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He completed therapy and achieved complete remission. Two months later, his bone marrow showed no evidence of hairy cell leukemia.
Why was a transplant so hard to get after remission?
Remission did not guarantee a new kidney. Most transplant centers told the patient he would need to be free of disease for five years before they would consider him.
Barrientos and her colleagues in the consortium disagreed. They believed ongoing dialysis posed a greater threat to his health than the leukemia, which may have already been cured.
Transplant surgeons also raised concerns that anti-rejection medications, which suppress the immune system, could leave him more susceptible to the cancer returning.
“These are uncharted waters,” Barrientos said. “There was no documentation or no published literature on cases such as his, especially because of his age.”
Time was another factor. The patient was told that if he waited too long, he would no longer be a candidate, and his age placed him low on the transplant list, where most kidneys go to younger recipients.
It took two years to find a center willing to perform the transplant. Barrientos spoke with the surgeon directly.
“I said, ‘Listen, he’s in complete remission. There’s no evidence of the hairy cell leukemia coming back,'” she recalled.
The kidney came from a young, healthy donor the patient had never met. The patient was well known in his community, and when word spread that he needed a transplant, the donor offered a kidney as a way of giving back for all the patient had done.
How do doctors balance anti-rejection drugs with cancer control?
Anti-rejection medications protect a transplanted kidney by suppressing the immune system, the same system that helps keep cancer in check. Managing that tension requires close coordination between specialists.
Barrientos works with the patient’s nephrologist to make sure his white blood cells, neutrophils and overall immune function stay in a healthy range. The nephrology team monitors his anti-rejection drug levels so they do not climb too high. Early on, the medication affected his kidney function, but the team has since found a level that preserves it.
Barrientos sees him every couple of months. The goals are a neutrophil count above 1,000, hemoglobin above 10 and platelets above 100. His hemoglobin, which had fallen to 6 before treatment and required multiple blood transfusions, is now 12. His white blood cell count is normal, and his platelets are around 140 to 150.
She also tracks serum soluble interleukin-2 receptor, also called CD25, a marker of disease activity. His level was 20,000 before treatment and has remained in the normal range since. A review of his blood smear has shown none of the characteristic “hairy” projections on lymphocytes that signal the disease.
His only infection came around the time of surgery, when he traveled from Miami to New Jersey in cold weather and contracted respiratory syncytial virus (RSV). He now receives annual flu and COVID-19 vaccines and is producing his own antibodies.
Post-transplant lymphoproliferative disorders can occur but are rare, and there has been no evidence of one, Barrientos noted.
“He’s in remission and he’s living life,” she said. “It can be done.”
What should patients with a rare cancer know about advocating for themselves?
For Barrientos, the case underscores the importance of patients speaking up, particularly with a disease that many doctors rarely see.
“If you don’t like your doctor or your doctor doesn’t listen to you, please ask for another doctor in the same practice or go to a different center,” she said. “Don’t be afraid to ask or question your doctor.”
She pointed to her patient in her early 40s, who was treated in another country by a doctor who followed treatment guidelines but did not anticipate the long-term immunosuppression from cladribine. Two years later, she still cannot produce enough antibodies and receives intravenous immunoglobulin (IVIG) every month to help prevent infections.
Patients who do not fit the typical profile, such as younger women hoping to become pregnant, should consider seeking care at a center of excellence, Barrientos said. Some patients can be safely monitored without treatment, while others need to start therapy right away. Many patients from Latin America travel to Miami to have their cases reviewed by members of the consortium.
She encouraged patients to learn from unbiased sources, such as webinars and educational forums from the Hairy Cell Leukemia Foundation, and cautioned that some websites promote products without data to support their use.
“An educated patient can actually ask questions that may not have come to the mind of the doctor or may have been missed,” she said.
Finding a community can also help. Other patients may help someone sort out whether symptoms such as fatigue stem from the disease or something else, Barrientos noted. At patient advocacy events, she has watched people meet others with the same diagnosis for the first time.
“You feel that you’re not alone for a condition that is so rare,” she said.
What’s next in hairy cell leukemia treatment?
Researchers are exploring new options for patients with relapsed or refractory hairy cell leukemia, including chimeric antigen receptor (CAR) T-cell therapy. The approach trains a patient’s own immune cells to recognize and kill leukemia cells, as it does in other lymphoproliferative disorders such as lymphoma.
Investigators are also studying agents used in other blood cancers, including BCL2 inhibitors and BTK inhibitors, as well as new combination strategies that could spare patients the complications of chemoimmunotherapy.
Even traditional therapies can keep patients in remission for more than a decade, Barrientos said.
“It is an amazing time,” she said. “I’m very happy to be living in this era where we can offer things to our patients.”
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