Mar 31, 2015 the authors searched the mayo clinic multiple myeloma database to identify nonsecretory myeloma patients diagnosed between january 1973 and june 2012. Patients with non secretory myeloma had a trend for longer survival 5 year os 68% vs 48% for the. Successful treatment of central nervous system myeloma. Nonsecretory multiple myeloma blood american society. For recipients with non secretory myeloma, all of the following criteria must be met. The emergence of nonsecretory multiple myeloma during the. Nonsecretory multiple myeloma nsmm accounts for 1 5% of all multiple myeloma mm cases. Myeloma cells produce an antibody known as paraprotein. Cytogenetic features and clinical outcomes of patients with non. Imwg criteria for the diagnosis of myeloma and guidelines for the diagnostic workup of myeloma criteria for a diagnosis of myeloma followed by the examinations, tests, and imaging studies that are recommended by the working group for the diagnosis of myeloma. We present the case of a 39yearold patient who was diagnosed with non secretory myeloma.
The study revealed that there was a preponderance of the t 11. Smouldering myeloma an overview sciencedirect topics. Patients were identified as having nonsecretory myeloma if their serum and urine immunofixation did not show any monoclonal protein at diagnosis and at all subsequent checkups. However, a small subset of mm called nonsecretory multiple myeloma nsmm produces no detectable ig. Non secretory multiple myeloma nsmm is a rare variant of the classic form of multiple myeloma in which no monoclonal gammopathy can be demonstrated in the serum or urine. The subset of these patients with non secretory myeloma a group of patients who do not secrete immunoglobulin or its component parts into either the blood or urinehas. Most reported cases of nsmm show the presence of lytic lesions on skeletal radiography. The percentage of plasma cells in the bone marrow aspirate and or biopsy may also. A case of nonsecretory multiple myeloma is described. Survival of nonsecretory multiple myeloma patients improves. Papanikolaou x, zhang q, heuck c, waheed s, atrash s, singh z et al. Nonsecretory multiple myeloma presenting as primary plasma.
Demonstration of serum monoclonal immunoglobulin in a case of non secretory myeloma. Nonsecretory myeloma or light chainproducing multiple. A rare case of nonsecretory multiple myeloma in lagos. Clinical lymphoma myeloma and leukemia vol 20, issue 1. Microabstractwe report the outcomes of patients with nonsecretory multiple myeloma nsm who were treated with autologous hematopoietic stem cell transplantation autohct compared with a matched cohort with secretory myeloma. My cohost for the talk is myeloma advocate and editor of, gary petersen.
However, a small subset of mm called non secretory multiple myeloma nsmm produces no. Non secretory multiple myeloma involving the maxilla. Nonsecretory multiple myeloma nsmm is the absence of a detectable monoclonal protein in serum and urine of a multiple myeloma mm patient and immunoglobulin light chain al amyloidosis is a. Discrepancies between the percentage of plasma cells in. However, a small subset of mm called non secretory multiple myeloma. Once you have been diagnosed with myeloma, doctors will regularly check your paraprotein levels to monitor the disease. Cytogenetic features and clinical outcomes of patients. International myeloma working group guidelines for serum. Diagnosing mm and monitoring treatment response, including eventual relapse, are largely based on sequential measurements of ig. Nonsecretory myeloma, diagnosed on renal biopsy as cast. A case of nsmm with al amyloidosis and nephrotic range proteinuria is presented. We present the case of a 39yearold patient who was diagnosed with non secretory myeloma with double autologous transplantation and underwent neurosurgery for spinal cord compression caused by a plasmocytoma at d5 level.
Information about serum flc measurement in nonsecretory myeloma, monoclonal light chain diseases, and reference populations is reported in the literature 15,7, but there is limited data about flcs in renal disease, monoclonal gammopathy of undetermined significance mgus, and myeloma patients after autologous stem cell transplants. The subset of these patients with non secretory myeloma. Use the multiple myeloma response criteria when determining the disease status for multiple myeloma. Bone marrow examination may be the only method of diagnosis in such cases. Nonsecretory multiple myeloma nsmm is the absence of a detectable monoclonal protein in serum and urine of a multiple myeloma mm patient and immunoglobulin light chain al amyloidosis is a significantly rare complication. Microabstractnon secretory multiple myeloma is a rare form of multiple myeloma characterized by the absence of a monoclonal protein in serum and urine. Multiple myeloma mm is a plasma cell disorder associated with clonal proliferation of plasma cells. Non producing multiple myeloma mm is a distinct subset of non secretory mm characterized by high cyclin d1 experession and decreased progression free survival. The clinical picture of nsm is different from that of secretory myeloma, as nsm patients usually have improved hemoglobin levels, lower esr values, higher neurological presentation incidence, less aggressive. Laboratory analysis showed 20% of circulating immature plasma cells. Patients with multiple myeloma mm that present at diagnosis with serum monoclonal protein mig of non secretory multiple myeloma nsmm is a rare variant of the classic form of multiple myeloma mm.
Non secretory myeloma an overview sciencedirect topics. After presenting with multiple plasmacytomas which led to a diagnosis of non secretory myeloma at age 56, the patient underwent multiple episodes of treatment for relapsing myeloma. A case of nonsecretory multiple myeloma with careful study of urine and serum immunoglobulins is presented. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. It is defined as monoclonal plasmocytic proliferation of the bone marrow with the same clinical and radiological manifestations of myeloma. Imwg criteria for the diagnosis of myeloma and guidelines. Nonsecretory multiple myeloma nsmm is a rare subtype of multiple myeloma mm characterized by the absence of monoclonal protein in. International uniform response criteria for multiple myeloma. Nonsecretory multiple myeloma presenting with diffuse. The disease is characterised by the absence of detectable monoclonal proteins in serum and urine using immunofixation electrophoresis ife, although the degree of bone marrow plasma cell infiltration is similar to that found in secretory patients. This variant usually poses a diagnostic challenge to. A case involving the maxillary tuberosity is presented along with an update on current understanding of myeloma biology, recent insights which provide, for the first time, hope of significant longer term survival. Dec 15, 2016 it is typically characterized by production of large amounts of defective immunoglobulin ig. Non secretory multiple myeloma nsmm is a rare variant of the classic form of multiple myeloma mm.
Nonsecretory myeloma, which comprises 15% of all myelomas, is a variant of plasma cell myeloma. Joining gary on the panel is myeloma advocate, jack aiello. Theses recommendations are excerpted from palumbo a et. Nonsecretory multiple myeloma nsmm is a rare clinical and biological variant of the classic form of mm characterized by the absence of monoclonal. The therapeutic approaches for nonsecretory myeloma are similar to those for symptomatic pcm. Diagnostic pitfalls of nonsecretory myeloma manifesting as multiple. The treatment of patients with multiple myeloma has dramatically changed over the past 10 years due to an improved understanding of plasma cell biology and the development of new targets. This is why autologous stem cell transplantation is included in the standard treatment of myeloma patients. The clinical picture of nsm is different from that of secretory myeloma. Comparable outcomes in nonsecretory and secretory multiple myeloma. Sep 11, 20 repeated hematological examinations, including bone biopsy with immunohistochemistry, disclosed diagnosis of multiple myeloma of the non secretory type. Multiple myeloma mm is a plasma cell neoplasm featuring symptoms of hypercalcemia, renal failure, anemia, and lytic bone lesions that comprises approximately % of all hematologic.
In nsmm, no monoclonal gammopathy can be detected in serum or urine by conventional techniques. Autologous stem cell transplantation in multiple myeloma. Marrow immunofluorescent studies failed to show production of identifiable immunoglobulins. Nonsecretory multiple myeloma blood american society of. At any response level, if some but not all criteria are met, the disease status should be downgraded to next lower level of response. Nonsecretory multiple myeloma and al amyloidosis presenting. An attempt is made to explain the absence of pathological proteins in the serum, based on the. Survival of nonsecretory multiple myeloma patients improves over last decade by maike haehle from the myeloma beacon results of a recent retrospective study conducted at the mayo clinic indicate that the survival of patients with non secretory multiple myeloma has improved over the last decade. Nonsecretory multiple myeloma relapsing as extramedullary liver. Clinical features, laboratory manifestations, and diagnosis formulary drug information for this topic no drug references linked in this topic. Non secretory myeloma of the maxilla is a rare occurrence. Non secretory plasma cell myeloma of the true non producer type. Outcome of patients with nonsecretory multiple myeloma.
The good news is that statistically non secretors live longer, the bad news is that it is more difficult to track our levels. Despite the presence of osteolytic lesions, no mcomponent could be demonstrated in serum protein electrophoresis, and serum and urine. Multiple myeloma mm is the second most common hematologic malignancy in the us. However, among patients diagnosed between 2001 and 2012, os was superior in non.
A case report nonsecretory multiple myeloma nsmm is a rare variant of the classic form of mm that has a similar clinical and. Very few patients present as non secretory myeloma when no immunoglobulins m spike are produced or only light chains are secreted which can only be detected in urine. Marrow immunofluorescent studies failed to show production of identifiable. You may also find it helpful to speak to a myeloma information specialist on the myeloma infoline. Nonsecretory multiple myeloma presenting as primary plasma cell.
Diagnosis of nonsecretory multiple myeloma wikilite. These patients are often a diagnostic dilemma and a high index of suspicion should be borne in mind, especially in a patient with osteolytic lesions. Nonsecretory myeloma, which comprises 15 % of all myelomas, is a variant of plasma cell myeloma. To make a diagnosis of myeloma, doctors will look for this paraprotein in the blood or urine. Sharing clinical, therapeutic, and prognostic characteristics with mm, real challenge. Double autologous stem cell transplantation in a case of non. However, a small subset of mm called non secretory multiple myeloma nsmm produces no detectable ig. We studied 124 patients diagnosed with multiple myeloma who had no monoclonal protein detected on serum and urine immunofixation at diagnosis and on all subsequent follow. Serum free lightchain measurements for identifying and. This is priya menon joining you from india on curetalks 121st episode.
It is defined as symptomatic myeloma without detectable monoclonal immunoglobulin on serum or urine electrophoresis. Acute renal failure due to cast nephropathy can rarely be the first presentation of multiple myeloma. Imwg criteria for the diagnosis of myeloma and guidelines for the diagnostic workup of myeloma criteria for a diagnosis of myeloma followed by the examinations, tests, and imaging studies that are recommended by the working group for the diagnosis of myeloma and related organ dysfunction. Atypical, nonsecretory presentation of multiple myeloma in a. Here, we report two cases of nonsecretory plasma cell myeloma that manifested as multifoci. Patients with multiple myeloma mm that present at diagnosis with serum monoclonal protein mig of non secretory are commonly excluded from participation in clinical trials due to undesirable disease. The study cohort consisted of 30 patients diagnosed between january 1, 2008 and december 31, 2018. Nonsecretory myeloma definition of nonsecretory myeloma by. To the best of our knowledge this is the first published case in which multiple myeloma developed during the treatment of essential thrombocythemia with the non cytotoxic drug anagrelide. The diagnosis was based on the clinical picture, typical radiological findings, and infiltration of the bone marrow by myeloma cells which showed specific immunofluorescence staining mainly with antisera for igm and kappa light chains. Non secretory multiple myeloma nsm is a symptomatic myeloma without detectable monoclonal immunoglobulin protein in serum or urine by immunofixation electrophoresis 1,5. Diagnosis of nsm is made by presentations of lytic bone lesions with bone pain, anemia, slight hypercalcemia, good renal function, negative results of protein and immunoelectrophoresis detecting monoclonal gammopathy, and positive clonal proliferation of plasma cells and atypical plasma cells in. Pdf nonsecretory myeloma is a myeloma subtype whose diagnosis, until a few years ago, was established by. Non secretory myeloma or light chainproducing multiple myeloma.
This study looks into the clinical and cytogenetic. Non secretory myeloma nsm is a rare form of myeloma. It is anticipated that the international response criteria for multiple myeloma will be widely used in future clinical trials of myeloma. It may however still be worth monitoring globulins in the future as it is plausible that the neoplastic cells may become secretory. Imwg criteria for the diagnosis of myeloma and guidelines for. Nonsecretory primary plasma cell leukemia a case report. A case of nonsecretorymultiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Ultrastructural features of the plasma cells in non. However, a small subset of mm called nonsecretory multiple myeloma nsmm produces no detectable. However, utilization of more sensitive techniques, such as serumfree light chain assay, may significantly reduce the number of these cases. Serum free lightchain measurements for identifying and monitoring patients with nonsecretory multiple myeloma brief report. A case involving the maxillary tuberosity is presented along with an update on current understanding of myeloma biology, recent insights which. Use of bcell gene rearrangement studies to establish.
Diagnostic pitfalls of nonsecretory myeloma manifesting as. The oral mass and lameness may however allow some objectivity with regards to monitoring chemotherapy. Cytogenetic features and clinical outcomes of patients with. Double autologous stem cell transplantation in a case of. Diagnostic pitfalls of nonsecretory myeloma manifesting as multiple foci of periosseous plasmacytomas. Hallmark of most multiple myeloma cases is the persistent production of some form of immunoglobulins, a phenomenon that brings the disease to attention.
A case of non secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. We hereby present a case of non secretory myeloma with increased kappa. Patients aged at least 18 years and who had symptomatic multiple myeloma or non secretory multiple myeloma based on bone marrow clonal plasma cells, organ or tissue impairment considered by the clinician to be myeloma related, or paraprotein mprotein in serum or urine were eligible for the initial randomisation. The other patients had abnormal flc ratio and 612 had iflc 100 mgl. Use the multiple myeloma response criteria when determining the disease status for multiple myeloma and solitary plasmacytoma. Jan 02, 2009 grannai too am a non secretory mm survivor. We report a case of central nervous system myeloma manifesting as cauda equina nodules, successfully treated with triple intrathecal it chemotherapy, lenalidomide and dexamethasone. It is typically characterized by production of large amounts of defective immunoglobulin ig. Non secretory multiple myeloma nsmm is a well described entity with an estimated prevalence of 3% characterized by typical morphological and pathological mm characteristics and the absence of an m. This study looks into the clinical and cytogenetic features of this disease. Petrous apex non secretory plasma cell myeloma with aberrant cytokeratin expression mohamed badreldine, eman. Hi david i was diagnosed with non secretary multiple myeloma a year ago. A search for non secretory multiple myeloma and nonsecretory multiple myeloma, in the pubmed, yielded less than 80 case reports since 1972.
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