scholarly journals Bortezomib maintenance for the treatment of Monoclonal Gammopathy of Renal Significance

Author(s):  
Holly Lee ◽  
Peter Duggan ◽  
Ernesta Paola Neri ◽  
Jason Tay ◽  
Victor Jimenez Zepeda

Monoclonal gammopathy of renal significance (MGRS) defines renal disease resulting from monoclonal proteins that are secreted from clonal B cells, that does not meet criteria for lymphoma or multiple myeloma. Recognizing MGRS in clinical practice is important because renal outcomes are poor and treatments targeting the underlying clonal disease have been associated with improved renal survival. In this case report, we present a case of a patient with membranoproliferative glomerulonephritis (MPGN) with IgG-kappa deposition who underwent clone directed treatment in a phased approach with induction and maintenance to achieve renal response. This is one of the first cases to report on MGRS treatment that required extended maintenance therapy.

Haematologica ◽  
2013 ◽  
Vol 99 (1) ◽  
pp. 155-162 ◽  
Author(s):  
L. S. Thiago ◽  
M. Perez-Andres ◽  
A. Balanzategui ◽  
M. E. Sarasquete ◽  
B. Paiva ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Siriluk Inthasotti ◽  
Rungsima Wanitphakdeedecha ◽  
Jane Manonukul

Necrobiotic xanthogranuloma (NXG) is a rare destructive xanthomatous granuloma with chronic, indolent, and progressive course. The morbidity and mortality are the results from wound complications and associated disorders. Because of its strong association with monoclonal gammopathy and multiple myeloma, early recognition of disease is mandatory to monitor and prevent systemic involvements of hematologic malignancies.


2020 ◽  
Vol 8 ◽  
pp. 232470962094050
Author(s):  
Asim Kichloo ◽  
Najma Nawaz ◽  
Jagmeet Singh ◽  
Michael Aljadah ◽  
Michael Stanley Albosta ◽  
...  

Monoclonal gammopathy of undetermined significance is a precursor to multiple myeloma characterized by monoclonal gammopathy without evidence of end organ damage. Some patients with clonal plasma cell disorder that do not meet the requirements for multiple myeloma have been seen to develop pathologic renal disease due to direct effects from deposition of monoclonal protein, referred to as monoclonal gammopathy of renal significance. In this article, we present a rare renal manifestation of monoclonal gammopathy of renal significance as focal segmental glomerulosclerosis.


Blood ◽  
2011 ◽  
Vol 118 (11) ◽  
pp. 2985-2987 ◽  
Author(s):  
Rishi K. Wadhera ◽  
Robert A. Kyle ◽  
Dirk R. Larson ◽  
Angela Dispenzieri ◽  
Shaji Kumar ◽  
...  

Abstract During the course of multiple myeloma (MM), new monoclonal proteins of an isotype distinct from the original clone, referred to as secondary monoclonal gammopathy of undetermined significance (MGUS), have been described. We report on the frequency, characteristics, and outcome of secondary MGUS. Of the 1942 patients with MM, 128 (6.6%) developed a secondary MGUS, at a median of 12 months from the diagnosis of MM. The median duration of secondary MGUS was 5.9 months. Secondary MGUS was more common in patients after stem cell transplantation than in those who had not undergone such treatment (22.7% vs 1.6%, P < .001). Overall survival was significantly superior in MM patients who developed secondary MGUS compared with the rest of the cohort (73 vs 38 months, respectively; P < .001). The time of onset and the duration of secondary MGUS, as well as failure to resolve spontaneously, had an effect on overall survival and require further study.


Author(s):  
Adrian S.W. Tordiffe ◽  
Nicky Cassel ◽  
Emily P. Lane ◽  
Fred Reyers

Multiple myeloma is a rare, systemic proliferation of neoplastic plasma cells. A case was reported in an 11-year-old male captive lion (Panthera leo) at the National Zoological Gardens of South Africa, Pretoria. The classic features of symptomatic multiple myeloma were all evident in this case; namely osteolytic lesions, monoclonal gammopathy in the serum with excretion of monoclonal proteins in the urine, neoplastic plasma cells in the bone marrow and associated renal failure and anaemia. In addition, similar to the common pattern of this disease in domestic felids, at least three extramedullary tumours were found and several organs were infiltrated by neoplastic plasma cells. The cytoplasm of approximately 50%of the neoplastic round cells, including a few giant myeloma cells, stained weakly to strongly using immunohistochemical stains for B-lymphocytes (CD79a). The normal haematological parameters and lack of any osteolytic lesions in the lion at the time of the first evaluation suggest that the primary neoplastic cells could have originated from one of the extramedullary tumour sites. Only two cases of multiple myeloma have previously been reported in captive wild felids. To the authors’ knowledge, there are no case reports of multiple myeloma in lions.


2020 ◽  
Vol 18 (12) ◽  
pp. 1720-1729
Author(s):  
Timothy M. Schmidt ◽  
Natalie S. Callander

The presence of monoclonal proteins is common, with a prevalence in the United States around 5% that increases with age. Although most patients are asymptomatic, most cases are caused by a clonal plasma cell disorder. Monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) are asymptomatic precursor conditions with variable risk of progression to multiple myeloma. In recent years, significant progress has been made to better understand the factors that lead to the development of symptoms and progression to myeloma. This review summarizes the current diagnosis treatment guidelines for MGUS and SMM and highlights recent advances that underscore a shifting paradigm in the evaluation and management of plasma cell precursor conditions.


2005 ◽  
Vol 51 (5) ◽  
pp. 878-881 ◽  
Author(s):  
Jerry A Katzmann ◽  
Roshini S Abraham ◽  
Angela Dispenzieri ◽  
John A Lust ◽  
Robert A Kyle

Abstract Background: The quantitative assay for free light chains (FLCs) is a recently introduced commercial test reported to be sensitive and specific for detecting FLC diseases such as primary systemic amyloidosis (AL), light chain deposition disease (LCDD), nonsecretory multiple myeloma (NSMM), and light chain multiple myeloma. We evaluated its diagnostic performance in clinical practice. Methods: All FLC clinical test results generated in 2003 were abstracted from the Laboratory Information System. Diagnoses were obtained from the Dysproteinemia database and the patient medical history. Results: In 2003, we received samples for FLC assays from 1020 Mayo Clinic patients. The majority of these patients (88%) had bone marrow-derived monoclonal plasma cell disorders (PCDs). The 121 patients who did not have monoclonal gammopathy all had FLC κ/λ ratios within the range of values obtained for a reference population in our laboratory. Among the patients with monoclonal gammopathies were patients with multiple myeloma (330), AL (269), monoclonal gammopathy of undetermined significance (114), smoldering multiple myeloma (72), plasmacytoma (22), NSMM (20), macroglobulinemia (9), LCDD (7), and a variety of other PCDs. Among the 110 AL patients who had not been previously treated and who had a FLC assay performed within 120 days of diagnosis, the FLC κ/λ ratio was positive in 91% compared with 69% for serum immunofixation electrophoresis (IFE) and 83% for urine IFE. The combination of serum IFE and serum FLC assay detected an abnormal result in 99% (109 of 110) of patients with AL. Conclusion: The performance of the FLC assay in this analysis of clinical laboratory data is consistent with results from published retrospective validation studies.


Sign in / Sign up

Export Citation Format

Share Document