Plasma Cell Leukemia: Causes, Symptoms, Treatment, Prognosis, Risk Factors
What is Plasma Cell Leukemia?
PCL or Plasma Cell Leukemia is a rare and aggressive type of cancer in which the patient has increased levels of abnormal plasma cells (subtype of white blood cells) circulating in the peripheral blood. Plasma Cell Leukemia is a lymphoproliferative disorder. Antibodies which fight infection are produced by the normal plasma cells in the bone marrow.
In myeloma, majority of the abnormal plasma cells are present in the bone marrow and not in the peripheral blood. However, in Plasma Cell leukemia, the abnormal plasma cells are present in the peripheral blood. For this reason, Plasma cell leukemia is considered an advanced and aggressive type of myeloma. Plasma cell leukemia can originate as the primary manifestation of the disease (primary plasma cell leukemia with no history of myeloma) or it can occur as a progression of myeloma (secondary PCL with transformation of previously diagnosed myeloma).
What is the Incidence & Risk Factors of Plasma Cell Leukemia?
Primary Plasma Cell Leukemia is rare. Secondary Plasma Cell Leukemia occurs in 1 to 4 patients out of 100 patients who are with myeloma and it is becoming more common as the life span of myeloma patients is increasing. Men are at a slightly increased risk for developing Plasma Cell Leukemia than women. Plasma Cell Leukemia is more commonly seen in African Americans than Caucasians.
What Causes Plasma Cell Leukemia to Develop?
The causes of Plasma Cell Leukemia are similar to myeloma. A sequence of alterations or changes in the genes when the plasma cells are developing, results in the uncontrolled growth of the cells. The exact cause of the genetic alteration; however, is not clearly understood. There are certain risk factors, such as age and exposure to environmental and industrial elements which can contribute to development of Plasma Cell Leukemia.
What are the Symptoms of Plasma Cell Leukemia?
The symptoms and complications of Plasma Cell Leukemia are similar to that of myeloma, but only the severity is less. Symptoms and complications of Plasma Cell Leukemia are:
- Bone pain.
- Recurring infections.
- Hypercalcemia (high levels of calcium).
- Kidney damage.
- Thrombocytopenia (decreased number of platelets).
- Enlarged spleen or liver occurring as a result of accumulation of large numbers of abnormal plasma cells in these organs.
How is Plasma Cell Leukemia Diagnosed?
The diagnosis of Plasma Cell Leukemia is made based upon the number of abnormal plasma cells, which are circulating in the patient's blood. Patients suffering from Plasma Cell Leukemia will have more than 20% of the total number of white blood cells in the form of abnormal plasma cells in the peripheral blood. The diagnosis of Plasma Cell Leukemia is made the same way as the diagnosis of myeloma is made.
How is Plasma Cell Leukemia Treated?
The treatment for Primary Plasma Cell Leukemia is same as myeloma. Many of the secondary Plasma Cell Leukemia patients will already have had multiple anti-myeloma treatments, and in few patients, their secondary Plasma Cell Leukemia may have become refractory or resistant to the treatments. For such patients, more intensive treatments is needed which involves the combination of steroids, chemotherapy drugs, and other new agents which may be used.
Treatment for Plasma Cell Leukemia includes:
- Chemotherapy agents, such as cyclophosphamide, Adriamycin and cisplatin.
- Immunomodulatory agents that include thalidomide and lenalidomide.
- Proteasome inhibitors, such as bortezomib.
- Stem cell transplantation and high-dose therapy for healthier and/or younger patients.
- Other newer agents, such as pomalidomide and carfilzomib are used for patients whose Plasma Cell Leukemia has become resistant to lenalidomide and bortezomib.
What is Prognosis of Plasma Cell Leukemia?
The prognosis of Plasma Cell Leukemia is poor as this disease is very aggressive and is a rare lymphoproliferative disorder. The survival rate of patients with Plasma Cell Leukemia is lesser than patients suffering from a typical myeloma.
What does the Treatment for Plasma Cell Leukemia Look Like in the Future?
Research is going on to investigate the risk factors and characteristics of Plasma Cell Leukemia. Better understanding of the genetic and biological features that allow the development of Primary Plasma Cell Leukemia and progression from myeloma to Secondary Plasma Cell Leukemia will help in development of improved therapies for treatment as well as better prognosis of Plasma Cell Leukemia.
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