Multiple myeloma and other plasma cell dyscrasias cancer. University of groningen multiple aspects of a plasma cell. Renal pathologic spectrum in an autopsy series of patients. Investigations revealed an erosive seronegative inflammatory arthritis in association with bilateral carpal tunnel syndrome, anaemia, hepatic impairment and nephroticrange. Free kappa and lambda light chains in plasma cell dyscrasias. The diagnosis of plasma cell dyscrasia is not enough to determine whether or not this is a malignancy. The resulting group of genetically identical cells called a clone produces a large quantity of a single type of antibody immunoglobulin. Molecular mutations below the resolution of fish will not be. Start studying multiple myeloma and related plasma cell dyscrasias.
Sep 10, 2007 multiple myeloma can occasionally manifest with joint disease. Plasma cell enrichment diagnosis increased as much as 50% to 100%. Plasma cell dyscrasia with renal impairment including mgrs. Treatment of plasma cell dyscrasias by antibodymediated. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Multiple myeloma can evolve from a premalignant monoclonal gammopathy. Cooper dl, munday wr, moeckel gw 2015 c3 glomerulonephritis and plasma cell dyscrasia. Poems syndrome, characterized by polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes, is a rare, paraneoplastic disorder secondary to a plasmacell dyscrasia. Plasma cell dyscrasias are treatable but rarely curable.
In patients with this type of cancer, a rogue plasma cell starts reproducing uncontrollably, leading to changes in blood chemistry and causing symptoms like anemia and fatigue. If you have problems viewing pdf files, download the. Plasma cells are positive for cd38 brightly expressed, cd8, cytoplasmic light either kappa or lambda and heavy chain immunoglobulins. Multiple myeloma is a malignant manifestation of plasma cell dyscrasia that results in bone lesions. Plasma cell dyscrasia evaluation begins with an investigation of the presence and type of monoclonal proteins also known as m proteins. Plasma cell myeloma, kahler disease, myelomatosis, plasma cell dyscrasia, myeloma multiple.
Nc medicaid medicaid and health choice hematopoietic stem. Possible causes monoclonal gammopathy of unknown significance found in 3% of people 50 years. The exception to this rule is the disorder termed nonsecretory multiple myeloma. Jun 05, 2017 multiple myeloma also known as myeloma or plasma cell myeloma is a malignancy of plasma cells, which are the white blood cells responsible for the production of antibodies proteins. Sensitive electrophoretic methods have shown that the incidence of these diseases is as high as 5% in adult individuals. In a simple definition, plasma cells are a type of blood cell that help the body fight infection. Laboratory diagnosis and management of plasma cell dyscrasias. Hyperviscosity syndrome in plasma cell dyscrasias request pdf. Plasma cell dyscrasias form a heterogeneous group of diseases characterized by the expansion of the number of monoclonal bone marrow plasma cells that produce monoclonal immunoglobulins.
Although hyperviscosity syndrome in plasma cell dyscrasia pcd and thrombosis in myeloproliferative neoplasm mpn are major causes of morbidity and. Plasma cell dyscrasias also termed plasma cell disorders and plasma cell proliferative diseases are a spectrum of progressively more severe monoclonal gammopathies in which a clone or multiple clones of premalignant or malignant plasma cells sometimes in association with lymphoplasmacytoid cells or b lymphocytes overproduce and secrete into the blood stream a myeloma protein, i. Pdf free kappa and lambda light chains in plasma cell. Plasma cells develop from b cells b lymphocytes, a type of white blood cell that normally produces. Plasma cell neoplasms including multiple myeloma treatment.
The abnormal proliferation of the plasma cells leads to the appearance of plasma cell dyscrasia, with the monoclonal population of cells secreting immunoglobulin or fragments of immunoglobulin these fragments are known as paraprotein or m protein. Plasma cell dyscrasias are a heterogeneous group of disorders caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. However, the diagnostic criteria for this condition have not yet been revised and there is no specific treatment to significantly improve the course of the disease. The result is a corresponding increase in the serum level of its product, the monoclonal immunoglobulin protein mprotein.
The renal biopsy, performed to identify the responsible lesion, is not infrequently the initial indication of a plasma cell. Multiple myeloma is the most serious and prevalent plasma cell dyscrasia, with a median age of onset. Plasma cell dyscrasia 2 free download as powerpoint presentation. Treatment of plasma cell dyscrasia causing lightchain tubulopathyisamatterofdebate. Hematopoietic stem cell transplantation for plasma cell dyscrasias, including multiple 2 myeloma and poems syndrome depending on the disease being treated, an acceptable donor will match the patient at all or most. Candidate antigens that have been targeted or proposed for targeting in pcds include the immunoglobulin idiotype, cd19, cd38, cd54, cd126, hm1. Plasma cell dyscrasia is a medical condition, in which the plasma cells are affected. Plasma cell dyscrasia is a cancer of the plasma cells, with multiple myeloma being a wellknown example. Poems syndrome, characterized by polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes, is a rare, paraneoplastic disorder secondary to a plasma cell dyscrasia. Multiple myeloma mm is an incurable plasma cell malignancy of the bone marrow. Plasma cell dyscrasia definition of plasma cell dyscrasia. Other malignant plasma cell dyscrasias include solitary myeloma of bone, osteosclerotic myeloma, and amyloidosis of bone. They begin when a single plasma cell multiplies excessively.
The cells usually have plasma cell morphology, but may have lymphocytic or lymphoplasmacytic morphology. Mproteins may consist of both heavy and light chains. Multiple myeloma also known as myeloma or plasma cell myeloma is a malignancy of plasma cells, which are the white blood cells responsible for the production of antibodies proteins. All patients with mgus are generally kept under observation to detect increases in m protein levels and development of a plasma cell dyscrasia. Investigations revealed an erosive seronegative inflammatory arthritis in association with bilateral carpal tunnel syndrome, anaemia, hepatic.
Plasma cell disorders are of unknown etiology and are characterized by the disproportionate proliferation of one clone. Free kappa and lambda light chains in plasma cell dy. Plasma cell disorders is a difficult topic where most residents and students confuse with regarding to differentiating between various types of paraproteinemias or plasma cell dyscrasias. The changed plasma cell myeloma cell multiplies, and, if untreated, these cells continue to grow in the marrow and crowd out the. The initial workup for patients suspected of having a plasma cell dyscrasia should include. The hallmark biomarker in blood or urine is a monoclonal immunoglobulin, the monoclonal protein. Serum free light chain analysis for the diagnosis, management. This simple presentation will highlight the key points in differentiating, diagnosing these orders. Evaluation of plasma cell dyscrasias serum epp, immunoglobulins, immunofixation, and free light chains. Plasma cell dyscrasias testing algorithm arupconsult.
Plasma cell dyscrasias definition of plasma cell dyscrasias. The primary diagnosis was plasma cell dyscrasia in all of these patients. Overview of plasma cell disorders hematology and oncology. Plasma cell dyscrasias refer to a spectrum of disorders characterized by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow and, sometimes, tissue deposition of monoclonal immunoglobulins or their components table 211. Overview of plasma cell disorders blood disorders msd. Schwartz departments of medicine and pathology, rush university medical center, chicago, illinois j am soc nephrol 17. Mm is characterized by a proliferation of malignant plasma cells and a subsequent overabundance of monoclonal paraprotein m protein. Plasma cell disorders may be suspected because of clinical manifestations most often bone disease, renal failure, and low blood counts, or an incidental finding of elevated serum protein or proteinuria that leads to further evaluation with serum or urine protein electrophoresis. The fish results on the enriched assay should not be used as a quantitative assay, since the abnormal cells do not represent the percentage of abnormal cells in the aspirate. Serum free light chain sflc quantification aids in diagnosing multiple myeloma and determining prognosis. Plasma cell dyscrasias indications for testing individual signs and symptoms suggestive of plasma cell dyscrasia order serum protein electrophoresis spep quantifies m protein serum immunofixation electrophoresis sife characterizes m protein serum kappa and flc ratio urine protein electrophoresis upep if primary. Sterken en volgens besluit van het college voor promoties. Plasma cell dyscrasia an overview sciencedirect topics.
Plasma cell dyscrasia with renal impairment including mgrs the importance of multidepartmental management dept. The use of serotherapy to treat patients with plasma cell dyscrasias pcds has been sought by us and others. The normal plasmacell production of free light chains is from 0. Polyclonal gammopathy pg is another electrophoretic. A diagnosis of a plasma cell dyscrasia is not always known prior to the discovery of abnormal kidney function. Multiple myeloma can occasionally manifest with joint disease. In some cases, hematopoietic cell transplantation is considered as therapy.
This complex, multiorgan disease was first described in 1938, but the acronym poems was coined in 1980, reflecting hallmark characteristics of the syndrome. Multiple myeloma and related plasma cell dyscrasias. The percentage of patients with plasma cell dyscrasia who died and underwent autopsy examination was 10% during the span of time this study covered. The renal biopsy, performed to identify the responsible lesion, is not infrequently the initial indication of a plasma cell dyscrasia. Once the plasma cell gate is established, the following markers are useful in subclassification of pcd. Imaging of multiple myeloma and related plasma cell dyscrasias. Cbc with differential count and platelet count routine serum chemistry panel to include calcium, blood urea nitrogen, creatinine. Therearereportedcasesof improved kidney function with treatment912.
Serum free light chain analysis for the diagnosis, management, and prognosis of plasma cell dyscrasias executive summary background plasma cell dyscrasias pcds are a group of neoplastic disorders characterized by the uninhibited expansion of a monoclonal population of malignant plasma cells. We report the case of an individual with a progressive bilateral carpal syndrome and a symmetrical severe seronegative polyarthritis and joint swelling. A diverse group of diseases characterized by the proliferation of a single clone of cells producing a monoclonal immunoglobulin or immunoglobulin fragment a serum m component. Although hyperviscosity syndrome in plasma cell dyscrasia pcd and thrombosis in myeloproliferative neoplasm mpn are major causes of morbidity and mortality, blood viscosity. Amer wahed md, amitava dasgupta phd, dabcc, in hematology and coagulation, 2015.
Apr 19, 2020 plasma cell dyscrasia is a cancer of the plasma cells, with multiple myeloma being a wellknown example. In some patients with a plasma cell dyscrasia, serum protein electrophoresis may be normal because the complete monoclonal immunoglobulin is absent or is present at a very low level. Plasma cell dyscrasia causing light chain tubulopathy. Monoclonal gammopathy of undetermined significance mgus is present in 34% of adults older than 50 years and is characterized by low levels of monoclonal protein, low bone marrow involvement, and the abse. Followup with the physician is recommended to see if further tests have been done or whether there is a clinical diagnosis of malignancy. Dec 16, 2011 plasma cell disorders is a difficult topic where most residents and students confuse with regarding to differentiating between various types of paraproteinemias or plasma cell dyscrasias. Diagnosis and treatment vaishali sanchorawala department of medicine, section of hematologyoncology, boston university medical center, boston, massachusetts lightchain al amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. Because plasma cell dyscrasia is a group of disorders that is characterized by dysfunction of plasma cells. Multiple myeloma, other plasma cell dyscrasia, or lymphoma will develop in 12% of patients by 10 years, 25% of patients by 20 years, and 30% of patients by 25 years. Most of the patients 47 had been treated with highdose chemotherapy and autologous stem cell transplants 61% of all cases. So lets go through some of these blood cells to figure out what exactly plasma cells are and how they work.
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