by Dr. Edward Libby on Sat Nov 22, 2014 5:37 pm
Hello from sunny Seattle (it just came through the clouds),
Primary plasma cell leukemia (pPCL) is diagnosed when a patient is first seen for multiple myeloma. It is best described as a very aggressive form of multiple myeloma. Patients will generally have one or more of the "CRAB" criteria and they will also have an elevated percentage of plasma cells in their bone marrow biopsy. Primary plasma cell leukemia is diagnosed when a newly diagnosed myeloma patient is found to have a high percentage of plasma (myeloma) cells in the bloodstream.The outcome for patients with pPCL is generally poor as compared to patients newly diagnosed with multiple myeloma that is not in the leukemia phase. Patients with pPCL generally have a short duration of response and shortened lifespan as compared to patients with a diagnosis of "standard" , non leukemic, multiple myeloma.
Secondary plasma cell leukemia(sPCL) is called "secondary" because it occurs after (usually years after) a patient is diagnosed with standard, regular multiple myeloma. Just as in primary plasma cell leukemia, sPCL is diagnosed when a patient is found to have a high percentage of plasma (myeloma) cells in the bloodstream. In a normal person, and even in a myeloma patient, there are usually few if any plasma cells in the bloodstream. Just as in pPCL there must be a high percentage of plasma cells to meet the definition of sPCL. That level is that 20% of the white blood cells in the bloodstream must be myeloma / plasma cells to have the diagnosis of sPCL.
Secondary plasma cell leukemia (and pPCL) are leukemic (bloodstream) stages / phases of multiple myeloma. Secondary plasma cell leukemia occurs primarily in patients who are in the later stages of treatment for their myeloma and who have been heavily pretreated. The myeloma cells have changed (mutated) and no longer stay in the primarily in the marrow. Usually sPCL is detected by the hematologist /oncologist when they see the white blood cell count rising and note that the cells that are increasing are plasma cells. sPCL may also become apparent if a patient's myeloma suddenly becomes more aggressive and unresponsive to treatment.
Unfortunately sPCL is thought to be even more aggressive than pPCL and to have a very poor prognosis. Treatment for sPCL would include any of the available myeloma drugs, although responses tend to be poor and the duration of response is usually short. Many myeloma experts would use bortezomib (Velcade) or carfilzomib (Kyprolis) based therapy for sPCL.
I do not wish to convey that there is no hope for sPCL patients though. Some patients may respond to treatment. Scientists are working to find new drugs (for instance daratumumab) that will work when the old ones fail us and these new drugs may be available in the foreseeable future.
All my best to you.
Last edited by
Dr. Edward Libby on Mon Nov 24, 2014 1:21 am, edited 1 time in total.