Diagnostic pitfalls of nonsecretory myeloma manifesting as multiple. This study looks into the clinical and cytogenetic features of this disease. Dec 15, 2016 it is typically characterized by production of large amounts of defective immunoglobulin ig. Light chain myeloma only k h li h h i only makes the light chain k kappa or lambda light chains excreted in urine preferentially nonsecretory myeloma no heavy chain no light chain bone marrow plasma cells are the only measure of disease plasmacytoma plasma cell leukemia plasma cell leukemia. Multiple myeloma invasion of the central nervous system. Pdf nonsecretory multiple myeloma with diagnostic challenges.
Multiple myeloma mm is a plasma cell disorder associated with clonal proliferation of plasma cells. The emergence of nonsecretory multiple myeloma during the noncytotoxic treatment of essential thrombocythemia. 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. A case of nsmm with al amyloidosis and nephrotic range proteinuria is presented. However, there is a subset of multiple myeloma patients who do not secrete immunoglobulin or its component parts into. Sep 11, 20 repeated hematological examinations, including bone biopsy with immunohistochemistry, disclosed diagnosis of multiple myeloma of the non secretory type. Pdf the emergence of nonsecretory multiple myeloma. Nonsecretory multiple myeloma, immunoglobulin d myeloma, and plasma cell leukemia. Here, we report two cases of nonsecretory plasma cell myeloma that manifested as multifoci periosseous plasmacytomas. The serum protein is often characterized by an intact immunoglobulin heavy and light chain, or it may be characterized by the light chain alone. Nonsecretory myeloma is a rare myeloma subtype whose diagnosis, until a few years ago, was established by demonstration of monoclonal.
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. This means gps may not consider myeloma when someone presents with symptoms and you may have. B, kaplanmeier curve comparing the survival of patients with non secretory mm to a 1. A, pie chart delineating the distribution of various primary cytogenetic abnormalities in the cohort of patients with non secretory mm. 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. It is defined as symptomatic myeloma without detectable monoclonal immunoglobulin on serum or urine electrophoresis. Nonsecretory myeloma definition of nonsecretory myeloma. Microabstractnonsecretory multiple myeloma is a rare form of multiple myeloma characterized by the absence of a monoclonal protein in serum and urine. Survival of nonsecretory multiple myeloma patients improves. Cytogenetic features and clinical outcomes of patients with. 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.
Papanikolaou x, zhang q, heuck c, waheed s, atrash s, singh z et al. It is typically characterized by production of large amounts of defective immunoglobulin ig. 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 15 338 339 1071. Jan 02, 2009 grannai too am a non secretory mm survivor. Multiple myeloma mm is characterized by the neoplastic proliferation of plasma cells producing a monoclonal immunoglobulin. An attempt is made to explain the absence of pathological proteins in the serum, based on the.
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. 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 followup testing non secretory myeloma. Most reported cases of nsmm show the presence of lytic lesions on skeletal radiography. Nonsecretory multiple myeloma nsmm is a rare clinical and biological variant of the classic form of mm characterized by the absence of monoclonal. Double autologous stem cell transplantation in a case of non. However, a small subset of mm called nonsecretory multiple myeloma nsmm produces no detectable ig. Patients with multiple myeloma mm that present at diagnosis with serum monoclonal protein mig of myeloma. 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. The good news is that statistically non secretors live longer, the bad news is that it is more difficult to track our levels.
Pdf nonsecretory myeloma is a myeloma subtype whose diagnosis, until a few years ago, was established by demonstration of monoclonal. Non producing multiple myeloma mm is a distinct subset of non secretory mm characterized by high cyclin d1 experession and decreased progression free survival. Nonsecretory mm, where serum globulin is within the reference interval, is very rarely reported in dogs, and in some of these cases a monoclonal gammopathy can still be detected with protein electrophoresis. Use the multiple myeloma response criteria when determining the disease status for multiple myeloma and solitary plasmacytoma. This is priya menon joining you from india on curetalks 121st episode. Multiple myeloma is a malignant proliferation of plasma cells within the bone marrow. Nonsecretory multiple myeloma nsmm is a rare variant of the classic form of multiple myeloma mm. A case of nonsecretory multiple myeloma is described. A search for nonsecretory multiple myeloma and nonsecretory multiple myeloma, in the pubmed, yielded less than 80 case reports since 1972. However, in the case of nonsecretory myeloma, plasma cells are unable to secrete immunoglobulin serum and urinary electropho. During my 6 months of remission i was on 8 gms curcumin, essiac tea and cbd oil. The plasma cells proliferate in the bone marrow and often results in extensive skeletal destruction with osteolytic lesions, osteopenia, andor pathologic fractures.
Nonsecretory multiple myeloma and al amyloidosis presenting. Multiple myeloma mm is the second most common hematologic malignancy in the us. Note, the second bullet point above was previously available in this section as a footnote. Multiple myeloma response criteria forms instruction. Oligosecretory myeloma patients have very low measureable levels of abnormal protein either paraprotein or light chains in their blood or urine. Nonsecretory multiple myeloma presenting as primary plasma cell. This variant usually poses a diagnostic challenge to. Diagnosing mm and monitoring treatment response, including eventual relapse, are largely based on sequential measurements of ig. The international myeloma working group established the below criteria in order to. A rare case of nonsecretory multiple myeloma in lagos.
Patients with multiple myeloma mm that present at diagnosis with serum monoclonal protein mig of mar 31, 2015 the authors searched the mayo clinic multiple myeloma database to identify nonsecretory myeloma patients diagnosed between january 1973 and june 2012. Cytogenetic features and clinical outcomes of patients. Nonsecretory 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. Patients with multiple myeloma mm that present at diagnosis with serum monoclonal protein mig of monoclonal protein nonsecretory are commonly excluded from participation in clinical trials due to undesirable 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 plasma cell myeloma of the true nonproducer type. Non secretory multiple myeloma nsmm is a rare variant of the classic form of multiple myeloma mm. Sharing clinical, therapeutic, and prognostic characteristics with mm, real challenge. Myeloma is characterized by the clonal infiltration of the marrow by plasma cells that typically produce a serum or urine paraprotein. Diagnosis of nonsecretory multiple myeloma wikilite. It is the most common primary malignant tumor of the bone, about 27% of the biopsied bone tumors.
Joining gary on the panel is myeloma advocate, jack aiello. Added the bullet points below to general reporting guidelines. Nonsecretory multiple myeloma presenting with diffuse. The emergence of nonsecretory multiple myeloma during the. This subset of true nsmm has become even smaller over time, as the advent of the serum free light chain assay has resulted in the majority of nsmm patients being recategorized as lightchain mm that is, mm cells that produce only the lightchain component of ig. Compared with a control group of patients with secretory multiple myeloma, matched for age and year of diagnosis, the patients with non secretory multiple myeloma had worse survival outcomes, but this was statistically. Nonsecretory myeloma, diagnosed on renal biopsy as cast. Cytogenetic features and clinical outcomes of patients with nonsecretory multiple myeloma in the era of novel agent induction therapy. This is why autologous stem cell transplantation is included in the standard treatment of myeloma patients.
Ultrastructural features of the plasma cells in non. Nonsecretory multiple myeloma nsmm accounts for 1 5% of all multiple myeloma mm cases. Atypical, nonsecretory presentation of multiple myeloma in a dog. However, there remains a group who comprise around 2% to 4% of patients and. Nonsecretory multiple myelomapresenting as primary plasma.
Non secretory plasma cell myeloma of the true non producer type. 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. We describe a rare case of nonsecretory myeloma in a 32year. Hi david i was diagnosed with non secretary multiple myeloma a year ago. Nonsecretory multiple myeloma nsmm is a rare variant of mm and accounts for approximately 1% to 5% of all cases. 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 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. A case of nonsecretorymultiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. We describe a rare case of nonsecretory myeloma in a 32yearold indian woman. 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 been challenging to treat and to assess. Some people with multiple myeloma dont make enough m proteins or light chains for tests to show they have it. In nsmm, no monoclonal gammopathy can be detected in serum or urine by conventional techniques. The study cohort consisted of 30 patients diagnosed between january 1, 2008 and december 31, 2018.
We describe a rare case of non secretory myeloma in a 32yearold indian woman. Multiple myeloma response criteria forms instruction manual 1. Hi david i was diagnosed with nonsecretary multiple myeloma a year ago. 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. 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. However, a small subset of mm called non secretory multiple myeloma nsmm produces no detectable ig. The study revealed that there was a preponderance of the t 11.
The clinical behaviour differs in patients presenting with nsmm from those presenting as oligosecretory myeloma at the outset or as secretory myeloma which later on converts to nonsecretory myeoma at relapse. Nonsecretory myeloma nsm is a rare form of my eloma. Nonproducing multiple myeloma mm is a distinct subset of nonsecretory mm characterized by high cyclin d1 experession and decreased progression free survival. My cohost for the talk is myeloma advocate and editor of, gary petersen. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Nonsecretory multiple myeloma presenting with diffuse sclero. Nonsecretory myeloma is a rare myeloma subtype whose diagnosis, until a few years ago, was established by demonstration of monoclonal plasma cells. 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.
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