Clinical and Biological Features of Biclonal Gammopathies. Review of 203 Cases

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5151-5151
Author(s):  
Olivier Decaux ◽  
Helene Leroy ◽  
Jean-Christophe Ianotto ◽  
Annie Ruelland ◽  
Lucienne Guenet ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Malignant Transformation ◽  
Monoclonal Gammopathy ◽  
Systemic Disease ◽  
Light Chains ◽  
Lymphoproliferative Diseases ◽  
Monoclonal Gammopathies ◽  
Biclonal Gammopathy ◽  
Monoclonal Component

Abstract Introduction: Biclonal gammopathies represent about 5% of clonal gammopathies. We describe the clinical and laboratory features of biclonal gammopathies identified in a French university hospital. Methods: Patients were selected by immunofixation registry of Biochemistry laboratory. Results: From 1987 to 2008, 203 biclonal gammapathies were identified. Patients were 113 men and 90 women. Median age was 72.0 years (35–95). Seventy eight patients (38.3%) had IgG and IgM components, 64 (31,9%) had two IgG, 24 (11,8%) had IgG and IgA, 23 (11,3%) had two IgM, 8 (3,9%) had IgM and IgA, 5 (2,4%) had two IgA, one (0.5%) had Ig G and IgD (0,5%). Of the 406 light chains, 260 (63,8%) were kappa, 146 (36.2%) were lambda. Eighty nine patients (44.1%) had two kappa light chains, 82 (40,2%) had both kappa and lambda and 32 (15,7%) had two lambda chains. Median gammaglobulin concentration was 13.3 g/L (3–59.9). The most frequent diagnosis was biclonal gammopathy of undeterminated significance (BGUS) in 123 patients (60.6%). Others patients could be divided into 3 groups. Forty seven patients (23.1%) had lymphoproliferative diseases, including Waldenström’s macroglobulinemia (21 cases), non Hodgkin lymphoma (20), chronic lymphocytic leukaemia (6). Eighteen (8.87%) had multiple myeloma. For the last 15 patients (7.4%), biclonal gammopathy was associated with a non lymphoid hemopathy or with systemic disease. Biclonal gammopathy was identified in 12 patients already known to have a monoclonal gammopathy (7 monoclonal gammopathy of undetermined significance-MGUS, 3 myeloma and 2 Waldenström’s macroglobulinemia). In 3 cases, the finding of the second monoclonal component was concurrent to the diagnosis of a MGUS malignant transformation to myeloma (2 cases) or Waldenström’s macroglobulinemia (1 case). Median follow-up was 23 months (12 to 252 months) for the 123 patients with BGUS. In 4 cases (3.2%) a malignant transformation was observed. Three patients developed a multiple myeloma (time to transformation was 2 years for one and 4 years for the 2 others) and one patient Waldenström’s macroglobulinemia (6 years). Conclusions: As for monoclonal gammopathies, BGUS represent the most frequent diagnosis. However, in contrast to monoclonal gammopathies, biclonal gammopathies are more frequently associated with lymphoproliferative diseases than with multiple myeloma. The apparition of a second monoclonal component during follow up of MGUS could be associated with malignant transformation and should lead to new evaluation. The risk of transformation of BGUS seems similar to MGUS but further studies are necessary to compare the evolution of MGUS and BGUS.

scholarly journals Role of different hematologic variables in defining the risk of malignant transformation in monoclonal gammopathy

Blood ◽  
1996 ◽  
Vol 87 (3) ◽  
pp. 912-918 ◽  
Author(s):  
L Baldini ◽  
A Guffanti ◽  
BM Cesana ◽  
M Colombi ◽  
O Chiorboli ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Cox Model ◽  
Univariate Analysis ◽  
Cost Benefit ◽  
Cumulative Probability ◽  
Relative Risks ◽  
Monoclonal Component

The presenting clinico-hematologic features of 386 patients with nonmyelomatous monoclonal gammopathy (MG) were correlated with the frequency of malignant transformation to evaluate the most important variables conditioning its evolution into multiple myeloma (MM) or Waldenstrom macroglobulinemia (WM). Most of the patients (335) had monoclonal gammopathy of undetermined significance (MGUS: 39 IgA, 242 IgG, 54 IgM): the remaining 51 patients (12 IgA, 39 IgG) fulfilled all of the MGUS diagnostic criteria (according to Durie) except that bone marrow plasma cell (BMPC) content was 10% to 30%, and so they were defined as having monoclonal gammopathy of borderline significance (MGBS). There were no significant differences between the MGUS and MGBS groups in terms of age, sex, or median follow-up. After a median follow- up of 70 and 53 months, respectively, 23 of 335 MGUS and 19 of 51 MGBS patients had undergone a malignant evolution. Univariate analysis of the IgA and IgG patients showed that the cumulative probability of the disease evolving into MM correlated with diagnostic definition (MGBS v MGUS), BMPC content (> or = 10% v < 5% and < or = 5% v > 5%) and reduced serum polyclonal Ig. In the IgG cases, there was also a significant correlation with detectable Bence Jones proteinuria, serum monoclonal component (MC) levels and age at diagnosis (> 70 v < = or 55 years). In the IgG cases as a whole, the same variables remained in the Cox model where the BMPC percentage was considered after natural logarithmic transformation and the monoclonal component as g/dL value. The relative risks of developing MM are the following: 2.4 for each 1 g/dL increase of IgG, serum MC, 3.5 for detectable light chain proteinuria, 4.4 for the increase of 1 unit in log. BMPC percentage, 6.1 for age > 70, 3.6 and 13.1 for a reduction in one or two polyclonal Ig. In conclusion, our study allows the identification of a particular subset of MGUS patients (MC < = or 1.5 g/dL, BMPC < 5%, no reduction in polyclonal Ig and no detectable light chain proteinuria) at very low- risk of evolution, who can be considered as having benign monoclonal gammopathies. We also describe a previously undefined group of MG patients (with monoclonal gammopathy of borderline significance) who are at high-risk of malignant evolution. These findings could have a considerable impact on the cost/benefit ratio of monitoring programs in these patients.

Pre Transplantation MGUS and Transformation to Multiple Myeloma in Kidney Transplantation: A Single Center Experience.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4779-4779
Author(s):  
Harris V.K. Naina ◽  
Robert Kyle ◽  
Thomas M. Habermann ◽  
Samar Harris ◽  
Fernando G. Cosio ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Kidney Transplantation ◽  
Renal Transplantation ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
M Protein ◽  
Biclonal Gammopathy ◽  
Undetermined Significance

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.

scholarly journals Gamapatias Monoclonais de Significado Indeterminado: Critérios de Diagnóstico e Acompanhamento Clínico

2014 ◽  
Vol 27 (5) ◽  
pp. 661
Author(s):  
Joana Parreira ◽  
Paulo Lúcio ◽  
Cristina João ◽  
Ana Macedo ◽  
Ana Bela Sarmento ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multiple Myeloma ◽  
Clinical Management ◽  
Monoclonal Gammopathy ◽  
Long Term Care ◽  
Term Care ◽  
Monoclonal Gammopathies ◽  
Undetermined Significance

<p>The Portuguese group of multiple myeloma of the Portuguese Society of Hematology proposes a national protocol for diagnosis and clinical follow-up of monoclonal gammopathies. The proposed protocol aims to standardize clinical management of monoclonal gammopathies. Furthermore, it would also define the major risk factors for progression to Multiple Myeloma that require a precocious close articulation between general practitioners and a Hematology Clinic.</p><p><br /><strong>Keywords: </strong>Monoclonal Gammopathy of Undetermined Significance; Prognosis; Disease Progression; Long-Term Care; Portugal.</p>

scholarly journals Two Cases of Heavy Chain MGUS

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Jan Van Keer ◽  
Björn Meijers ◽  
Michel Delforge ◽  
Gregor Verhoef ◽  
Koen Poesen
Keyword(s):  
Multiple Myeloma ◽  
B Cell ◽  
Heavy Chain ◽  
Monoclonal Gammopathy ◽  
Rare Variants ◽  
Light Chains ◽  
B Cell Lymphomas ◽  
Heavy Chains ◽  
Heavy Chain Diseases

Heavy chain diseases are rare variants of B-cell lymphomas that produce one of three classes of immunoglobulin heavy chains, without corresponding light chains. We describe two patients with asymptomatic heavy chain monoclonal gammopathy. The first patient is a 51-year-old woman with alpha paraprotein on serum immunofixation. The second case is a 46-year-old woman with gamma paraprotein on urine immunofixation. Neither patient had corresponding monoclonal light chains. Workup for multiple myeloma and lymphoma was negative in both patients. These two cases illustrate that heavy chain monoclonal gammopathy can exist in the absence of clinically apparent malignancy. Only a few reports of “heavy chain MGUS” have been described before. In the absence of specialized guidelines, we suggest a similar follow-up as for MGUS, while taking into account the higher probability of progression to lymphoma than to myeloma.

Association Between Immunoparesis and a Skewed Free Light Chain (FLC) Ratio: A New Prognostic Factor Of Progression from MGUS To Multiple Myeloma?

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5314-5314
Author(s):  
Michele Pizzuti ◽  
Alberto Santagostino ◽  
Giuseppina Smaldore ◽  
Ida Chitarrelli ◽  
Domenico Vertone ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Conflicts Of Interest ◽  
Light Chains ◽  
Molecular Events ◽  
Risk Of Progression ◽  
Two Parameters

Abstract Monoclonal gammopathy of undetermined significance (MGUS) occurs in 3% of people older than 50 years and up to 10% in those older than 70; it is associated with a 1%/year risk of progression to Multiple Myeloma (MM). In recent years there have been improvements in risk stratification models (involving molecular markers) of this disorder, which have led to better understanding of the biology and probability of progression of MGUS. In the context of numerous molecular events and heterogeneous risk of progression, developing individualized risk profiles for patients with MGUS represents an ongoing challenge that has to be addressed by prospective clinical monitoring and extensive correlative science. Free Lights Chains (FLC) ratio, plasma cells immunophenotype and DNA aneuplody are now important parameters of progression, in addition to the already known prognostic factors (immunoparesis, type and amount of the monoclonal component (MC). Recent data report immunoparesis and a skewed FLC ratio in 25% and 30%, respectively, of patients (pts) at diagnosis. In this study we evaluated the incidence of these two parameters in a cohort of 114 pts with MGUS, if they are associated and if their incidence is influenced by other parameters (time from diagnosis, type of Immunoglobulin (Ig) and/or light chains). The patients screened were 56 males and 58 females with a median age of 67 years (45-91). Median time from diagnosis to the time of observation was 3 years (0-21). The MC was IgA in 13 pts, IgG in 88, IgM in 13; 74 had a clonal Kappa (K) and 40 a lambda (L) light chain. K/L ratio was abnormal in 57 pts (50%). Immunoparesis was present in 60 pts (52,6%): 22 with a normal K/L ratio (38,5%) and 38 with an abnormal K/L ratio (66,6%) (p-0.004). In 18 pts two classes of Ig were involved. An association between the two parameters occurred in 39 pts (34,2%); it was more frequent in IgA MGUS (61,5%) than in IgG (31,8%) and IgM (23%); we did not observe any differences about immunoparesis between K MGUS (33,7%) and L MGUS (32,5%). The association between a skewed K/L ratio and immunoparesis was present in 25.4% of pts with time from diagnosis of less than 3 years and in 48,8% of pts with a longer time from diagnosis (p-0.04). Our new data confirm that immunoparesis is more frequent in pts with an abnormal K/L ratio. The association seems to be more frequent in case of IgA gammopathy; there are no differences between the two types of light chain. Our data also confirm that the longer is the time elapsed from diagnosis, the higher are the frequency of an abnormal K/L ratio and the incidence of immunoparesis, with a greater probability of association. We need still a larger number of pts with an adequate follow up to evaluate if the association between immunoparesis and abnormal K/L ratio has a prognostic value, although the higher frequency of association in the subset of pts with a longer time from diagnosis seems to contradict this hypothesis. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Iceland screens, treats, or prevents multiple myeloma (iStopMM): a population-based screening study for monoclonal gammopathy of undetermined significance and randomized controlled trial of follow-up strategies

2021 ◽  
Vol 11 (5) ◽  
Author(s):  
Sæmundur Rögnvaldsson ◽  
Thorvardur Jon Love ◽  
Sigrun Thorsteinsdottir ◽  
Elín Ruth Reed ◽  
Jón Þórir Óskarsson ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Early Treatment ◽  
Monoclonal Gammopathy ◽  
Controlled Trial ◽  
Participation Rate ◽  
Population Based ◽  
Early Therapy ◽  
Screening Study ◽  
Undetermined Significance

AbstractMonoclonal gammopathy of undetermined significance (MGUS) precedes multiple myeloma (MM). Population-based screening for MGUS could identify candidates for early treatment in MM. Here we describe the Iceland Screens, Treats, or Prevents Multiple Myeloma study (iStopMM), the first population-based screening study for MGUS including a randomized trial of follow-up strategies. Icelandic residents born before 1976 were offered participation. Blood samples are collected alongside blood sampling in the Icelandic healthcare system. Participants with MGUS are randomized to three study arms. Arm 1 is not contacted, arm 2 follows current guidelines, and arm 3 follows a more intensive strategy. Participants who progress are offered early treatment. Samples are collected longitudinally from arms 2 and 3 for the study biobank. All participants repeatedly answer questionnaires on various exposures and outcomes including quality of life and psychiatric health. National registries on health are cross-linked to all participants. Of the 148,704 individuals in the target population, 80 759 (54.3%) provided informed consent for participation. With a very high participation rate, the data from the iStopMM study will answer important questions on MGUS, including potentials harms and benefits of screening. The study can lead to a paradigm shift in MM therapy towards screening and early therapy.

scholarly journals Diffuse plane xanthomatosis associated with monoclonal gammopathy

2011 ◽  
Vol 86 (4 suppl 1) ◽  
pp. 50-52 ◽  
Author(s):  
Aristóteles Rosmaninho ◽  
Iolanda Fernandes ◽  
Arlindo Guimas ◽  
Isabel Amorim ◽  
Manuela Selores
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Underlying Disease ◽  
Systemic Diseases ◽  
Inherited Disease ◽  
Hematological Diseases ◽  
Laboratory Examinations

Diffuse plane normolipemic xanthomatosis (DPNX) is a rare, non-inherited disease that is often associated with systemic diseases, mainly malignant hematological (especially multiple myeloma) or lymph proliferative disorders. The DPNX can precede the appearance of such conditions by several years, so careful follow-up and periodic laboratory examinations are recommended even for patients that seemed to have no underlying disease. We describe a case associated with monoclonal gammopathy. This case shows that dermatological lesions can be the first manifestation of important hematological diseases and so physicians should be familiarized with this entity

Prognostic Factors for Malignant Transformation in Monoclonal Gammopathy of Undetermined Significance and Smoldering Multiple Myeloma

2002 ◽  
Vol 20 (6) ◽  
pp. 1625-1634 ◽  
Author(s):  
Clara Cesana ◽  
Catherine Klersy ◽  
Luciana Barbarano ◽  
Anna Maria Nosari ◽  
Monica Crugnola ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Extramedullary Plasmacytoma ◽  
Lymphocytic Leukemia ◽  
Cumulative Probability ◽  
Primary Amyloidosis ◽  
Smoldering Multiple Myeloma ◽  
Bence Jones Proteinuria ◽  
Undetermined Significance

PURPOSE: To evaluate the natural history of monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM), identify early predictors of evolution, and assess whether associated conditions correlate with disease progression. PATIENTS AND METHODS: A total of 1,231 consecutive patients with either MGUS (n = 1,104) or SMM (n = 127) diagnosed from July 1975 to March 1998 were included in the study. Cumulative survival probability and cumulative probability of transformation into lymphoproliferative disease were calculated by means of the Kaplan-Meier estimator. Univariate and multivariate Cox models were used to identify possible predictors of malignant evolution. RESULTS: Cumulative transformation probability at 10 and 15 years was 14% and 30%, respectively. At a median follow-up of 65 months (range, 12 to 239 months), 64 MGUS cases (5.8%) evolved to multiple myeloma (MM) (n = 43), extramedullary plasmacytoma (n = 1), primary amyloidosis (n = 1), Waldenström’s macroglobulinemia (n = 12), non-Hodgkin’s lymphoma (n = 6), and B-chronic lymphocytic leukemia (n = 1). At a median follow-up of 72 months (range, 12 to 247 months), 25 SMMs (19.7%) evolved to overt MM. A lower evolution risk was observed in MGUS than in SMM (P < .0001). Greater than 5% marrow plasmacytosis, detectable Bence Jones proteinuria, polyclonal serum immunoglobulin reduction, and high erythrocyte sedimentation rate (ESR) were independent factors influencing MGUS transformation. SMM progression correlated with greater than 10% marrow plasma cells, detectable Bence Jones proteinuria, and immunoglobulin (Ig) A isotype. Neither concomitant diseases nor immunosuppression correlated with progression. CONCLUSION: Careful evaluation of marrow plasmacytosis, urinary paraprotein, background immunoglobulins, ESR, and paraprotein isotype might help identify at presentation patients with benign monoclonal gammopathies requiring stricter monitoring.

scholarly journals Primary pulmonary plasmacytoma presenting with rare IgG lambda monoclonal gammopathy

2019 ◽  
Vol 12 (3) ◽  
pp. e227514
Author(s):  
Yasmin Rahim ◽  
Farrukh Zia Tareen ◽  
Rashida Ahmed ◽  
Javaid Ahmed Khan
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
Considerable Decrease ◽  
Disease Extent ◽  
Local Infiltration ◽  
The Right ◽  
Serum Igg Levels ◽  
Routine Chest ◽  
Lung Zone

Extramedullaryplasmacytoma (EMP) represents a peculiar and typically progressive malignancy that can originate outside the bone marrow. Primary pulmonary plasmacytoma (PPP) is a rare subset of EMP, confined to the lung. A 55-year-old man, diabetic, non-smoker presented to our clinic with a right chest wall swelling. A routine chest radiograph showed a well-circumscribed opacity in the right upper lung zone. A CT of the chest revealed a large right upper lobe mass with extensive local infiltration. Biopsy and immunohistochemical evaluation led to a diagnosis of PPP. Screening for multiple myeloma was negative. Serum immunofixation showed an IgG lambda monoclonal gammopathy, found in a minority of PPP patients. In view of disease extent, treatment with chemotherapy and radiotherapy was initiated. The patient is currently in out patient follow-up and has shown a favourable response to the treatment with a considerable decrease in serum IgG levels.

Multiple myeloma with serum IgM kappa and Bence Jones lambda biclonal gammopathy.

1986 ◽  
Vol 32 (12) ◽  
pp. 2220-2221 ◽  
Author(s):  
M S Graziani ◽  
U Lippi
Keyword(s):  
Multiple Myeloma ◽  
Erythrocyte Sedimentation Rate ◽  
Sedimentation Rate ◽  
Light Chains ◽  
Laboratory Studies ◽  
Rare Finding ◽  
Erythrocyte Sedimentation ◽  
Biclonal Gammopathy ◽  
High Erythrocyte Sedimentation Rate

Abstract We report a rare finding: IgM kappa and Bence Jones lambda double gammopathy in serum of a 80-year-old man with untreated symptomatic multiple myeloma. The unusual findings are confined to the laboratory studies demonstrating also a Bence Jones lambda proteinuria, high erythrocyte sedimentation rate (113 mm/h), and anemia. The synthesis of the different light chains seems to occur in separate cellular clones.

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