Plasma Cell Leukemia is a rare variant of multiple myeloma that is rated as an “Ultra High Risk” (UHiR) version of myeloma. Patient outcomes are dismal, even with the advent of several novel potential options.
PCL is either classified as Primary [pPCL] (when patients are diagnosed from the ‘get-go’ as having PCL) or Secondary [sPCL] (when patients’ multiple myeloma progresses to, most likely, a final stage].
Only about 600 – 1,000 patients are diagnosed with PCL per year in the US, with median survival between 12-18 months.
“This uncommon form of clonal plasma cell dyscrasia is the most aggressive variant of the human monoclonal gammopathies … “.
Given this small patient pool, and the severe and sudden onset of the disease, it is understandable that pharmaceutical companies are not keen to invest in clinical studies, despite the fact that there is a very clear need to provide medical/clinical treatment guidance.
PCL is typically treated with treatment protocols for high-risk myeloma, despite the fact that “pPCL is ( distinct clinic-pathologic entity from myeloma because its presenting features and its natural history including response to chemotherapy and poorer prognosis.” However, “… there is no standard of treatment and consensus treatment guidance remains controversial”.
There are few prospective and some retrospective studies that may give some treatment guidance, but nothing truly definitive.
An interesting paper was recently published in the journal Current Problems in Cancer, covering eight individual case reports at Northwell Hospital in Long Island, New York (4 pPCL and 4 sPCL). The intent of this paper was to evaluate the impact of the “newer agents” [such as e.g., the addition of monoclonal antibodies and/or venetoclax] to regimens that include one of the immunomodulators, one of the proteasome inhibitors – mostly Velcade - and typically, dexamethasone.
It is unfortunate that this paper is not available free of charge in the public domain, as it provides a tremendous amount of detail with respect to the eight patients (such as their hematological characteristics, the complexity of their chromosomal abnormalities, and details of their treatment regimens at time of PCL diagnosis and at time of subsequent relapse) as they provide some interesting comparison points for our own PCL.
Key conclusions presented in this paper are:
about the author
I am a patient diagnosed in 2014 with primary plasma cell leukemia (pPCL), a rare and aggressive variant of multiple myeloma and have been very fortunate to find successful treatment at the division of Cellular Therapy at the Duke University Cancer Institute. My wife, Vicki, and I have two adult children and two grandsons who are the ‘lights of our lives’. Successful treatment has allowed Vicki and I to do what we love best : traveling the world, albeit it with some extra precautions to keep infections away. My career in the pharmaceutical industry has given me insights that I am currently putting to use as an advocate to lower drug pricing, especially prices for anti-cancer drugs. I am a firm believer that staying mentally active, physically fit, compliant to our treatment regimen and taking an active interest in our disease are keys to successful treatment outcomes.