Researchers reported a number of important findings in the treatment of myeloproliferative neoplasms (MPNs) at the 2018 Annual Meeting of the American Society of Clinical Oncology:

Retrospective study showed increased risk of aggressive lymphomas when patients with MPNs treated with JAK inhibitors

A retrospective study showed that a small but significant proportion of patients with myeloproliferative neoplasms developed aggressive lymphomas during treatment with a Janus kinase (JAK) inhibitor. In a study of 929 patients, 9.7 percent of those treated with JAK1/2 inhibitors developed aggressive B-cell lymphomas compared to 0.54 percent of patients treated with other drugs.

What Patients Need to Know

Researchers determined that patients most at risk for developing an aggressive lymphoma had a pre-existing B-cell clone that underwent transformation during treatment with JAK inhibitors; they suggest that people with myelofibrosis be tested for this B-cell clone before undergoing treatment with JAK inhibitors.

Experimental drug being evaluated as treatment for anemia in MPN-associated myelofibrosis

Anemia is a significant complication of MPN-associated myelofibrosis, with few effective therapies other than red blood cell transfusions. A phase II multicenter global trial is evaluating the experimental drug luspatercept as a potential treatment option for anemia in these patients.

What Patients Need to Know

The trial will evaluate a number of endpoints, including the safety of luspatercept and the improvement in the anemia both short-term and long-term. A determination will also be made whether a randomized phase III trial should be conducted.

JAK1/2 inhibitors being studied in the context of stem cell transplantation for patients with myelofibrosis

Since its FDA approval in 2011, the JAK ½ inhibitor ruxolitinib has become a routine treatment for MPN-associated myelofibrosis. However, allogeneic hematopoietic stem cell transplant remains the only treatment with the potential to treat myelofibrosis. (In an allogeneic hematopoietic stem cell transplant, the person receives blood-forming stem cells from a genetically similar donor.)

Ongoing research is being conducted on the impact JAK ½ inhibitors could have on the timing of allogeneic hematopoietic stem cell transplant and their effect on post-transplantation outcomes.

What Patients Need to Know

Researchers say the identification of guidelines for the use of JAK1/2 inhibitors in the context of transplantation may lead to new treatment strategies for patients with myelofibrosis.