Unusual Presentation of Hairy Cell Leukemia Treated at Sylvester Adds Evidence to Pending Center of Excellence Designation

A 68-year-old Hispanic woman provided doctors at Sylvester Comprehensive Cancer Center at the University of Miami Miller School of Medicine with an unusual opportunity to treat hairy cell leukemia (HCL).

“She didn’t meet the classic description (more often seen in middle-aged white males), which may be why she was initially diagnosed with something else at another facility,” said Justin Taylor, M.D., head of the Taylor Lab at Sylvester and assistant professor in the Department of Medicine, Division of Hematology, at the Miller School.

The case report by Dr. Taylor, lead author Deborah Soong, M.D., and colleagues at Sylvester and Memorial Sloan Kettering Cancer Center was published online June 17 in the JCO Precision Oncology journal.

The report outlines how the woman first received a diagnosis of CD5+ lymphoproliferative disease. Her doctors “realized they were not treating what they thought they were treating,” Dr. Taylor said. “That was the big tipoff.”

The woman was referred to Memorial Sloan Kettering and later to Sylvester to continue her clinical management. Dr. Taylor and colleagues ran a series of tests, including biopsies, immunohistochemical analysis and next-generation genomic sequencing.

A confirmed diagnosis

After all signs pointed to HCL, the investigators ordered a test for the BRAF V600E genetic mutation, which almost all people with HCL feature. The result confirmed their suspicion that the patient, in fact, had the relatively rare HCL.

Correct diagnosis of HCL is essential because purine analogs, including cladribine or pentostatin, can effectively treat the cancer. In fact, the therapy is associated complete remission in about 75% of patients.

“We’re not saying everyone should be tested for it — it is rare,” Dr. Taylor said.

But if a diagnosis remains unclear and you cannot rule out HCL, consider either a genetic or immunohistochemical test.

Even though the BRAF V600E test is becoming more standard outside of academic medical centers, “physicians who see people with hairy cell leukemia can send their patient here for evaluation and further workup,” Dr. Taylor said.

A collaborative effort

Dr. Taylor credits the interdisciplinary collaboration at Sylvester, including clinical leukemia expert Justin Watts, M.D., and pathologist Jennifer Chapman, M.D., with their success in treating this woman and other people with HCL.

“Sylvester is not a Center of Excellence for hairy cell leukemia, but there is no reason it should not be,” Dr. Taylor said. Given the expertise in clinical care, research, and pathology, the Hairy Cell Leukemia Foundation is considering adding Sylvester to its official Centers of Excellence.

“Many doctors will treat few, if any, patients with hairy cell leukemia in their lifetimes. Being an HCL Center of Excellence is an important designation. Patients know that at a Center of Excellence they will receive expert care. This alleviates for the patient some of the stress and anxiety that diagnosis of a rare disease can produce,” said Anna Lambertson, Executive Director of the Hairy Cell Leukemia Foundation.

“We’re excited to be collaborating more closely with the University of Miami. We are proud to have developed a network of 29 HCL Centers of Excellence in 13 countries,” she added.

The designation will allow Sylvester to conduct new clinical trials for patients, improve therapies, and contribute to and benefit from a research registry for this rare cancer.

“We want to study the natural history of the disease and figure out more ways we can help people with the disease,” Dr. Taylor said.

Tags: Dr. Justin Taylor, Hairy Cell Leukemia, Hairy Leukemia Foundation, JCO Precision Oncology, Sylvester Comprehensive Cancer Center