International myeloma working group guidelines for serum. Nonsecretory myeloma, diagnosed on renal biopsy as cast. However, plasma cells are unable to secrete immunoglobulin serum and urinary electrophoresis are negative and free light chain measurement is. 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. Use the multiple myeloma response criteria when determining the disease status for multiple myeloma and solitary plasmacytoma. Smouldering myeloma an overview sciencedirect topics. It is characterised by a clonal population of bone marrow plasma cells which secrete a monoclonal paraprotein or an immunoglobulin free light chain flc figure 1a, b. However, some myelomas are non secretory or hypo secretory and are therefore difficult to manage both in the primary diagnosis, as well as during therapy 7.
Maintenance therapy with lenalidomide significantly improved progression free survival in patients with newly diagnosed multiple myeloma compared with observation, but did not improve overall survival in the intentiontotreat analysis of the whole trial population. Nonsecretory multiple myeloma and al amyloidosis presenting. Exploiting exosomes for diffeial diagnosis of multiple myeloma credit to. Nonsecretory myeloma definition of nonsecretory myeloma by. This is priya menon joining you from india on curetalks 121st episode. Non secretory myeloma is a myeloma subtype with evolving diagnostic criteria. It accounts for 23% of all plasma cell dyscrasias with poor prognosis. It may present with myriad complications including anaemia, hypercalcaemia, renal failure, recurrent infections or bony lytic. However, some myelomas are nonsecretory or hyposecretory and are therefore difficult to manage both in the primary diagnosis, as well as during therapy 7. Nonsecretory multiple myeloma nsmm is a rare variant of the classic form of mm that has a similar clinical and radiologic presentation except for the absence of the mprotein in serum andor urine. Nonsecretory myeloma patients have no detectable levels of abnormal protein in their blood or urine. Serum free light chains in the evaluation of the response of nonsecretory multiple myeloma.
Nonsecretory myeloma definition of nonsecretory myeloma. Non secretory myeloma is an uncommon subtype of multiple myeloma. Multiple myeloma mm is a plasma cell disorder associated with clonal proliferation of plasma cells. 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. Diagnostic pitfalls of nonsecretory myeloma manifesting as multiple. Nonsecretory myeloma nsm is a rare clinical form of multiple myeloma with monoclonal plasmocytic proliferation of the bone marrow and the same clinical and radiological manifestations. Multiple myeloma response criteria forms instruction. Patients with monoclonal gammopathy may have negative serum protein electrophoresis as well as serum immunofixation. However, among patients diagnosed between 2001 and 2012, os was superior in non secretory myeloma n 36 compared to secretory myeloma n 2942, median 8. A diagnosis of multiple myeloma cannot be excluded based on a negative bencejones protein result for the following reasons. We are presenting a case report of this rare variant involving the sternum of an adult male. Mar 31, 2015 the authors of the new study found that, among multiple myeloma patients diagnosed between 1973 and 2001, the median overall survival was similar for patients with nonsecretory myeloma 3.
Therefore, comprehensive imaging studies, the detection of free light chains, and. Clinical course and prognosis of nonsecretory multiple myeloma. Nonsecretory primary plasma cell leukemia a case report. Jan 02, 2009 grannai too am a non secretory mm survivor.
Hallmark of most multiple myeloma cases is the persistent production of some form of immunoglobulins, a phenomenon that brings the disease to attention. Non secretory myeloma comprises less than 01% of this spectrum where serum protein electrophoresis and quantitative immunoglobulins remain essentially normal. Outcome of patients with nonsecretory multiple myeloma after autologous hematopoietic stem cell transplantation. Cytogenetic features and clinical outcomes of patients with.
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 nonsecretory multiple myeloma has improved over the last decade. Depending on the author, nsmm may have or not detectable monoclonal free light chain in urineserum. 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. Renal insufficiency frequently complicates secretory myeloma and is the second most common cause of death after infection in these patients 1,5. Multiple myeloma mm, also known as symptomatic plasma cell myeloma, is a plasma cell malignancy. Survival of nonsecretory multiple myeloma patients improves. The absolute frequency of nonsecretory myeloma can be difficult to analyze, and such quantification is, in fact, even more challenging as we enter an era of different methods of response assessment. In a series from france, it was reported that there was a higher proportion of patients with the t11. A rare case of nonsecretory multiple myeloma in lagos. The good news is that statistically non secretors live longer, the bad news is that it is more difficult to track our levels. Microabstractnon secretory multiple myeloma is a rare form of multiple myeloma characterized by the absence of a monoclonal protein in serum and urine. The excessively produced ig free light chains are excreted by kidneys and. Non secretory multiple myeloma nsmm is a rare variant of the classic form of mm, ranging from 1% to 5% of all cases of mm, and has a similar clinical and radiologic presentation except for the absence of monoclonal gammopathy. We report a 65 year old sudanese lady presented to oncology clinic with complaint of inability to walk for 2 weeks.
A search for non secretory multiple myeloma and nonsecretory multiple myeloma, in the pubmed, yielded less than 80 case reports since 1972. 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. First, you may have to undergo more local radiation in order to kill the mm in the lesion that is causing bone damage. Plasma cell leukemia pcl is a rare disease and the least common variant of multiple myeloma. 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. The study cohort consisted of 30 patients diagnosed between january 1, 2008 and december 31, 2018. Laboratory findings showed anemia, thrombocytopenia and white blood cell count of 49. Non secretory myeloma an overview sciencedirect topics. The subset of these patients with nonsecretory myelomaa group of patients who do not secrete immunoglobulin or its component parts into either the blood or urinehas been challenging to treat and to assess.
It accounts for 15% of all cases of multiple myeloma, and is supposed to be associated with longer survival. Nonsecretory myeloma nsm is a rare form of multiple myeloma with the feature of absence of monoclonal gammopathy in serum and urine by immunofixation electrophoresis. These are patients who met criteria for oligosecretory free light only myeloma, since their protein secretion may not be as high as that seen in. This has not been evaluated in populations from which patients with free lightonly disease are excluded, but there is no reason to expect that. Nsmm must also be differentiated form free light chain only myeloma needing free light chain assay for diagnosis. The low or immeasurable levels of abnormal protein make it challenging for these types of myeloma to be diagnosed and monitored using traditional blood and urine tests. The clinical features of patients with nonsecretory myeloma from a series of 172 consecutive multiple myelomas are presented. Secondly, i believe that you need to expand the types of evidencebased therapies you choose. A rare variant of multiple myeloma article pdf available in journal of the college of physicians and surgeonspakistan. Nonsecretory multiple myeloma nsmm is a rare variant of the classic form of mm, ranging from 1% to 5% of all cases of mm, and has a similar clinical and radiologic presentation except for the absence of monoclonal gammopathy.
However, in the case of nonsecretory myeloma, plasma cells are unable to secrete immunoglobulin serum and urinary electropho. Imwg guidelines for serum free light chain analysis in multiple myeloma and related disorders1 for the more than 3% of myeloma patients who have nonsecretory or oligosecretory disease, and for the majority of. Sharing clinical, therapeutic, and prognostic characteristics with mm, real challenge. Oligosecretory myeloma patients have very low measureable levels of abnormal protein either paraprotein or light chains in their blood or urine. Here, we report two cases of nonsecretory plasma cell myeloma that. It is defined as symptomatic myeloma without detectable monoclonal immunoglobulin on serum or urine electrophoresis. For the more than 3% of myeloma patients who have nonsecretory or oligosecretory disease, and for the majority of patients with al amyloidosis al, the traditional methods of measuring circulating monoclonal immunoglobulins electrophoresis, immunoelectrophoresis, immunofixation electrophoresis, and nephelometric measurement of immunoglobulin heavy chains of serum are not adequate. Joining gary on the panel is myeloma advocate, jack aiello. This study looks into the clinical and cytogenetic features of this disease. 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. Nonsecretory myeloma survived significantly longer than. Once you have been diagnosed with myeloma, doctors will regularly check your paraprotein levels to monitor the disease. A case of nsmm with al amyloidosis and nephrotic range proteinuria is presented.
However, utilization of more sensitive techniques, such as serum free light chain assay, may significantly reduce the number of these cases. A search for nonsecretory multiple myeloma and nonsecretory multiple myeloma, in the pubmed, yielded less than 80 case reports since 1972. The authors of the new study found that, among multiple myeloma patients diagnosed between 1973 and 2001, the median overall survival was similar for patients with non secretory myeloma 3. Uk myeloma forum ukmf and nordic myeloma study group. Microabstractnonsecretory multiple myeloma is a rare form of multiple myeloma characterized by the absence of a monoclonal protein in serum and urine. Serum and urine immunofixation did not show monoclonal immunoglobulin chains whereas serum free light kappa and lambda chains and beta 2.
This variant usually poses a diagnostic challenge to. Imwg guidlines serumfree light chain int myeloma fn. Note, the second bullet point above was previously available in this section as a footnote. My cohost for the talk is myeloma advocate and editor of, gary petersen. Nonsecretory multiple myeloma nsmm is a rare variant of the classic form of mm. You can better understand and cope with your myeloma if you familiarize yourself with the tests used to assess your status throughout the disease course. My experience also as a non secretory myeloma survivor is two fold. We report the case of a 52yearold caucasian man who presented to our hospital because. For the more than 3% of myeloma patients who have non secretory or oligosecretory disease, and for the majority of patients with al amyloidosis al, the traditional methods of measuring circulating monoclonal immunoglobulins electrophoresis, immunoelectrophoresis, immunofixation electrophoresis, and nephelometric measurement of immunoglobulin heavy chains of serum are not adequate. Understanding your test results booklet outlines the various tests that are used to diagnose and monitor myeloma, and to detect response and relapse.
Myeloma cells produce an antibody known as paraprotein. It is defined as monoclonal plasmocytic proliferation of the bone marrow with the same clinical and radiological manifestations of myeloma. Use of bcell gene rearrangement studies to establish. Nonsecretory multiple myeloma nsmm accounts for 1 5% of all multiple myeloma mm cases. The good news is that statistically non secretors live longer, the bad news is. My experience also as a nonsecretory myeloma survivor is two fold. Several cases have been published so far, pointing out the impact of a cytotoxic effect during treatment of essential thrombocythemia on the development of multiple myeloma. The international myeloma working group established the below criteria in order to. 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. However, among patients diagnosed between 2001 and 2012, os was superior in nonsecretory myeloma n 36 compared to secretory myeloma n 2942, median 8.
The emergence of multiple myeloma as a second malignancy in patients with essential thrombocythemia is extremely rare. The nonsecretors survived significantly longer that the secretors, median 46 months versus 21 months p less than 0. The study revealed that there was a preponderance of the t 11. A mayo clinic study of 124 patients initially diagnosed as having nonsecretory multiple myeloma were later found to be composed of 65% free light chain secretors and 35% true nonsecretors. The combination of these three tests has a diagnostic sensitivity of 9798%. However, there is a subset of multiple myeloma patients who do not secrete immunoglobulin or its component parts into. Those patients in whom more modern immunoglobulin testing methods can detect no serum, urine, or free lightbased disease are the patients with truly nonsecretory myeloma. Nonsecretory myeloma was associated with a higher incidence of neurological presentation. Nonsecretory myeloma or light chainproducing multiple. Icd 9 code for kappa light chain myeloma shelly lighting. 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 malignancies in the united states.
Clinical course and prognosis of non secretory multiple myeloma. Survival of nonsecretory multiple myeloma patients. Non secretory multiple myeloma nsm is a symptomatic myeloma without detectable monoclonal immunoglobulin protein in serum or urine by immunofixation electrophoresis 1,5. To make a diagnosis of myeloma, doctors will look for this paraprotein in the blood or urine. Prevalence and monitoring of oligosecretory myeloma. Nonsecretory myeloma or light chainproducing multiple myeloma. The manageable safety profile of this drug and the encouraging results in subgroup analyses of patients across all cytogenetic. As a group, these patients response to therapy, time to disease recurrence, and overall survival were similar to typical myeloma patients. Multiple myeloma mm is the second most common hematologic malignancy in the us. Cytogenetic features and clinical outcomes of patients. A case report of nonsecretorymyeloma with renal failure. Non secretory myeloma nsm is a rare form of myeloma. Clinical course and prognosis of nonsecretory multiple. Nonsecretory multiple myeloma nsmm is a rare variant of mm and accounts for approximately 1% to 5% of all cases.
However, among patients diagnosed between 2001 and 2012, os was superior in non. Pdf nonsecretory myeloma is a myeloma subtype whose. However, plasma cells are unable to secrete immunoglobulin. The emergence of nonsecretory multiple myeloma during the.