scholarly journals A Rare Presentation of Biclonal Gammopathy in Multiple Myeloma with Simultaneous Extramedullary Involvement: A Case Report

2019 ◽  
Vol 12 (2) ◽  
pp. 537-542
Author(s):  
Pragnan Kancharla ◽  
Eshan Patel ◽  
Kenneth Hennrick ◽  
Sherif Ibrahim ◽  
Mendel Goldfinger

Multiple myeloma is characterized by an abnormal clone of plasma cell infiltration in the bone marrow and presence of a high level monoclonal immunoglobulin (M-protein) in the serum or urine in most cases. The monoclonal protein is usually detected as a discrete band in the gamma globulin region by serum protein electrophoresis. Rarely, it may show a simultaneous presence of two distinct bands, which could be either from a single clone, or two separate clones. We report a very unusual presentation of biclonality with lambda restricted IgG predominant cells from cervical lymph node and kappa restricted IgA cells from the bone marrow simultaneously.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5146-5146
Author(s):  
Rosane Bittencourt ◽  
Andreia D. Almeida ◽  
Joao R. Friederich ◽  
Flavo Fernande ◽  
Laura Fogliatto ◽  
...  

Abstract In the last two decades, we have seen a radical change in the therapy and prognosis of Multiple Myeloma (MM). Recent research on the role of the bone marrow microenvironment as integrant part of the biology of MM and the possible therapeutic interference at this level are leading to control and regression of the malignant plasma cell clone with a recognized clinical impact. Thalidomide, an old drug thought to interfere on the bone marrow microenvironment, is active not only for the treatment of refractory patients but also, as was recently shown, for induction and/or remission maintenance therapy. Most of the side effects (somnolence, constipation, fatigue, tremor, bradycardia, edema, and neuropathy) can be managed by reducing the dose and most patients need a dose reduction. The appropriate dose of thalidomide in myeloma is unknown. We treated thirty-five patients with MM with low-dose thalidomide (200mg or less). Twenty four patients were on maintenance therapy (13 after bone marrow transplantation, and 11 after chemotherapy with VCAP/VMCP); 5 patients were treated after relapse, 4 with refractory disease and 2 for remission induction as a first line therapy. Patients were treated at the Hematology and Bone Marrow Transplantation Service of the Hospital de Clínicas de Porto Alegre, RS, Brazil, from march/2001 to December/2003. The response as measured by hemoglobin level, immunoglobulin (M component) or urinary light chain concentration and bone marrow examination was evaluated before, 3, 6, and 12 months after the beginning of thalidomide. Results: All patients are alive and well. Fifty one percent are on 100mg schedule; of the patients on maintenance therapy 90% are on complete remission or on a sustained plateau. A Wilcoxon ranks test for the immunoglobulin level before and after 6 months for the entire group showed p=0.001 (25–75%). Conclusion: Low dose thalidomide is tolerable and effective.


2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Maciej R. Czerniuk ◽  
Artur Jurczyszyn ◽  
Grzegorz Charlinski

Multiple myeloma (myeloma multiplex (MM)) is a malignant non-Hodgkin’s lymphoma derived from B cell. Its essence is a malignant clone of plasma cells synthesizing growth of monoclonal immunoglobulin, which infiltrate the bone marrow, destroy the bone structure, and prevent the proper production of blood cells components. The paper presents a case of 62-year-old patient who developed symptoms in addition to neurological and haematological changes in the oral mucosa in the course of multiple myeloma. The treatment resulted in partial improvement. The authors wish to draw attention not only to nonspecificity and rarity of changes in the mouth which can meet the dentist but also to the complexity of the multidisciplinary therapy patients diagnosed with MM.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5618-5618
Author(s):  
Ashtami Banavali ◽  
Elvira Neculiseanu ◽  
Padma L. Draksharam ◽  
Sireesha Datla ◽  
Maushmi Savjani ◽  
...  

Abstract Introduction Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of monoclonal immunoglobulin (Ig), without the diagnosis of multiple myeloma (MM), while the diagnosis of MM requires the presence of a monoclonal immunoglobulin (Ig) ≥ 3g/dl or Bence-Jones protein (BJP) ≥ 500mg/24h, with bone marrow involvement of greater than 10% clonal plasma cells. Kings County Hospital (KCH) is an institution that serves a large population of Afro-Caribbean population. In our institution, an increased number of patients were observed to present with MM with monoclonal Ig <3g/dl but yet have end organ damage. The clinical characteristics of Afro-Caribbean population have not been well studied and further characterization may have diagnostic implications. Methods This is a retrospective study conducted at KCH. Data regarding the clinical profile of patients diagnosed with MM from 2000- 2013 was collected from the institution's tumor registry. Patients with monoclonal Ig < 3g/dl and BJP <500mg/24h were analyzed and compared to standard MM patients with monoclonal Ig ≥ 3g/dl or BJP ≥ 500mg/24h. Data was collected for lab parameters which included quantity and type of monoclonal Ig, serum free light chains and ratio, monoclonal plasma cell percentage in the bone marrow, International staging system (ISS) stage, cytogenetics, presence of anemia, hypercalcemia, renal failure and lytic lesions. Epidemiologic parameters age and gender were also collected. Results were analyzed by a Chi-square test to calculate a mid-P exact. Results A total of 287 patients with MM were screened, of which 56 patients with incomplete electronic records were excluded. Of the remaining 231 patients, 63 (27%) had monoclonal Ig <3 g/dl without the presence of BJP ≥ 500mg/24h. These patients were labeled hyposecretory MM. 168 (73%) patients had standard MM, with monoclonal Ig ≥ 3g/dl or BJP ≥ 500mg/24h. In the hyposecretory MM group, 35% of patients presented with International staging system (ISS) stage I vs. 13% in the standard MM group (P= 0.0001). IgG monoclonal Ig was present in 75% of patients with hyposecretory MM compared to 62% in the standard MM group (P=0.04). Mean plasma cell percentage in the bone marrow was similar in both groups, 40% in the hyposecretory MM group compared to 51% in standard MM. Average age of presentation was 64 yrs in the hyposecretory MM group as compared to 63 yrs in the standard MM group. There were more females than males in both groups, 61% females in hyposecretory MM and 53% in standard MM group. The most common presenting symptom was anemia in both groups and there were no statistically significant differences noted in gender, presence of hypercalcemia, presence of bone lesions, and renal dysfunction. Cytogenetic data was insufficient. Conclusion In our study, a substantial number of Afro-Caribbean patients with low levels of serum monoclonal Ig without the presence of Bence-Jones protein were diagnosed with active multiple myeloma. The results of our study underline the importance of an aggressive diagnostic approach with a bone marrow biopsy at initial presentation with MGUS in Afro-Caribbean population. This may prevent disease progression and end organ damage. Unlike the general population with MM, there were more females. Disclosures No relevant conflicts of interest to declare.


2021 ◽  
Author(s):  
Somnath Roy ◽  
Satvik Khaddar ◽  
Amit Agrawal ◽  
Geeta Rathnakumar ◽  
Lingaraj Nayak ◽  
...  

Abstract Multiple myeloma is a prototype of plasma cell dyscrasias characterized by monoclonal abnormal proliferation of immunoglobulin secreting plasma cell in the bone marrow ; resulting in production of monoclonal (M) protein (IgG,IgA,IgM,IgD) and or light chain concentrations (kappa or lamda) identified by protein electrophoresis and or immunofixation of serum or urine. The term biclonal multiple myeloma are defined by coexistence of two different M components, which could be either from a single clone or two separate clones producing two distinct bands in electrophoresis and or immunofixation of serum or urine. Biclonal gammopathy is a rare entity with upto 1% of newly diagnosed case of multiple myeloma have two M component in serum immunofixation electrophoresis. Here we share our experience of four cases of biclonal myeloma successfully diagnosed and treated with standard chemotherapy with satisfactory clinical outcome from a single tertiary care centre.


1997 ◽  
Vol 33 (1) ◽  
pp. 45-47 ◽  
Author(s):  
EN Peterson ◽  
AC Meininger

A nine-year-old golden retriever was referred for evaluation of chronic anorexia, vomiting, and diarrhea. Low body weight, mucous membrane pallor, and palpably enlarged liver and spleen were detected by physical examination. Anemia, hyperglobulinemia, and concurrent trichuriasis and coccidiosis were identified upon initial diagnostic evaluation. Punctate vertebral lysis was apparent radiographically. Atypical plasma cell proliferation was found in the bone marrow, liver, and spleen. An immunoglobulin A and immunoglobulin G biclonal gammopathy was demonstrated by serum protein electrophoresis and immunoelectrophoresis. The dog was diagnosed with multiple myeloma and euthanized per owner request. Multiple myeloma should be considered in the differential diagnosis for biclonal gammopathy in the dog.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4779-4779
Author(s):  
Harris V.K. Naina ◽  
Robert Kyle ◽  
Thomas M. Habermann ◽  
Samar Harris ◽  
Fernando G. Cosio ◽  
...  

Abstract Background: Monoclonal gammopathy of undetermined significance (MGUS) is reported in 3 to 5 percent of population, with the prevalence increasing with advancing age. Patients with MGUS are at increased risk for progression to multiple myeloma or other plasma cell dyscrasias. There is a paucity of information on clinical outcomes of patients with MGUS undergoing renal transplantation. A retrospective study was performed to determine wether MGUS is a contraindication to renal transplantation. Methods: Data was collected from both the kidney transplant and MGUS database. The diagnosis of MGUS was made on the basis of either serum protein electrophoresis (SPEP) or immunofixation after excluding multiple myeloma, amyloidosis and monoclonal immunoglobulin deposition disease. Results: Between 1977 and 2004, 3518 patients underwent kidney transplantation of whom 23 patients had a preexisting monoclonal gammopathy of undetermined significance (MGUS). Fourteen (61%) of these patients were males. The median age at the time of transplant was 59 ±12 years. Ten patients (43.5%) had IgG Kappa (GK), 7 (30.4%) had IgG Lambda (GL), 2 (8.7%) had IgA Lambda (AL), 1 (4.3%) had IgA Kappa (AK), 2 (8.7%) had IgM Lambda (ML). One patient had a biclonal gammopathy GL and ML. Patients were monitored with either SPEP or immunofixation for median duration of 1542 days after transplantation. Thirteen patients had either no change or stable monoclonal protein, 6 had a decrease in their paraprotein level. Two patients had a mild increase in their paraprotein. Two patients with GK developed into biclonal gammopathy (GK and AK). The median follow up of this cohort after the renal transplant was 1783 days. Twelve (52%) patients remained alive at the time of the study. A patient with GK prior to the transplant who underwent kidney transplantation twice developed a biclonal gammopathy and was found to have increased plasma cells (20%) in bone marrow after 14 years. On follow up for 6 years, his M-protein remained stable. Another patient was found to have 17% plasma cells around the time of kidney transplantation. He had a stable M-protein at follow-up, but underwent a stem cell transplant for recurrent immunotactoid glomerulonephritis. Two (9%) patients developed more than 15% plasma cells in their bone marrow with a stable M-protein. None of the patients with a preexisting MGUS evolved into multiple myeloma. Conclusion: In this small study, the presence of MGUS prior to kidney transplantation did not appear to have increased the incidence of multiple myeloma post transplant. Therefore, MGUS by itself should not be considered as an absolute contraindication for renal transplantation.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2933-2933
Author(s):  
Sarah Waheed ◽  
Christoph Heuck ◽  
Pingping Qu ◽  
Frits van Rhee ◽  
Saad Z Usmani ◽  
...  

Abstract Abstract 2933 Monoclonal gammopathy of undetermined significance (MGUS), a premalignant plasma cell dyscrasia, is differentiated from asymptomatic multiple myeloma (AMM), based on the level of monoclonal immunoglobulin (M spike) and bone marrow plasma cell infiltration (M spike > 3 g/dl and/or marrow plasmacytosis >10% in AMM). MGUS progresses to active Multiple Myeloma (MM) at a rate of 1–2% per year, thus imparting an average risk of 25% of progression over a lifetime. No single clinical variable, imaging modality or molecular study has yet been identified to predict progression. As a part of SWOG observational trial S0120, 262 patients were enrolled at MIRT and their GEP of purified plasma cells (PC) prospectively obtained. Here we report the results of GEP as predictor of progression. Among those with baseline GEP data available, 127 were non-IgM, AMM or MGUS and were otherwise eligible for the study. Baseline samples were restricted to those samples taken no more than 6 months prior to or 14 days following registration to S0120. If a patient had multiple baseline samples, then the sample taken closest to the patient's registration date was used. GEP studies were performed to assign molecular classification and risk scores, along with GEP-derived amp1q21, delTP53, DKK1 and other variables. With a follow-up of 36 months in the subset with evaluable GEP baseline data in 40 MGUS and 87 AMM cases, GEP characteristics were compared with those of untreated MM patients treated with TT2, TT3, TT4, and TT5 protocols, in the context of normal donor PC signatures. Comparing MGUS with AMM, CD-2 molecular subgroup was over-represented in MGUS and HY in AMM. PSMD4 on chromosome 1q21 was also linked to AMM. In terms of GEP-70 risk model, 36% of AMM and 15% of MGUS had scores exceeding -0.26. Univariate predictors for treatment-requiring MM included the well-known serum-M and bone marrow PC variables, and GEP-70 score >-0.26 (HR=8.82, p<0.001). Whole bone marrow biopsy (BMB) GEP samples from MGUS/AMM were available in 26 samples. Applying a BMB-65 model distinguishing MM-BMB from NL-BMB (normal donors) and MGUS/AMM-BMB, we observed that there was a strong trend for delayed MM progression when MGUS/AMM-BMB scores were in the top tertile (most NL-like). Independent validation data will be presented. Disclosures: Dhodapkar: Celgene: Research Funding; KHK: Research Funding.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2921-2921
Author(s):  
Andrew Stiff ◽  
Alberto Rocci ◽  
Craig C. Hofmeister ◽  
Paola Omedè ◽  
Susan Geyer ◽  
...  

Abstract Abstract 2921 Background: Multiple myeloma (MM) is a clonal B-cell malignancy characterized by the aberrant expansion of clonal plasma cells (PCs) within the bone marrow. Malignant PCs produce intact or partial monoclonal immunoglobulin (M protein) and cause organ damage. More than 20,000 new cases of multiple myeloma (MM) are diagnosed every year in the US with approximately 10,700 deaths occurring. The pathogenesis of MM is still largely unclear, but several reports suggest that interaction of tumor cells with the bone marrow microenvironment and microRNAs (miRNAs) deregulation may play a role in the etiology and progression of MM. miRNAs are small non-coding RNAs capable of regulating protein expression by binding to mRNA, and have been implicated in the development of MM. First identified inside cells, miRNAs can also be detected in body fluids, including serum and plasma, and may be a valid biomarker. Few studies have investigated the agreement between circulating miRNAs and intracellular myeloma PC miRNAs at diagnosis. Methods: Using Nano-String nCounter technology we first performed a screening analysis on serum samples obtained from MM patients and healthy controls. We identified a candidate set of miRNAs differentially expressed in the serum of MM patients. The levels of these miRNA markers were validated by RT-PCR in both serum and bone marrow PCs from the same cohort. Agreement of the quantitative miRNA marker levels between sample types was evaluated using intraclass correlation coefficients (ICC) (both for normalized and log2 measures). Results: Thirty-nine MM patients (21 male, 18 female) with a median age of 72 years (range: 65 – 83) were included in the analysis. Most were ISS stage I or II (59% vs. 41% ISS stage III) and 39% were high risk according to FISH abnormalities – 21% of patients carried del17p, 24% t(4;14) and 5% t(14;16). Medians and ranges for lab markers were as follows: hemoglobin 10.0 g/dl (7.2 to 15.1), beta2-microglobulin 5.18 ug/ml (1.38 – 12.1), creatinine 0.94 mg/dl (0.65 – 2.49), CRP = 1.6 mg/dl (0.02 – 116.0). Nine age-matched healthy controls were also used for the analysis. After the screening analysis, the following miRNAs were differentially expressed between healthy subjects and MM patients in serum samples: miR-92a, miR-451, miR-19b, miR-21, miR-16, miR-25, miR-30a, and miR-126. There was no significant agreement or correlation between serum and myeloma cell samples using either untransformed as well as log2measures (all p>0.40) (Table 1). Conclusion: Our preliminary results suggest a difference between circulating miRNAs in myeloma patients from controls. This indicates that future studies are needed to better define the role of miRNAs in the peripheral blood as a prognostic and even diagnostic biomarker in myeloma. From our preliminary data it also appears that circulating miRNAs are not simply secreted into the peripheral blood by myeloma PCs as it seems that circulating miRNAs do not reflect those of myeloma PCs. Differential expression could be determined by other cells that can release and or modify their miRNA expression in response to MM. Ongoing studies are examining the origin and function of miRNAs in the peripheral blood. Disclosures: No relevant conflicts of interest to declare.


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