scholarly journals A case of the Light Chain Disease Associated with the Combined Light Chain Nephropathy

1980 ◽  
Vol 3 (3) ◽  
pp. 152-158
Author(s):  
Keigo Ono ◽  
Hirohide Ino ◽  
Jun Akimoto ◽  
Shigeru Sho ◽  
Takeo Kuroyanagi
Keyword(s):  
Light Chain ◽  
Light Chain Disease ◽  
Light Chain Nephropathy

scholarly journals Unusual case of amyloidosis presenting as a jejunal mass

2021 ◽  
Vol 14 (5) ◽  
pp. e240226
Author(s):  
Sachin Mohan ◽  
Elliot Graziano ◽  
James Campbell ◽  
Irshad H Jafri
Keyword(s):  
Small Intestine ◽  
Light Chain ◽  
Protein Misfolding ◽  
Unusual Case ◽  
Gi Tract ◽  
Organ Systems ◽  
Light Chain Disease ◽  
Wide Range ◽  
Amyloid Light Chain ◽  
Possible Cause

Amyloidosis constitutes a heterogeneous group of disorders of protein misfolding that can involve different organ systems. The disease can occur either in a systemic or localised manner that is well known to involve the gastrointestinal (GI) tract. GI amyloidosis can present with a wide range of symptoms including diarrhoea, bleeding and obstruction. This case illustrates a patient with localised jejunal amyloid light chain disease that was diagnosed serendipitously during a workup for haematuria. Our patient was otherwise asymptomatic, but this case underscores the importance of considering amyloidosis as a possible cause of isolated masses of the small intestine.

Light Chain Nephropathy

1992 ◽  
Vol 26 (2) ◽  
pp. 207-209 ◽  
Author(s):  
Muhammad Yaqoob ◽  
Gordon M. Bell ◽  
John M. Davies ◽  
Ian W. McDicken
Keyword(s):  
Light Chain ◽  
Light Chain Nephropathy

Light chain disease and massive proteinuria: comment.

1985 ◽  
Vol 31 (10) ◽  
pp. 1763-1763
Author(s):  
L V De Silva ◽  
F Taylor
Keyword(s):  
Light Chain ◽  
Light Chain Disease

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 <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 < .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.

Correlation of Serum Free Light Chain Levels with Other Parameters in Myeloma.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5019-5019
Author(s):  
Regina Stein ◽  
Jayesh Mehta ◽  
Eric Vickrey ◽  
William Resseguie ◽  
Seema Singhal
Keyword(s):  
Light Chain ◽  
Poor Correlation ◽  
Serum Free ◽  
Monoclonal Protein ◽  
Additional Information ◽  
Serum Free Light Chain ◽  
Light Chain Disease ◽  
Total Light ◽  
Plasma Cell Dyscrasias ◽  
Anuric Renal Failure

Abstract Estimation of serum free light chains (SFLC; SFKLC - kappa, SFLLC - lambda, SFKLR - kappa:lambda ratio; normal 0.26–1.65.) is useful in selected patients with non-secretory myeloma, and in light chain disease with anuric renal failure. Its utility in other clinical situations is unclear. 489 SFLC levels in 135 myeloma patients were analyzed to see if there was a significant correlation between them and other, more conventional immunologic parameters such as serum total light chain (STLC; STKLC - kappa, STLLC - lambda) levels and immunoglobulin (Ig) levels. The underlying premise was that lack of significant correlation would suggest potential value of SFLC independent of standard tests, whereas significant correlation would suggest questionable independent value of SFLC. The table below shows SFLC and STLC levels and ratios. Parameter Median (range) Correlation with SFKLC Correlation with SFLLC Correlation with free k:l ratio SFKLC 14.4 (0.59–11525) SFLLC 18.2 (0.72–10100) Free k:l ratio 0.83 (0–1211.76) STKLC 745 (11–8810) r=0.08; P=0.07 STLLC 256 (11.6–11000) r=0.11; P=0.01 Total k:l ratio 2.51 (0–245) r=0.12; P=0.008 Poor correlation between free and total results within each light chain subtype (Figure 1 - log scale) and between free and total ratios (Figure 2- log scale) suggests that SFLC values provide information that is independent of STLC values because STLC values cannot be used to predict SFLC values. Figure Figure Figure Figure The table below shows correlation between Ig and SFLC/STLC levels. For these analyses, the total Ig of the specific heavy chain subtype and SFLC/STLC of the specific light chain subtype were studied (IgG and SFKLC with IgG kappa monoclonal protein, IgA and STLLC with IgA lambda monoclonal protein, etc). Very strong correlation between STLC and Ig levels suggests that STLC values do not provide information that is independent of Ig levels. On the other hand, poor correlation between SFLC and Ig levels suggests that SFLC values do provide information that is independent of Ig values. Monoclonal protein SFLC correlation STLC correlation IgG kappa r=0.04; P=0.66 r=0.89; P<0.0001 IgG lambda r=0.13; P=0.51 r=0.99; P<0.0001 IgA kappa r=0.49; P=0.004 r=0.77; P<0.0001 IgA lambda r=-0.04; P=0.89 r=0.94; P<0.0001 These data show that SFLC estimation provides information that is independent of standard quantitative serologic tests used in plasma cell dyscrasias. However, rigorous prospective evaluation of the test is needed to see if this additional information is of any clinical relevance in settings other than non-secretory disease and light chain disease with anuria.

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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