Plasma cell leukemia is a rare and aggressive form of multiple myeloma that involves high levels of plasma cells (part of the white blood cells – the body’s infection fighters) circulating in the blood. Too many plasma cells prevent other blood components, including red blood cells and platelets, from doing their job. Abnormal plasma cells known as myeloma cells can cause tumors on the bone – weakening them and causing them to break. To determine if one has plasma cell leukemia, abnormal plasma cells must infiltrate the bone marrow with more than 20 percent of cells circulating in the blood. in cicon’s case, his blood counts confirmed 37 percent of these cells were plasma cells. “mark’s fall was a blessing in disguise, as it prompted evaluation much sooner,” says Rutgers cancer institute hematologist/oncologist mecide Gharibo, mD, who is cicon’s doctor and a member of the cancer center’s Blood and marrow Transplant Program. “Had more time passed, the disease could have been worse with high white blood cell counts possibly causing additional complications with bleeding, infection and organ failure.” There are two forms of plasma cell leukemia. ‘Secondary’ disease is end-stage multiple myeloma that progresses into plasma cell leukemia. cicon presented with ‘de novo’ disease, meaning there was no previous myeloma diagnosis. “Less than one percent of multiple myeloma patients present with newly diagnosed plasma cell leukemia,” notes Dr. Gharibo, who is also an associate professor of medicine at Rutgers Robert Wood Johnson medical School. The prognosis is very poor, with median survival of less than a year (Liedtke, Expert Review of Hematology, 2014). tional cancer institute-designated comprehensive cancer center like Rutgers cancer institute. “it is beneficial for patients to have access to the numerous specialists and resources afforded through a program like the Blood and marrow Transplant Program at Rutgers cancer institute and our flagship hospital Robert Wood Johnson University Hospital. in some cases, it’s critical for patients with rare hematologic disorders to be treated at such a center because of the ability of the physicians there to evaluate patients for transplant in a timely manner where remission in aggressive blood cancers may only last for a short period of time, as well as the ability to refer them to the National cancer institute or collaborate on therapy options with other comprehensive cancer centers,” adds Gharibo. cicon began to accept his diagnosis. “it’s a law of averages,” he says. “There’s the good and the bad. i knew i needed to focus on things that would get me to the good end of things.” He educated himself about the transplant process and knew the risks involved – including the possibility of not finding a match for the second transplant. “i was committed to doing this and knew a positive attitude would help me get through it.” cicon’s father, a former triathlete who is approaching 80 and has been a quadriplegic for the past two decades due to an accident, has been a great source of inspiration for him. “Seeing my dad’s attitude and his ability to stay positive all of these years really helped me. Despite the risks and possible complications, i looked at the transplant process as a long-term gain – an opportunity – and felt it would put me back to where i wanted to be.” This back-to-back, or tandem, transplant procedure starts with induction therapy – chemotherapy that is administered to deplete the body of as many cancer cells as possible causing a temporary remission. For cicon that meant hospitalization for two weeks at the end of September 2010 immediately after being diagnosed. His own stem cells were collected through a process similar to a blood transfusion. They would later be reinfused into his body. A steroid and a chemotherapy combination were prescribed to get his disease under control. During this time, Gharibo started looking ahead to the more difficult part of the process – finding a donor for the second transplant. She asked cicon’s only sibling, his older brother, to get tested to see if he would be a match. He wasn’t. The Blood and marrow Transplant team then began a search through the National marrow Donor Program registry to find a suitable candidate. Time really was of the essence. cicon responded well to induction therapy. “So well that i really didn’t want to endure an invasive treatment,” he recalls. But he Where to Start “U nfortunately, there is limited data on this patient population to help drive treatment options,” Gharibo reflects. “Protea- some inhibitors and chemotherapy can help achieve remission in a certain subset of patients for a short period of time. Usually we try to give chemotherapy, then perform an autologous transplant to optimize outcome.” But given cicon’s age and the fact that he was in good health prior to being diagnosed, Gharibo recommended a less common treatment regimen – an autologous transplant (in which a patient’s own peripheral blood stem cells are collected and reinfused in the body) followed by an allogeneic transplant, which utilizes stem cells or bone marrow from another donor. “Time is of the essence,” she says – noting the reason why this strategy is not widely pursued for plasma cell leukemia is that some patients don’t have the advantage of being treated at a Na- 10 I Cancer Connection I Autumn 2016