scholarly journals Renal Tubular Acidosis type II Secondary to .LAMBDA.-Light Chain Excretion in an Elderly Patient with Multiple Myeloma.

1998 ◽  
Vol 35 (6) ◽  
pp. 477-481
Author(s):  
Kent Doi ◽  
Shinji Teramoto ◽  
Takayuki Hosoi ◽  
Mariko Miyao ◽  
Takeshi Matsuse ◽  
...  
Keyword(s):  
Elderly Patient ◽  
Multiple Myeloma ◽  
Light Chain ◽  
Renal Tubular Acidosis ◽  
Type Ii ◽  
Lambda Light Chain ◽  
Renal Tubular

scholarly journals Renal tubular acidosis type II associated with vitamin D deficiency presenting as chronic weakness

2014 ◽  
Vol 5 (4) ◽  
pp. 86-89 ◽  
Author(s):  
Yaseen Ali ◽  
Amila Parekh ◽  
Mirza Baig ◽  
Taseen Ali ◽  
Tazeen Rafiq
Keyword(s):  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Renal Tubular Acidosis ◽  
Type Ii ◽  
Renal Tubular

A mouse model of pseudohypoaldosteronism type II reveals a novel mechanism of renal tubular acidosis

2018 ◽  
Vol 94 (3) ◽  
pp. 514-523 ◽  
Author(s):  
Karen I. López-Cayuqueo ◽  
Maria Chavez-Canales ◽  
Alexia Pillot ◽  
Pascal Houillier ◽  
Maximilien Jayat ◽  
...  
Keyword(s):  
Mouse Model ◽  
Renal Tubular Acidosis ◽  
Type Ii ◽  
Pseudohypoaldosteronism Type Ii ◽  
Renal Tubular ◽  
Pseudohypoaldosteronism Type

scholarly journals Metastatic Multiple Myeloma to the Skin

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Alex C. Holliday ◽  
Mohammed I. Khan ◽  
Sean E. Mazloom ◽  
Rahul N. Chavan ◽  
Douglas J. Grider
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Standard Treatment ◽  
Plasma Cells ◽  
Treatment Approach ◽  
Prognostic Indicator ◽  
Lambda Light Chain ◽  
Stage Disease ◽  
Poor Prognostic Indicator

Cutaneous involvement of multiple myeloma (MM) is uncommon, typically occurs in late stage disease, and is a poor prognostic indicator with an approximate eight month median survival. We present a 51-year-old man with relapsed lambda light chain MM who developed abrupt asymptomatic skin metastases. Biopsy revealed a dermis replete of atypical plasma cells, positive for CD138 and CD45. In situ hybridization confirmed lambda light chain restriction. Despite rescue antimyeloma therapy with the anti-CD38 drug daratumumab, he rapidly declined clinically and succumbed to the disease four weeks after presentation. A standard treatment approach for cutaneous MM does not currently exist; however, various techniques to detect cytogenetic abnormalities are emerging and will provide additional prognostic value and direct individualized therapy.

Marked efficacy of rituximab in two patients with Waldenstrom macroglobulinemia-like multiple myeloma.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e18548-e18548
Author(s):  
Christoph J. Heuck ◽  
Saad Zafar Usmani ◽  
Erming Tian ◽  
Qing Zhang ◽  
Frits Van Rhee ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Plasma Cells ◽  
Single Agent ◽  
Organ Damage ◽  
Detailed Examination ◽  
Lambda Light Chain ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
First Case

e18548 Background: Rituximab (R) has been deemed to be ineffective in multiple myeloma (MM), despite CD20 expression in 10-15% of MM. Here we report two cases, selected by a genomic approach, with an excellent response to single agent R. Methods: as below Results: Patient 1: A 49 yr old male with IgG lambda MM with 80% bone marrow (BM) plasma cells (PC) and IgG level of 23 g/L had been treated elsewhere with one cycle of CRD. Here, we noted CD-2 subclass by gene expression profilin (GEP), however without spiked expression of CCND1 and CCND3 genes as manifestation of a t[11:14] or a t[6:14]. GEP further revealed a del 6q and overexpression of EBI2, both commonly seen in Waldenstrom Macroglobulinemia (WM). All findings were confirmed by FISH. Unsupervised clustering in the context of MGUS, untreated MM and WM-PC, confirmed WM-like MM in this patient. Sole therapy with R (750 mg/m2/d x 5d, weekly x 4, bi-weekly x 4 and then monthly) resulted in a reduction of IgG from 1850 mg/dL to 950 mg/dl and BM PC from 60% to 10% at 9 months and a decrease in sLFLC from 68 mg/dL to 10 mg/dL at 12 months follow up. Patient 2: Based on the above observation, we identified a second patient. This 37-yr old male had been diagnosed with lambda light chain MM 42 months earlier with a BM PC of 15%, lambda light-chain proteinuria of 1.9 g/d and sLFLC in the 200mg/dL range. Because of absence of CRAB criteria, he was followed expectantly. Rising BM PC to 50% and concern for end-organ damage motivated a detailed examination of GEP data. GEP showed high expression of CD20 and EBI2 and absence of CCND1 and CCND3 spikes. This was confirmed by FISH, which also revealed a del 6q. As in the first case, this patient co-segregated with WM. R treatment on the same schedule resulted in a reduction of sLFLC levels from 249 mg/dL to 29.9 mg/dl and of Bence Jones proteinuria from 1766 mg/d to 242 mg/d. Conclusions: The presumed lack of activity of R in MM needs to be revisited in light of the marked response noted in these 2 patients. Studies are in progress (a) to extend R therapy to similar cases, and (b) to more fully characterize the prevalence of genetic/phenotypic characteristics, as seen in these 2 cases, among several thousand MM patients. This updated information will be presented at the meeting.

Complex Acid-Base Disorders in Subacute Necrotizing Encephalomyelopathy (Leigh's Syndrome)

PEDIATRICS ◽  
1978 ◽  
Vol 61 (2) ◽  
pp. 278-281
Author(s):  
Gladys H. Hirschman ◽  
James C. M. Chan
Keyword(s):  
Metabolic Acidosis ◽  
Clinical Data ◽  
Renal Tubular Acidosis ◽  
Respiratory Alkalosis ◽  
Type I ◽  
Type Ii ◽  
Acid Base ◽  
Hybrid Type ◽  
Leigh’S Syndrome ◽  
Renal Tubular

This report describes a case of subacute necrotizing encephalomyelopathy (Leigh's syndrome) in a 7-month-old boy. The clinical data suggest an association with a disorder of renal tubular acidification, characterized by both (proximal) type II and (distal) type I renal tubular acidosis (hybrid type). Concomitantly, the initial uncompensated metabolic acidosis evolved into a mixed metabolic acidosis and respiratory alkalosis-features of this syndrome not previously reported.

scholarly journals Triphenotypic multiple myeloma expressing kappa or lambda light chain or both

2012 ◽  
Vol 160 (1) ◽  
pp. 4-4 ◽  
Author(s):  
Yang Liu ◽  
Yan Zhang ◽  
Weidong Han
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Lambda Light Chain

scholarly journals A Prospective Observational Study of Clinico-Pathological Features, Prognostic Factors and Treatment Response to Primary Therapy in Multiple Myeloma at a Tertiary Care Centre

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5559-5559
Author(s):  
Saurabh Mishra ◽  
Ashok K Vaid ◽  
Nitin Sood ◽  
Manorama Bhargava ◽  
Bhuvan Chugh ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Treatment Response ◽  
Light Chain ◽  
Prospective Observational Study ◽  
Tertiary Care ◽  
High Dose ◽  
Primary Therapy ◽  
Lambda Light Chain ◽  
Tertiary Care Centre ◽  
Cell Support

Title: "A prospective observational study of clinico-pathological features, prognostic factors and treatment response to primary therapy in Multiple Myeloma at a tertiary care centre" Background: Multiple myeloma (MM) is characterised by the neoplastic proliferation of plasma cells leading to excess monoclonal immunoglobulins. The incidence of multiple myeloma in India ranges from 1.2 to 1.8 per 100,000. There is paucity of cytogenetic data from this part of subcontinent. The aim of our study is to report various clinicopathological features, evaluate biological markers of prognostication including cytogenetic variables and assess treatment response after standard primary therapy. Methods and Materials: The study was carried out at a tertiary care center in Northern India. After final diagnosis of multiple myeloma was established, each patient was risk stratified via FISH cytogenetic analysis as per the revised ISS. Definitive management plan was individualised, including assessment for high dose therapy with peripheral blood stem cell support. Treatment response were recorded as per standard IMWG response criteria. Significant toxicities associated with treatment were also recorded. Results: Eighty consecutive patients with newly diagnosed multiple myeloma were enrolled prospectively from April 2017 to November 2018. The median age at diagnosis was 63 years, and number of males & females were equal. The most common presenting symptom was back pain (67.8 % patients) and most frequent clinical sign was pallor (86.4 %). All four CRAB features were documented only in 16.9% patients. M-Band was present in 96.6% patients and on SFLC assay 59.3% and 40.7% were kappa and lambda light chain restricted respectively. Most common heavy chain abnormality detected was IgG. Seventy percent patients had lytic lesions on imaging while only 3% suffered skeletal related events. Cytogenetic evaluation by interphase FISH was carried out in all patients upfront. No chromosomal abnormality was documented in 61.25 % while among those with chromosomal abnormalities, most commonly detected was del13q14.3 (23.75 %). Overall, 66.6% patients were stratified as standard risk, 28.1% as intermediate risk and only 3 (5.3%) patients were categorised as high risk. Most common induction regimen was VRD. Overall response rate was 94.9 % and VGPR or better responses were observed in 77.9% patients. Most common adverse effect of therapy was peripheral neuropathy of all grades. Of the 77 patients who completed primary therapy, 21.4% patients underwent high dose therapy with peripheral blood stem cell support while 67.7% patients were started on maintenance therapy. Four (5.1 %) non-responder was started on 2nd line treatment. On univariate analysis, higher deeper responses (VGPR or better) were observed in patients with IgA & IgG related myeloma and better overall response rates were seen in IgG related myeloma. Those with kappa light chain myeloma had 5.67 times higher likelihood of achieving response as compared to lambda light chain myeloma. Also, patients with kappa light chain myeloma achieved higher VGPRs as compared to lambda light chain myeloma. There was 17 times high risk of non-response in the presence of local bony tenderness. None of the findings were found to be significant on multivariate analysis. Conclusions: Use of interphase FISH to identify various cytogenetic markers help in stratification & staging of the disease which in turn act as a marker for prognostication. They should be a part of standard care in multiple myeloma. In majority we could administer treatment in accordance to standard practice guidelines and response rates were similar to those reported my seminal studies. Our study in a longer follow up will yield some useful information which will help in the better care of the patients. Disclosures No relevant conflicts of interest to declare.

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