scholarly journals Transient Monoclonal Gammopathy Induced by DisseminatedStaphylococcus aureusInfection

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Dimitrios Stoimenis ◽  
Christina Spyridonidou ◽  
Nikos Papaioannou
Keyword(s):  
Staphylococcus Aureus ◽  
B Cell ◽  
Light Chain ◽  
Complete Resolution ◽  
Monoclonal Gammopathy ◽  
Diagnostic Procedures ◽  
Plasma Cell Dyscrasia ◽  
Renal Abscess ◽  
Lambda Light Chain ◽  
Laboratory Findings

Monoclonal gammopathy reflects a serological disorder suggesting a plasma cell dyscrasia or a B-cell abnormality. However, it may occasionally be encountered as a transient manifestation in the course of several diseases including infections. This is the first reported case of a transient monoclonal gammopathy IgG lambda light chain associated with aStaphylococcus aureusinfection that was complicated with renal abscess and vertebral spondylodiscitis in a previously healthy 68-year-old male. We observed a complete resolution of the gammopathy within three months of medical treatment before the entire restoration of all clinical and laboratory findings. Many invasive and cost-intensive diagnostic procedures had preceded the exclusion of a malignancy. The clinical significance and the exact pathogenesis of transient monoclonality are poorly understood and remain a matter of speculation.

Monoclonal immunoglobulin–mediated kidney disease: What is beyond amyloidosis in real practice?

2019 ◽  
Vol 3 (3) ◽  
pp. 105-112 ◽  
Author(s):  
Elena V Zakharova ◽  
Tatyana A Makarova ◽  
Ekaterina S Stolyarevich ◽  
Olga A Vorobyeva
Keyword(s):  
Kidney Disease ◽  
B Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Monoclonal Immunoglobulin ◽  
B Cell Malignancies ◽  
Cast Nephropathy ◽  
Monoclonal Immunoglobulin Deposition Disease ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

Background:Monoclonal immunoglobulin–mediated kidney disease with various patterns of damage may occur in patients with B-cell malignancies and non-malignant monoclonal gammopathies, and the latter are actually merged under the umbrella of monoclonal gammopathy of renal significance. Amyloidosis is the most well-known monoclonal immunoglobulin–related kidney damage. We focused on the rarer conditions and aimed to evaluate the non-amyloid spectrum of monoclonal immunoglobulin–mediated patterns of renal damage in real clinical practice.Methods:A single-center non-interventional retrospective study included 45 patients with pathology-proven non-amyloid monoclonal immunoglobulin–mediated kidney disease, followed during 2002–2018. Disease duration, proteinuria, serum creatinine, need for dialysis at the time of kidney biopsy, clinical diagnosis, and kidney pathology findings were analyzed.Results:No significant differences in the median age, disease duration at the time of biopsy, or main clinical presentation of kidney disease were found between patients with monoclonal gammopathy of renal significance and patients with B-cell malignancies. Pathology patterns like proliferative glomerulonephritis with monoclonal immunoglobulin deposits, membranous nephropathy, C3 glomerulopathy, cryoglobulinemic glomerulonephritis, and combinations of light chain proximal tubulopathy with monoclonal immunoglobulin deposition disease, and of C3 glomerulopathy with light chain proximal tubulopathy were found in monoclonal gammopathy of renal significance setting only. In contrast, light chain proximal tubulopathy alone, anti-glomerular basement glomerulonephritis, and combinations of cast nephropathy with light chain proximal tubulopathy, and cast nephropathy with monoclonal immunoglobulin deposition disease were associated with multiple myeloma only. Monoclonal immunoglobulin deposition disease, intracapillary monoclonal immunoglobulin M deposits, and cast nephropathy alone were seen in both settings.Conclusion:The presence of monoclonal gammopathy in patients with proteinuria and/or impaired kidney function demands kidney biopsy. Neither duration of kidney disease nor its clinical presentation allows differentiating malignant and non-malignant causes of monoclonal immunoglobulin–mediated renal damage. Several pathology patterns, even cast nephropathy, can be found both in cases of monoclonal gammopathy of renal significance and in cases of B-cell malignancies. Dual patterns of damage, including combinations of organized and non-organized deposits, or organized deposits with monoclonal immunoglobulin–induced damage without monoclonal immunoglobulin deposition, constitute up to 9%, mostly in multiple myeloma cases.

Impact of bone marrow fibrosis (BMF) at diagnosis in patients with multiple myeloma (MM).

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e19522-e19522 ◽  
Author(s):  
Mohan Preet ◽  
Jinli Liu ◽  
Constantine A Axiotis ◽  
Albert S Braverman ◽  
Gurinder Singh Sidhu
Keyword(s):  
New York ◽  
Bone Marrow ◽  
Light Chain ◽  
World Health ◽  
African Origin ◽  
Lambda Light Chain ◽  
Laboratory Findings ◽  
Kings County ◽  
Health Organization ◽  
The Impact

e19522 Background: BMF is known to occur in a minority of myeloma patients, but its incidence in American patients of African origin is not known. The impact of BMT on presenting clinical and laboratory findings, and its relationship to genetic variants has not been defined. Methods: Kings County Hospital is located in the East Flatbush section of Brooklyn, New York, where the population is mainly of African-Caribbean origin. Records and bone marrow specimens of myeloma patients who presented from 2000 through 2010 were reviewed. Degree of fibrosis was graded according to World Health Organization criteria: mild, moderate and severe. Results: Records of 113 patients were reviewed, 110 (97%) 97 of whom were African American or Caribbean; 62 (55%) were female and 51 (45%) male. Their ages ranged from 38 to 89 (median 65). Of the 27 patients with BMF (24%), 17 (63%) were female. Mild, moderate and severe BMF were present in 14 (52%), 7 (26%) and 6 (22%) patients respectively. Presentation calcium and creatinine levels were normal in all patients. Hemoglobin levels were similar (median 9.6 G/dl) in patients without BMF and in those with mild and moderate grades, the median level was 7.5 G/dl in those with severe BMF. Immunoglobulin G, A and D levels (67, 20 and 1) were similar in patients with and without BMF, but lambda light chain expression was greater in the BMF patients: 41 vs 24%. Cytogenetic data (CGD) was available in 46 patients; and abnormal in 10 (22%). All patients with abnormalities of chromosomal number were hyperdiploid. Of the 27 BMF patients CG data was available in 17, and was abnormal in 2 (12%). FISH results were normal in 23 of 25 patients, and in all of those with BMF. The fraction of the BMF and non BMF patients surviving after median follow up periods of 828 and 885 days were similar. Conclusions: BMF in this population was 24%, and severe in 5%. Female preponderance is a characteristic of MM patients of African origin, and was more marked in those with BMF. The BMF patients were characterized by more severe anemia and greater lambda light chain expression.

Managing Systemic Light-Chain Amyloidosis

2007 ◽  
Vol 5 (2) ◽  
pp. 179-187 ◽  
Author(s):  
Raymond L. Comenzo
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Response Rate ◽  
Specific Protein ◽  
Plasma Cell Dyscrasia ◽  
Variable Degree ◽  
Organ Function ◽  
Term Survival ◽  
Light Chain Amyloidosis

Amyloidosis is a rare disease in which a specific protein is deposited as aggregated interstitial fibrils that can compromise organ function and lead to death. Immunoglobulin (Ig) light-chain amyloidosis (AL), caused by the monoclonal gammopathy of a plasma cell dyscrasia, is the most common type. A hereditary type is also caused by mutant transthyretin and other proteins. Rarely, a patient with amyloid has both a monoclonal gammopathy and a hereditary protein. In AL, circulating monoclonal Ig light chains can be measured with the free light-chain (FLC) assay and provide a target for therapy to eliminate the underlying plasma cell dyscrasia while supporting the patient's organ function. Amyloid deposits can be resorbed and organ function restored if the amyloid-forming precursor FLC is eliminated. For patients with limited organ involvement, intravenous melphalan in doses from 100 to 200 mg/m2 with autologous stem cell support (SCT) is an effective approach and, when followed at 3 months post-SCT with adjuvant thalidomide and dexamethasone for persistent plasma cell disease, has a 1-year hematologic response rate of 77%. Monthly oral melphalan and dexamethasone for 1 year can also be effective therapy for patients too sick for SCT (67% response rate). Hematologic complete responses are usually durable and result in long-term survival and a variable degree of organ recovery. For patients with advanced cardiac involvement, the prognosis remains guarded even with treatment. Drugs effective in multiple myeloma are usually active in AL, depending on side effects. New agents such as bortezomib and lenalidomide have shown promising activity, and novel antibody-based approaches for imaging amyloid and accelerating removal of deposits are being actively investigated.

Monoclonal Gammopathy of Undetermined Significance (MGUS) Precedes the Diagnosis of AL Amyloidosis by up to 14 Years

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1827-1827 ◽  
Author(s):  
Brendan M Weiss ◽  
Joseph Hebreo ◽  
Daniel Cordaro ◽  
Mark J Roschewski ◽  
Kevin C Abbott ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Conflicts Of Interest ◽  
Diagnostic Delay ◽  
Control Sample ◽  
Light Chains ◽  
Plasma Cell Dyscrasia ◽  
Al Amyloidosis ◽  
Serum Samples

Abstract Abstract 1827 Background. AL amyloidosis (AL) is a plasma cell dyscrasia characterized by tissue deposition of amyloidogenic monoclonal light chains resulting in organ dysfunction and death. Early diagnosis and treatment are critical to prevent severe, irreversible organ damage and prolong survival. However, diagnostic delay is common and there are no current methods for early diagnosis. We have performed the first study to detect a monoclonal gammopathy (MG) prior to the clinical diagnosis of AL. Methods. Twenty cases of AL treated at U.S. Military Treatment Facilities were identified using an electronic database between 1985–2010. Up to three pre-diagnostic serum samples were retrieved from the Department of Defense Serum Repository (DoDSR) along with 20 healthy controls matched for age, sex and race. The DoDSR prospectively collects serum samples every 2 years on all active duty servicemembers; now comprising over 27 million samples on over 4 million servicemembers. Capillary-based serum protein electrophoresis (SPEP) (Sebia Electrophoresis, Norcross, GA), gel-based immunofixation electrophoresis (IFE) (Sebia Electrophoresis, Norcross, GA) and serum free light chain analysis (sFLC) (The Binding Site, San Diego, CA) were performed on all samples. A MG was defined as a positive SPEP, IFE or abnormal sFLC ratio. Results were compared to pre-diagnostic samples from MM patients (Weiss, Blood, 2009). The difference between involved and uninvolved light-chains (FLC-diff) was calculated to account for possible changes in renal function. Results. The median age at diagnosis was 49.5 yrs (30–64), with 95% males, 70% Caucasians and 20% African-American. The median number of samples per case was 3 (1–3), ranging from 0.23 yrs to 19.3 yrs prior to diagnosis. A MG was detected prior to diagnosis in 20/20 (100%) cases and 1/20 (5%) controls. The MG detected in the control sample was an abnormal sFLC ratio that was not present on a subsequent sample. Light chain-MGUS was detected in 11/20 (55%) AL cases, lambda in 8/11(73%) and kappa in 3/11 (27%). Heavy chain expression was detected in 9/20 (45%) cases, IgG in 8/9 (89%) and IgA in1/9 (11%). Among all cases of AL, light chain expression was lambda in 16/20 (80%) and kappa in 4/20 (20%). A MG was present in 100%, 80%, and 42% at less than 4 years, 4–11 years and greater than 11 years prior to diagnosis; a comparison to MGUS prior to MM and controls is shown (figure). The pattern of FLC-diff levels prior to the diagnosis followed evolving and non-evolving patterns. Conclusions. MGUS precedes the clinical presentation of AL for many years providing an opportunity for early diagnosis. MGUS is a heterogeneous condition comprising several subtypes with distinct clinical behavior. Using current methods, including molecular analysis of plasma cells, MGUS destined for AL or MM are indistinguishable. Future studies should investigate methods to distinguish pre-AL MGUS from other subtypes of MGUS, to provide a window for early treatment in this devastating disease. Disclosures: No relevant conflicts of interest to declare.

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