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Regional HCL Patient Forum (Chicago Area)
July 27, 2022
The Hairy Cell Leukemia Foundation hosted a regional forum for patients at Elgin Community College in Elgin, IL. The speaker was Dr. Martin S. Tallman, a clinical investigator and Director of Faculty Mentorship and Career Development at Northwestern University Feinberg School of Medicine and Robert H. Lurie Comprehensive Cancer Center. (Dr. Tallman was previously at Memorial Sloan Kettering Cancer Center in New York.)
Dr. Tallman is an internationally respected clinician with expertise in Hairy Cell Leukemia. This was Dr. Tallman’s second time presenting at a regional forum hosted by the Hairy Cell Leukemia Foundation. Dr. Tallman previously spoke at the Foundation’s inaugural regional forum for patients in July 2019. You can find a summary of that forum here. >>
Summary
Dr. Tallman introduced his talk by describing Hairy Cell Leukemia, including how rare it is (i.e., there are 1,000 cases per year in the United States) and that it is a B-Cell malignancy.
Dr. Tallman discussed the diagnosis of HCL. He shared slides describing the symptoms of hairy cell leukemia as well as the physical examination, complete blood count, peripheral blood smear (microscopic exam), immunophenotyping, and bone marrow aspirate and biopsy used to diagnose the disease.
Dr. Tallman then spent time discussing treatment options for HCL. He pointed out that treatment does not necessarily need to start immediately after a HCL diagnosis, because some patients can be monitored. This option is available if the patient’s blood counts are stable and consistent. He mentioned the 1, 10, 100 rule as the thresholds at which treatment should be considered. The “rule” advises that when absolute neutrophils drop below 1, hemoglobin drops below 10, and/or platelets dip below 100, treatment may be advised.
The treatment options he discussed were Purine Analogs (Cladribine and Pentostatin), Vemurafenib, Rituximab, Bendamustine, and Moxetumomab Pasudotox.
Purine Analogs
Purine Analogs can lead to a long-term remission rate of 75-90% and a near normal life expectancy, but these drugs are not likely a permanent cure for HCL. Purine analogs remain the most common treatment choice for HCL.
Cladribine is a chemotherapy drug that stops or slows down the growth of cancer cells. Pentostatin is an irreversible inhibitor of adenosine enzymes and stops or slows down the growth of cancer cells. While both drugs are equally effective, cladribine may be more convenient because it is administered over a single five or seven-day course. Side effects of Cladribine include: nausea, vomiting, rash, constipation, and swelling.
Pentostatin is administered via injection. The length of treatment depends on how well the patient’s body responds to the drug. The side effects of Pentotstain may include: nausea, vomiting, rashes, hair loss, muscle or joint pain, and sores in the mouth or throat.
Vemurafenib
Vemurafenib is a small molecule inhibitor that is administered orally. A study done at Memorial Sloan Kettering showed that after two days of Vemurafenib, the spleen began to decrease in size. After a little over a month of treatment, complete remission was achieved. Like with most treatment options, Vemurafenib has side effects, but they are tolerable. Side effects may include arthralgias, joint aches, rashes, or abnormality in liver function.
Rituximab
Rituximab is an anti-CD20 antibody with a remission rate of about 32% when used alone. When used concurrently or following a purine analog, many patients achieve complete remission. Common side effects when using Rituximab could be: nausea, chills, fever, tiredness, headache, and mouth sores.
A disadvantage to the cladribine and rituximab treatment is a profound and extended immunosuppression, putting the patient at risk of opportunistic infection.
Bendamustine
Bendamustine is a chemotherapy drug that can be used to treat HCL. Side effects of the drug include: nausea, vomiting, heartburn, sores or white patches in the mouth, and weight loss.
Moxetumomab Pasudotox
Moxetumomab Pasudotox is a FDA-approved treatment option for relapse or refractory disease. When approved by the FDA, it was the first new HCL treatment drug in about 28 years. Moxetumomab Pasudotox is an intravenously administered immunotoxin, an antibody linked to a toxin with a remission rate of 46%. There are side effects, including constipation and pale skin, as well as issues with the eyes such as dry eyes, pain, swelling, and vision changes. Rare but serious side effects of the drug are capillary leak syndrome and hemolytic uremic syndrome.
Dr. Tallman wrapped up the patient forum by answering questions from participants. Questions regarding treatment options and combination therapies, as well as inquiries about how the Hairy Cell Leukemia Foundation is helping patients were discussed and clarified.