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

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.

2021 ◽  
Vol 11 (5) ◽  
Author(s):  
Sæmundur Rögnvaldsson ◽  
Thorvardur Jon Love ◽  
Sigrun Thorsteinsdottir ◽  
Elín Ruth Reed ◽  
Jón Þórir Óskarsson ◽  
...  

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.


2011 ◽  
Vol 86 (4 suppl 1) ◽  
pp. 50-52 ◽  
Author(s):  
Aristóteles Rosmaninho ◽  
Iolanda Fernandes ◽  
Arlindo Guimas ◽  
Isabel Amorim ◽  
Manuela Selores

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


2002 ◽  
Vol 20 (6) ◽  
pp. 1625-1634 ◽  
Author(s):  
Clara Cesana ◽  
Catherine Klersy ◽  
Luciana Barbarano ◽  
Anna Maria Nosari ◽  
Monica Crugnola ◽  
...  

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.


Blood ◽  
2010 ◽  
Vol 116 (12) ◽  
pp. 2019-2025 ◽  
Author(s):  
Giada Bianchi ◽  
Robert A. Kyle ◽  
Colin L. Colby ◽  
Dirk R. Larson ◽  
Shaji Kumar ◽  
...  

Abstract Monoclonal gammopathy of undetermined significance (MGUS) is associated with a long-term risk of progression to multiple myeloma (MM) or related malignancy. To prevent serious myeloma-related complications, lifelong annual follow-up has been recommended, but its value is unknown. We reviewed all patients from southeastern Minnesota seen at Mayo Clinic between 1973 and 2004 with MGUS who subsequently progressed to MM. Of 116 patients, 69% had optimal follow-up of MGUS. Among these, abnormalities on serial follow-up laboratory testing led to the diagnosis of MM in 16%, whereas MM was diagnosed only after serious MM-related complications in 45%. In the remaining, workup of less serious symptoms (25%), incidental finding during workup of unrelated medical conditions (11%), and unknown (3%) were the mechanisms leading to MM diagnosis. High-risk MGUS patients (≥ 1.5 g/dL and/or non-IgG MGUS) were more likely to be optimally followed (81% vs 64%), and be diagnosed with MM secondary to serial follow-up testing (21% vs 7%). This retrospective study suggests that routine annual follow-up of MGUS may not be required in low-risk MGUS. Future studies are needed to replicate and expand our findings and to determine the optimal frequency of monitoring in higher-risk MGUS patients.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4755-4755
Author(s):  
Lucie Kovarova ◽  
Ivana Buresova ◽  
Ludek Pour ◽  
Renata Suska ◽  
Zdenek Adam ◽  
...  

Abstract Introduction: Multiple myeloma (MM) is a B-cell neoplasia caused by the proliferation of clonal plasma cells (PCs). MM and benign monoclonal gammopathy of unknown significance (MGUS) are routinely distinguished on the basis of paraprotein concentration, level of PCs infiltration and the presence or absence of other clinical features. Flowcytometric detection of PCs according to the expression of CD38 and CD138 has limitation in discrimination between normal and abnormal PCs, but this is possible in multicolor phenotypic analysis. The aim of this study is to compare the numbers of normal and abnormal PCs in MGUS and MM subjects and to find some parameter useful for evaluation PCs distribution. Materials and methods: 51 newly diagnosed untreated MM patients (63,9±10,0 years old) and 31 non-treated MGUS subjects (64,2±13,8 years old) were analysed. Lysed whole bone marrows were analysed by flowcytometric immunophenotyping and PCs were indentified by expression of CD38, CD138, CD45 and also CD56 and CD19. Results: Discrimination between normal CD19+ PCs and abnormal CD56+ PCs was done on CD38+CD138+ population. Ratio of normal PCs count and abnormal PCs counts (normal/abnormal=N/A) was used to describe a distribution of PCs. Subjects with MGUS had 0,97±1,65% (average±SD) of CD38+CD138+ cells (median 0,45; range 0,03–7,47%) with average N/A ratio 2,91±3,94 (median 1,36; range 0,01–18,44). Newly diagnosed MM patients had 4,96±9,94% of CD38+CD138+ cells (median 0,85; range 0,02–41,80%) with average N/A ratio 1,70±3,03 (median 0,13; range 0–11,5). In 9,7% MGUS subjects evaluation of distibution of PCs showed transformation of MGUS into MM. In some newly diagnosed MM patients (31,4%) CD38+CD138+ plasma cells were positive for CD19 although they were aberrant and these PCs were mostly CD45+. In some patients (9,7% of MGUS; 17,6% of MM) PC did express neither CD19 nor CD56 and this fact may complicate further evaluation of PCs. Conclusions: Results confirmed that in MGUS subjects we can find lower numbers of PCs which are mostly CD45+CD19+. A majority of plasma cells in newly diagnosed MM patients are abnormal CD56+ PCs and these plasma cells are usually CD45−. Further follow-up of patients can confirm the N/A ratio as a predictive factor for transformation of MGUS into MM and its value for evidence of relapse or progression to active myeloma.


2020 ◽  
Vol 26 (4) ◽  
pp. 40
Author(s):  
Helen Olugbeje ◽  
Arthur Fourcade

Introduction: Extramedullar plasmocytoma (EMP) is a plasma cell disease, such as solitary plasmocytoma of the bone and multiple myeloma. It is developed in the soft tissues. Observation: A 79-year-old patient consulted for a painful tumefaction of the gum associated to a radiolucent area on the right side of the mandible on the panoramic x-ray. After surgical enucleation, histological examination showed that the diagnosis was either an IgA extramedullar plasmocytoma or a multiple myeloma. The spreading assessment eliminated the diagnosis of multiple myeloma. The patient was then treated with radiotherapy. Discussion: EMP is a rare tumoral disease. Few cases described oral localisation. The main differential diagnosis, that must be excluded is multiple myeloma. Transformation in multiple myeloma exists. The first intention treatment is radiotherapy of the lesion. Conclusion: EMP of the oral cavity is very rare. After treatment, follow-up is required to detect second localisation or progression in multiple myeloma.


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5368-5368
Author(s):  
M Hasib Sidiqi ◽  
Mohammed A Aljama ◽  
David S. Viswanatha ◽  
David Dingli

Abstract T cell large granular lymphocytic (T-LGL) leukemia has been reported to occur in patients with plasma cell disorders (PCD). We conducted a retrospective review of patients diagnosed with T-LGL leukemia and a PCD at the Mayo Clinic. 22 patients were identified with T-LGL leukemia and a plasma cell disorder. The T-LGL leukemia preceded the PCD in 18% (n=4), was synchronous in 50% (n=11) and diagnosed post plasma cell disorder in 32% (n=7) of patients. The PCD diagnosis varied and included monoclonal gammopathy of undetermined significance (MGUS, n=13), multiple myeloma (MM, n=5), smoldering multiple myeloma (SMM. N=2), lymphoplasmacytic lymphoma (LPL, n=1) and monoclonal gammopathy of renal significance (MGRS, n=1). 5 patients developed T-LGL leukemia after treatment for a PCD (4 with MM and 1 with LPL). 4 patients with MGUS progressed to a more aggressive disease, 3 to MM and 1 to LPL. Neutropenia (76%) and anemia (70%) were the most common clinical presentation. None of the patients had rheumatoid arthritis. Treatment for the TLGL was variable with a number of different agents used listed in Table 1. 45% (n=10) of patients had an indolent course and did not receive specific therapy for TLGL. 6 patients responded to a single line of therapy, all of whom received either cyclophosphamide or methotrexate based regimens. The remainder had a relapsing course with multiple lines of therapy including 2 patients that received splenectomy. Nine patients were identified as having symptomatic multiple myeloma and TLGL, Table 2. Four patients had progressed from a preexisting plasma cell disorder, 3 with MGUS and 1 with SMM. The diagnosis of TLGL preceded myeloma in 1 patient was concurrent in 4 and post myeloma diagnosis in 4 patients. Time to diagnosis of TLGL post myeloma ranged from 10 to 63 months. At time of LGL diagnosis neutropenia was present in 7/9 patients and anemia in 6/8 (data unavailable for 1 patient). Cytogenetics data was available in 7 patients. Hyperdiploidy was the most common abnormality (3/7) followed by deletion 13q (2/7), t(14;16) in 1 patient and 1q amplification in 1 patient. The majority of patients were treated with novel agents with 7 receiving bortezomib based therapy. 3 patients underwent autologous stem cell transplantation. Therapy directed at the TLGL was given to 4/9 patients. This consisted of a combination of cyclophosphamide and prednisone in 3/4 patients all of whom responded to therapy with resolution of cytopenias. One patient had TLGL with multiple relapses and required multiple lines of therapy including eventual splenectomy. 3 patients with TLGL diagnosed after the diagnosis of myeloma did not receive specific therapy directed at the TLGL. The clinical course of the TLGL in these 3 patients was indolent and did not appear to be affected by therapy for multiple myeloma. At last follow up 5 patients have died. After a median follow up of 76 months post TLGL diagnosis the median overall survival (OS) post TLGL diagnosis was not reached for the entire cohort. In the cohort of patients with multiple myeloma, median OS from time of myeloma diagnosis was 71 months. Median OS from time of TLGL diagnosis was not reached. T-LGL leukemia can present in patients with a variety of plasma cell disorders and occur at any stage of the disease process. It is an important differential to consider in patients with unexplained cytopenias that are incongruent with the activity of the plasma cell disorder. Disclosures Dingli: Millennium Takeda: Research Funding; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.; Millennium Takeda: Research Funding.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4778-4778
Author(s):  
Harris V.K. Naina ◽  
Robert Kyle ◽  
Thomas M. Habermann ◽  
Samar Harris ◽  
Fernando G. Cosio ◽  
...  

Abstract Background: Post-transplant lymphoproliferative disorder (PTLD) represents one of the most serious consequences of immunosuppression in patients with solid organ transplantation. The incidence of PTLD is related to the organ transplanted and is dependent on the duration of follow-up. In various publications, the incidence of PTLD in renal transplantation ranges between 0.8% to 1.2%. In a previous study, the development of monoclonal (M) protein following liver transplantation is associated with the development of PTLD. In this study, we investigate this relationship in the kidney transplant population. Methods: A total of 3518 patients underwent kidney transplantation between 1963 to March 2006. These patients were cross referenced with the Monoclonal Gammopathy of Undetermined Significance (MGUS) database. Results: We identified 97 patients who had a monoclonal gammopathy either before or after transplantation. Patients with amyloidosis, multiple myeloma, heavy and light chain deposition disease and multi-organ transplantation were excluded from the analysis. A total of 69 patients met the inclusion criteria. Ten of the 69 (14.5%) patients developed PTLD. Median follow-up was 14.8 years. Twenty three patients had pretransplant MGUS, 20 patients developed MGUS following the transplant, and the other 26 did not have a monoclonal protein study prior to the transplant. Of the 23 patients who had a positive MGUS prior to the transplant, 4 patients (17.3%) developed PTLD, 1 patient developed EBV positive diffuse large cell lymphoma (DLCL), 1 developed EBV negative DLCL, 1 developed Hodgkin’s lymphoma and 1 developed increased plasma cells in bone marrow (20%) with stable M protein with no evidence of progression to multiple myeloma. None of these patients had a quantifiable M-protein prior to transplantation. The mean duration from diagnosis of MGUS to PTLD was 8.2 years (range 3 to 14 years). Of the 20 patients with a negative pre-transplant study for para proteniemia, 2 (10%) developed PTLD (T cell lymphoproliferative disorder). Two patients developed MGUS after the transplant at 1 and 12 years post transplant. It took an average of 15 years to develop PTLD after the diagnosis of MGUS. Four of the 26 patients who did not have a pretransplant study for MGUS developed PTLD. These included an EBV positive gamma delta type T cell lymphoproliferative disorder, an EBV positive plasmablastic lymphoma, one multiple myeloma and a plasmacytoma. The latter two patients had M-protein > 3g/dL. It took an average of 14 years after their transplant for these patients to develop PTLD. Conclusion: Our study showed that the development of a monoclonal protein in patients undergoing kidney transplantation is a strong risk factor for PTLD. Monoclonal protein study should be performed pretransplant and monitored post transplant as a surveillance of PTLD. Those who are positive or convert should be monitored closely for development of lymphoproliferative disorder.


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 ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5151-5151
Author(s):  
Olivier Decaux ◽  
Helene Leroy ◽  
Jean-Christophe Ianotto ◽  
Annie Ruelland ◽  
Lucienne Guenet ◽  
...  

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.


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