Laboratory diagnosis and management of plasma cell dyscrasias. The anion gap in asymptomatic plasma cell dyscrasias. Pdf the kidneys are commonly injured in plasma cell dyscrasias. Hematopoietic cell transplantation for plasma cell dyscrasias, including multiple myeloma and poems syndrome. In plasma cell leukemia, however, circulating plasma cells reach far higher numbers and at these circulating levels are associated with exceptionally poor survival rates.
This disease can be clinically indolent or aggressive. Plasma cell dyscrasias are a heterogeneous group of diseases characterized by the expansion of monoclonal bone marrow plasma cells that produce a. 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. Major criteria minor criteria known associations possible associations.
This has been related to the retention of chloride and bicarbonate anions to offset the cationic charge of the monoclonal immunoglobulins. Most plasma cell dyscrasias pcds develop after affinity maturation has occurred in the germinal center, as the gene sequence of most myeloma cells are. Endocrinopathy adrenal, thyroid, pituitary, gonadal, parathyroid. The cells usually have plasma cell morphology, but may have lymphocytic or lymphoplasmacytic morphology. They begin when a single plasma cell multiplies excessively. Treatment for plasma cell dyscrasias usually involves. Plasma cell dyscrasias are treatable but rarely curable. Kidney disease associated with plasma cell dyscrasias blood. We now report the presence of a low anion gap in 50 patients with asymptomatic plasma cell dyscrasias. Application of flow cytometry in the diagnosis of hematological disorders. 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. Plasma cell dyscrasia 2 free download as powerpoint presentation. This book provides a concise overview of the state of the art in the biology and treatment of plasma cell malignancies a heterogeneous group of diseases primarily characterized by the presence of clonal plasma cells.
Feb 16, 2016 what malignancy is common to occur in patients with mgus a. This simple presentation will highlight the key points in differentiating, diagnosing these orders. The anion gap in asymptomatic plasma cell dyscrasias annals. Community preventive services task force recommendation for teambased care for patients with type 2 diabete. Diagnosis of plasma cell dyscrasias and monitoring of minimal. 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. Multiple myeloma is a malignant manifestation of plasma cell dyscrasia that results in bone lesions. Multiple myeloma plasma cell dyscrasias ppt presentation summary. Plasma cell dyscrasias are diseases of the hematologic system. What malignancy is common to occur in patients with mgus a. 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.
Plasma cell dyscrasia must be in complete remission for 3 to 5 years with low and stable monoclonal ig levels. Plasma cell disorders are of unknown etiology and are characterized by the disproportionate proliferation of one clone. Other malignant plasma cell dyscrasias include solitary myeloma of bone, osteosclerotic myeloma, and amyloidosis of bone. Poems syndrome, characterized by polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes, is a rare, paraneoplastic disorder secondary to a plasmacell dyscrasia. Reportable diseases include lymphoplasmacytic lymphoma, multiple myeloma, waldenstrom macroglobulinemia and heavy chain disease. Sensitive electrophoretic methods have shown that the incidence of these diseases is as high as 5% in adult individuals. Plasma cell dyscrasias are a heterogeneous group of disorders caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. Plasma cell dyscrasias include multiple myeloma are a multifocal clonal plasma cell proliferation infiltrating the bone marrow. Multiple myeloma can evolve from a premalignant monoclonal gammopathy.
Reproduction without authorization from blue shield of california is prohibited. References centers for disease control and prevention. Overview of plasma cell disorders hematology and oncology. Plasma cell dyscrasias are defined as an excessive proliferation of a single clone of cells producing entire immunoglobulins, immunoglobulin fragments, heavy chains, or light chains. Monoclonal plasma cell disorders are common with monoclonal gammopathy of undetermined significance mgus affecting up to 3. The plasma cell dyscrasias investigated include monoclonal gammopathy of. Prepared by the tufts evidencebased practice center under contract no. The plasma cell dyscrasias pcds, of which multiple myeloma is the most common and also the prototype disease, represent a group of conditions that have as their common feature an abnormal and frequently, but not invariably, neoplastic proliferation of plasma cells. Poems syndrome, characterized by polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes, is a rare, paraneoplastic disorder secondary to a plasma cell dyscrasia. Plasma cell dyscrasia definition of plasma cell dyscrasia.
Treatment for plasma cell dyscrasias usually involves chemotherapy to kill the cells and may require a. 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. The presence of a low anion gap in patients with multiple myeloma has recently been emphasized. What are the major criteria in diagnosis of multiple myeloma. Serum free light chain analysis for the diagnosis, management, and prognosis of plasma cell dyscrasias. Plasma cell dyscrasia is a cancer of the plasma cells, with multiple myeloma being a wellknown example. Varun malignant proliferation of plasma cells kahlers disease young physician and chemist henry bence jones described the protein in detail mc primary malignant tumor of bone. Plasma cell dyscrasia an overview sciencedirect topics.
Hematopoietic cell transplantation for plasma cell dyscrasias. Pdf the kidney in plasma cell dyscrasias researchgate. Rarely, transient plasma cell disorders occur in patients with drug hypersensitivity eg, sulfonamide. Laboratory evaluation for suspected plasma cell dyscrasias, including multiple myeloma, should be considered for individuals with any of the following presentations. Plasma cell dyscrasias 2017 pdf free pdf epub medical books. Documents the state of the art in the biology and treatment of plasma cell. Amyloidosis nhl other b cell lymphoproliferative disorders smm waldenstroms solitary plasmacytoma 0. The resulting group of genetically identical cells called a clone produces a large quantity of a single type of antibody immunoglobulin. Leung et al, 87 short et al, 88 and rostaing et al 89. Plasma cell dyscrasia includes a diverse group of diseases that produce monoclonal immunoglobulin fragment.
The result is a corresponding increase in the serum level of its product, the monoclonal immunoglobulin protein mprotein. Khan sha, mahesh k, varghese rg 2018 expression of cd 20 and cyclin d1 in bone marrow biopsy of patients with multiple myeloma and. Possible causes monoclonal gammopathy of unknown significance found in 3% of people 50 years. Plasma cells develop from b cells b lymphocytes, a type of white blood cell that normally produces. Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and. Primary plasma cell leukemia ppcl is the most aggressive form of the plasma cell dyscrasias. Deaths attributed to multiple myeloma from 19881997 by age cohort as a percentage of. 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.
Multiple myeloma, waldenstromsmacroglobulinemia, primary amyloidosis, and the heavy chain diseases comprise. Plasma cell dyscrasias pcds are a spectrum of disorders characterized by the expansion of a population of monoclonal bonemarrow plasma cells that produce monoclonal immunoglobulins. Mproteins may consist of both heavy and light chains. How i treat plasma cell leukemia blood american society. 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. B lymphocytes b cells, a type of white blood cell that is found in the bone marrow, develop into plasma cells. Plasma cell dyscrasia is a medical condition, in which the plasma cells are affected. As a result, this clone produces vast amounts of a single antibody monoclonal antibody known as the m. The term plasma cell dyscrasias includes a number of diseases characterized by a single clone expansion of plasma cells and their immediate precursors, the activated b cells. Multiple myeloma and related plasma cell dyscrasias jama. Both are cancers and both are cancers of the blood.
In some cases, hematopoietic cell transplantation is considered as therapy. Evaluation of plasma cell dyscrasias serum epp, immunoglobulins, immunofixation, and free light chains. Evaluation of a perceived cluster of plasma cell dyscrasias among workers at a natural gas company illinois, 2014 announcement. 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. Plasma cell dyscrasias definition of plasma cell dyscrasias. Sensitive electrophoretic methods have shown that the incidence of these diseases is. Where mm derives from the plasma cells which is a mature white blood cell type, leukemias come from the prestages of mature blood cells from both myeloid as lymphoid cell lines red blood cells and white blood cells respectively. Varun babu plasma cell dyscrasias multiple myeloma. In these plasma cell dyscrasias, the presence of even very small numbers of circulating plasma cells is a poor prognostic indicator. Unexplained anemia, elevated serum protein, bone pain with lytic lesions on plain films, recurrent infections, renal failure, hypercalcemia, fatigue, or other systemic symptoms. 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. Normal plasma cell secretion of whole immunoglobulin and free. Immunosuppressive medications may increase risk of recurrence or progression of plasma cell dyscrasia. Apr 19, 2020 plasma cell dyscrasia is a cancer of the plasma cells, with multiple myeloma being a wellknown example.
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. Deaths attributed to multiple myeloma from 19881997 by age cohort as a percentage of total 21,257 deaths. Initial management principles are discussed as well. Plasma cell dyscrasias, characterized by the monoclonal growth of plasma cells, include multiple myeloma, waldenstrom macroglobulinemia wm, monoclonal gammopathy of undetermined significance mgus, and amyloidosis. Editorial on laboratory diagnosis and management of plasma cell dyscrasias special issue. Overview of plasma cell disorders blood disorders merck. 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. 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. Healthy plasma cells are part of the immune system and make proteins. Plasma cell disorders can vary from asymptomatic, stable conditions in which only the monoclonal protein is present to progressive cancers eg, multiple myelomafor classification, see table classification of plasma cell disorders.
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