t(11;14) Plasma Cell Disorder Presents as a True Nonsecretory, Nonproducer Multiple Myeloma

2009 ◽  
Vol 9 (3) ◽  
pp. 243-246 ◽  
Author(s):  
Wei Chen ◽  
Mark McNamara ◽  
Young Kim ◽  
Qin Huang
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cell Disorder ◽  
Cell Disorder

scholarly journals Monoclonal gammopathies of undetermined significance and smoldering myeloma

2020 ◽  
Vol 51 (4) ◽  
pp. 193-202
Author(s):  
Artur Jurczyszyn ◽  
Ruth Hutch ◽  
Anna Waszczuk-Gajda ◽  
Anna Suska ◽  
Katarzyna Krzanowska ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Standard Of Care ◽  
Current Standard ◽  
Malignant Plasma Cell ◽  
Plasma Cell Disorder ◽  
Smoldering Myeloma ◽  
Cell Disorder ◽  
Undetermined Significance

AbstractMonoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder implicated as a precursor of multiple myeloma (MM), while smoldering multiple myeloma (SMM) is a malignant plasma cell disorder without evidence of a myeloma-defining event(s) (MDE). This is a review article of both disorders outlining their current definition and management according to the current standard of care. We focus on the pathogenesis of MM and the role of MGUS and SMM in the development of active MM. MGUS is a benign disorder and, subsequently, is followed by observation. In contrast, for SMM, although the current standard of care is “watch and wait”, this paper will explore the circumstances in which treatment should be considered to prevent MDE.

T Cell Large Granular Lymphocytic Leukemia and Co-Existing Plasma Cell Disorders

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5368-5368
Author(s):  
M Hasib Sidiqi ◽  
Mohammed A Aljama ◽  
David S. Viswanatha ◽  
David Dingli
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Mayo Clinic ◽  
Monoclonal Gammopathy ◽  
Specific Therapy ◽  
Plasma Cell Disorders ◽  
Plasma Cell Disorder ◽  
Cell Disorder ◽  
Lgl Leukemia

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.

A Pre-Existing Plasma Cell Disorder Occurs in Most Patients with Multiple Myeloma.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1693-1693 ◽  
Author(s):  
Brendan M Weiss ◽  
Pramvir Verma ◽  
Jude Abadie ◽  
Robin Howard ◽  
Michael Kuehl
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Light Chain ◽  
Medical Center ◽  
Walter Reed Army Medical ◽  
Cell Transplant ◽  
Serum Samples ◽  
Published Evidence ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Abstract Background. A pre-existing plasma cell disorder (PPCD), such as monoclonal gammopathy of undetermined significance (MGUS), is thought to be present in at least one-third of patients presenting with symptomatic multiple myeloma (MM). However, no study has comprehensively evaluated the proportion of patients with MM that had a PPCD by laboratory testing on pre-diagnostic sera. Methods. The Walter Reed Army Medical Center autologous stem cell transplant database was cross-referenced with the Department of Defense Serum Repository (DoDSR) database, which catalogs serum samples collected every 2 years on over 4 million active-duty service members. All samples 32 years prior to the diagnosis of MM were retrieved. Serum protein electrophoresis (SPEP), immunofixation electrophoresis (IFE) and serum free light chain analysis (sFLC) (The Binding Site, San Diego, CA) were performed on all samples. A PPCD was defined as a positive SPEP, IFE or abnormal sFLC ratio. Results. Serum samples prior to the diagnosis of MM were available for 30/90 patients, and the median number of samples per patient was 3.5 (range, 1–14). The median age at diagnosis of MM was 48.1 yrs (29–67), with 96% male, 53% Caucasian, and 47% African-American. The Ig isotype of MM was IgG 76%, IgD 10%, light-chain 7%, and non-secretory 7%. A PPCD was detected in 27/30 patients (90%, 95% CI 74–97%). The initial PPCD was detected by sFLC alone in 6/27 (22.2%), IFE alone 2/27 (7.4%), SPEP+IFE 5/27 (18.5%), SPEP+IFE+sFLC 13/27 (48.1%) and IFE+sFLC 1/27 (3.7%). There were 4 patients whose only positive sera was 2.5–3.5 years prior to diagnosis, with all preceding sera negative. Conclusions. First, a pre-existing plasma cell disorder is present in most MM patients at least 2.5 years prior to diagnosis. Second, consistent with published evidence for a small fraction of patients with high risk MGUS, 4/30 patients were documented to progress rapidly through an MGUS phase to MM. Third, 4/4 patients with light chain only or non-secretory MM had a PPCD that was detected only by sFLC, thereby indicating that all these tumors are preceded by a light chain only or non-secretory PPCD.

IgM Multiple Myeloma: Disease Definition, Prognosis, and Differentiation From Waldenstrom's Macroglobulinemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1828-1828 ◽  
Author(s):  
Steven Schuster ◽  
Angela Dispenzieri ◽  
S. Vincent Rajkumar ◽  
Alvaro Moreno Aspitia ◽  
Robert Kyle ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Median Overall Survival ◽  
Bone Lesions ◽  
Lytic Lesions ◽  
Lytic Bone Lesions ◽  
Plasma Cell Disorder ◽  
Definition Of ◽  
Cell Disorder

Abstract Abstract 1828 Poster Board I-854 Background IgM Multiple Myeloma (MM) and Waldenstrom's Macroglobulinemia (WM) are two hematologic diagnoses with the common variable of an IgM monoclonal gammopathy. Distinguishing these two diagnoses is critical as the approach to therapy is different. A study by Avet-Loiseau et al demonstrated the presence of t(11;14) translocations in 7 of 8 patients with IgM MM that was absent in all of a series of 17 cases of WM (Semin Oncol 2003 30:2;153). However, 6 of the 8 IgM MM cases lacked classic lytic lesions. Method A priori, based on the literature and the natural history of MM, we defined IgM MM as a symptomatic clonal plasma cell proliferative disorder characterized by a serum IgM monoclonal protein (regardless of size) plus presence of t(11;14) on fluorescent in situ hybridization (FISH) and/or lytic bone lesions felt to be related to the underlying plasma cell disorder. The cases for the study were screened by a computerized database search for ‘IgM’ and ‘Myeloma’ of all patients seen at Mayo Clinic in the last 30 years at all three sites (Rochester, Arizona and Florida). Patients identified on the computerized screen were then audited by chart review to identify the study cohort. Results 38 cases were identified on initial screen of the computerized database as potential patients with IgM MM. Of these, a total of 22 cases met our specific definition of IgM myeloma (t(11;14 and/or lytic lesions). Of the remaining 16 cases, 8 were IgM MGUS, 5 were WM based on clinical presentation (hyperviscosity, lymphadenopathy and organomegaly) and biopsy findings of lymphoplasmacytic lymphoma, 1 was excluded due to lack of information, and the remaining 2 patients were indeed considered to have clinical IgM MM. Interestingly, these two patients did not have either the t(11;14) or lytic lesions, but rather had immunophenotypic features suggestive of MM and not WM (CD138+, CD20-). Table 1 summarizes the clinical characteristics of the 22 patients who met our criteria for IgM MM. All 22 patients had lytic bone lesions. Of the 17 evaluated with FISH, 6 (35%) demonstrated the t(11;14) abnormality. Median overall survival by Kaplan-Meier analysis was 37 months represented in Figure 1. Conclusion IgM MM is a discrete clinical entity that can and should be distinguished from WM. Our definition of IgM MM is designed to be specific and requires the presence of a symptomatic IgM secreting plasma cell proliferative disorder plus presence of t(11;14) and/or lytic bone lesions felt related to the underlying plasma cell disorder. In this, the largest series of patients with IgM MM, the t(11;14) abnormality is very specific for IgM MM, but may not be sensitive, being present in approximately 1/3 of patients. The median overall survival is similar to non-IgM myeloma patients treated during this period, and much shorter than what would be expected for WM. The minority of symptomatic patients with IgM monoclonal gammopathy who do not meet this criteria, but have immunophenotypic features more suggestive of MM rather than WM need further study. Disclosures No relevant conflicts of interest to declare.

scholarly journals A Case with Hepatic Involvement Mimicking Metastatic Disease in Multiple Myeloma

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Mert Erciyestepe ◽  
Tarık Onur Tiryaki ◽  
İpek Yönal Hindilerden ◽  
Gülçin Yeğen ◽  
Meliha Nalçacı
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Metastatic Disease ◽  
Current Knowledge ◽  
Primary Amyloidosis ◽  
Hepatic Involvement ◽  
Malignant Plasma Cell ◽  
Risk Of Progression ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Multiple myeloma is a type of plasma cell disorder and can be seen in different forms. According to current knowledge, it is not a curable disease. Smoldering multiple myeloma (SMM) is an asymptomatic clonal plasma cell disorder and distinguished from monoclonal gammopathy of undetermined significance by a much higher risk of progression to multiple myeloma. We present a 53-year-old female patient who started with SMM which turned into multiple myeloma after four years. Despite 26 cycles of lenalidomide treatment, we performed the second autologous stem transplantation. After 12 years from the diagnosis of the disease, it was transformed into plasma cell leukemia and widespread nodular lesions were seen in the liver. Different presentations could be seen due to malignant plasma cell infiltrations or primary amyloidosis. Liver involvement is one of them and is less common than other organ involvement. We report a case of myeloma presenting with extensive nodular involvement in the liver and misdiagnosed as metastatic disease. It is important because of its rarity and change of the treatment approach.

scholarly journals Multiple Myeloma and Diabetes

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Zeinab A. Issa ◽  
Mira S. Zantout ◽  
Sami T. Azar
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Multiple Myeloma ◽  
Bone Marrow ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Malignant Plasma Cell ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Multiple myeloma is a malignant plasma cell disorder that accounts for approximately 10% of all hematologic cancers. It is characterized by accumulation of clonal plasma cells, predominantly in the bone marrow. The prevalence of type 2 diabetes is increasing; therefore, it is expected that there will be an increase in the diagnosis of multiple myeloma with concomitant diabetes mellitus. The treatment of multiple myeloma and diabetes mellitus is multifaceted. The coexistence of the two conditions in a patient forms a major challenge for physicians.

scholarly journals Diagnosis and Management of Monoclonal Gammopathy and Smoldering Multiple Myeloma

2020 ◽  
Vol 18 (12) ◽  
pp. 1720-1729
Author(s):  
Timothy M. Schmidt ◽  
Natalie S. Callander
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Treatment Guidelines ◽  
The United States ◽  
Smoldering Multiple Myeloma ◽  
Risk Of Progression ◽  
Plasma Cell Disorder ◽  
Monoclonal Proteins ◽  
Cell Disorder

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.

scholarly journals 2021 European Myeloma Network review and consensus statement on smoldering multiple myeloma: how to distinguish (and manage) Dr. Jekyll and Mr. Hyde

Haematologica ◽  
2021 ◽  
Author(s):  
Pellegrino Musto ◽  
Monika Engelhardt ◽  
Jo Caers ◽  
Niccolo’ Bolli ◽  
Martin Kaiser ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Plasma Cell ◽  
Working Group ◽  
Consensus Statement ◽  
Smoldering Multiple Myeloma ◽  
Bone Marrow Plasma ◽  
Plasma Cell Disorder ◽  
Cell Disorder ◽  
International Myeloma Working Group

According to the updated International Myeloma Working Group criteria, smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder characterized by an M-component >3 g/dL, bone marrow plasma cell infiltration >10% and

Biology of the Transition of Monoclonal Gammopathy of Undetermined Significance (MGUS) to Multiple Myeloma

1998 ◽  
Vol 5 (3) ◽  
pp. 209-217 ◽  
Author(s):  
John A. Lust ◽  
Kathleen A. Donovan
Keyword(s):  
Multiple Myeloma ◽  
Adhesion Molecules ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Therapeutic Strategies ◽  
Plasma Cell Disorder ◽  
Novel Therapeutic Strategies ◽  
Cell Disorder ◽  
Undetermined Significance

Background Approximately 25% of patients with monoclonal gammopathy of undetermined significance (MGUS) eventually develop multiple myeloma (MM) or a related plasma cell disorder that is universally fatal. In this report, we examine the changes that occur in the clonal plasma cell that are likely to be important in the progression of MGUS to active myeloma. Methods Studies that investigate the mechanisms involved in the multistep pathogenesis of monoclonal gammopathies are reviewed. Cytokines such as IL- 6 and IL-1β, adhesion molecules, viruses, and oncogenes including ras, bcl-2, Rb, and p53 are discussed. Results IL-1β is produced by plasma cells from virtually all MM patients but is undetectable in most MGUS patients. IL-1β has potent osteoclast activating factor activity, can increase the expression of adhesion molecules, and can induce paracrine IL-6 production. The increased production of adhesion molecules could explain why myeloma cells are found predominantly in the bone marrow. Subsequently, these “fixed” monoclonal plasma cells could now stimulate osteoclasts through the production of IL-1β and paracrine generation of IL-6 resulting in osteolytic disease. With continued progression of the myeloma, the monoclonal plasma cells may later acquire the ability to produce IL-6 in an autocrine fashion that will be manifested clinically by an elevated labeling index. Conclusions A better understanding of the progression of MGUS to myeloma may lead to novel therapeutic strategies to prevent the development of MM.

scholarly journals Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case

2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Sassine Ghanem ◽  
Gwenalyn Garcia ◽  
Liu Ying ◽  
Matthew Hurford ◽  
Marcel Odaimi
Keyword(s):  
Multiple Myeloma ◽  
Organ Dysfunction ◽  
Plasma Cell ◽  
Systemic Mastocytosis ◽  
The Body ◽  
World Health ◽  
Management Options ◽  
Smoldering Multiple Myeloma ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body. It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD). Over 80% of SM-AHNMD cases involve disorders of the myeloid cell lines. To our knowledge, there are only 8 reported cases to date of SM associated with a plasma cell disorder. We report a patient with ISM who was found to have concomitant smoldering multiple myeloma. His disease later progressed to ASM. We discuss this rare association between SM and a plasma cell disorder, and potential common pathophysiologic mechanisms linking the two disorders will be reviewed. We also discuss prognostic factors in SM as well as the management options considered during the evolution of the patient’s disease.

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