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Information on Plasma Cell Leukemia UAMS Information on PLASMA CELL LEUKEMIA What is Plasma Cell Leukemia? The causes of PCL are similar to those of myeloma. A Plasma cell leukemia (PCL) is an aggressive form series of genetic alterations during the development of multiple myeloma characterized by high levels of of a plasma cell may to lead to the cell’s uncontrolled abnormal plasma cells circulating in the peripheral growth. However, what triggers these alterations is not (circulating) blood. Normal plasma cells in the bone fully known. Risk factors, such as age and exposure to marrow produce antibodies that fight infection. industrial and environmental elements, are thought to In myeloma most of the abnormal plasma cells play important roles. remain in the bone marrow and are not found in the peripheral blood. In PCL, the abnormal plasma Signs and Symptoms cells are in the peripheral blood. Therefore, PCL is While PCL patients often have symptoms and considered to be an advanced form of myeloma. PCL complications similar to those of myeloma patients, can either originate as the primary manifestation of the they tend to be more severe. Symptoms and disease (primary PCL with no history of myeloma) or complications include: as a transformation of myeloma (secondary PCL with progression of previously diagnosed myeloma). Bone pain Fatigue Recurring infections Key Statistics and Risk Factors Bleeding Primary PCL is rare, with an estimated 1 per million of High levels of calcium (hypercalcemia) the general population diagnosed each year. Secondary Kidney damage PCL occurs in one to four out of 100 cases of myeloma Enlarged liver or spleen resulting from large and is becoming more common as myeloma patients numbers of abnormal plasma cells deposited in these are living longer. organs. As with myeloma, PCL is more common in African Diagnosing PCL Americans than in Caucasians and is slightly more PCL is diagnosed by the number of abnormal plasma common in men than in women. As new insights and cells circulating in the blood. In PCL patients, knowledge about the biology of myeloma and PCL abnormal plasma cells make up more than 20% of are gained, it may be possible to determine which the total number of white blood cells present in the myeloma patients are at increased risk for developing peripheral blood. PCL is diagnosed in the same way as PCL. myeloma. Treatment Prognosis Current treatments for primary PCL are the same as Because of the aggressive nature of the PCL, it is those for myeloma. Most secondary PCL patients usually associated with a poor prognosis and a survival have already had several anti-myeloma treatments, expectancy that is shorter than that of patients with and for some patients their disease may have become typical myeloma. resistant, or refractory, to the treatments. For these patients, more intensive treatments using combinations Future of chemotherapy drugs, steroids and new agents may Scientists at the Myeloma Institute are investigating be considered. Treatments may include: the characteristics of and risk factors for PCL. Understanding the biological and genetic features that Chemotherapy agents, such as cisplatin, adriamycin, enable development of primary PCL and progression and cyclophosphamide from myeloma to secondary PCL will lead to improved Proteosome inhibitors, such as Velcade® therapies for better patient outcomes. (bortezomib) Immunomodulatory agents, such as Revlimid® Patients are encouraged to consult with their (lenalidomide) and thalidomide physicians about the most current therapies available. High-dose therapy and stem cell transplantation for younger and/or healthier patients Newer agents, such as Kyprolis® (carfilzomib) and This information about PCL and other patient Imnovid® (pomalidomide), for those whose disease is education materials are available at refractory to Velcade and Revlimid www.myeloma.uams.edu Other novel agents Myeloma Institute www.myeloma.uams.edu.
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