemotherapy. The median TTP was 14.0 months in pts who responded to treatment with len and dx (CR, nCR, VGPR, PR). The TTP in pts with disease stabilization, or disease progression was significantly shorter (p = 0.0000), median 5.0 months. The median TTP was 9.0 months in pts with resistant MM, 8.0 months in pts with relapsed MM, and was significantly shorter (p = 0.00007) compared with median TTP in pts continuing treatment (16.50 months). The median OS was 15.0 months in pts who responded to treatment with len and dx (CR, nCR, VGPR, PR), and it was significantly longer (p = 0.00000) than the median OS in pts with disease stabilization, or disease progression (median 8.0 months). In pts with resistant MM median OS was 10.50 months, in pts with relapsed MM -11.0 months, and were significantly shorter (p = 0.00077) comparing to pts continuing treatment (18.0 months). Summary Lenalidomide with dexamethasone is an orally administrated and effective both in resistant and relapsed patients as well as maintenance therapy. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Rare Presentation of Primary Extramedullary Plasmacytoma as Lip Lesion

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Mali Him ◽  
Maggie Meier ◽  
Vikas Mehta
Keyword(s):  
Multiple Myeloma ◽  
Cell Proliferation ◽  
Plasma Cell ◽  
Light Chain ◽  
Extramedullary Plasmacytoma ◽  
Solitary Plasmacytoma ◽  
Rare Presentation ◽  
Lower Lip ◽  
Light Chain Disease ◽  
Extramedullary Plasmacytomas

Malignant plasma cell proliferation can be presented as part of disseminated disease of multiple myeloma, as solitary plasmacytoma of bone, or in soft tissue as extramedullary plasmacytoma. Extramedullary plasmacytomas represented approximately 3% of all plasma cell proliferation. Approximately 80% of extramedullary plasmacytomas occur in the head and neck region while the other 4% occur in the skin and to a lesser extent in the lip. In this paper, we report a rare case of primary cutaneous plasmacytoma involving the lip in a 65-year-old male. The patient presented with a nonhealing lower lip sore for the past 3 years. Upon further workup, there was no evidence of multiple myeloma or light chain disease. The patient was treated with radiation therapy and his last follow-up revealed no evidence of multiple myeloma or light chain disease.

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