For many people living with mantle cell lymphoma (MCL), the standards-of-care include Bruton’s tyrosine kinase (BTK) inhibitors. But there is a subset of patients who do not respond well, or at all, to BTK inhibitor treatment. Unfortunately, this often correlates with a poor overall prognosis. As reported by Inside Precision Medicine, a new treatment option is now available for those living with MCL who have had at least two prior lines of systemic therapy, including at least one BTK inhibitor. The U.S. Food and Drug Administration recently approved JAYPIRCA (pirtobrutinib) for adults with MCL. It is the first and only FDA-approved reversible BTK inhibitor.

In the Prescribing Information, JAYPIRCA is described as:

a kinase inhibitor indicated for the treatment of adult patients with relapsed or refractory mantle cell lymphoma (MCL) after at least two lines of systemic therapy, including a BTK inhibitor. This indication is approved under accelerated approval based on response rate.

The drug’s approval hinged on data from the Phase 1/2 BRUIN study, which highlighted the benefits of JAYPIRCA within this patient population. Further research will be completed within the Phase 3 BRUIN MCL-321 study.

JAYPIRCA is orally administered; patients take 200mg daily. While generally safe and well-tolerated, common adverse reactions have included decreased neutrophils, platelet counts, lymphocytes, and hemoglobin; fatigue; diarrhea; easy bruising; and musculoskeletal pain.

Mantle Cell Lymphoma (MCL): A Brief Overview

Mantle cell lymphoma exists under the larger umbrella of non-Hodgkin’s lymphoma (NHL), or cancers that affect the lymphatic system. This rare and aggressive cancer arises from cancerous white blood cells called lymphocytes in the “mantle zone,” or the outer ring of small lymphocytes surrounding a lymphatic node. MCL can metastasize (spread) throughout the body.

In early stages, those living with mantle cell lymphoma may show no symptoms. The first sign of MCL is typically persistent lymph node swelling in the neck and throat. However, swollen lymph nodes may also appear in the chest, elbows, and shoulders. If additional symptoms appear, they can (but do not always) include:

• Nausea and vomiting
• Repeated fever with no known cause
• Unintended weight loss
• Appetite loss
• Indigestion or heart burn
• Enlarged liver, spleen, and/or tonsils
• Feeling of abdominal fullness and/or bloating
• Lower back pain/pressure, which may continue down the legs
• Fatigue
• Diarrhea or other bowel issues
• Headache
• Dizziness
• Poor balance and coordination

Treatment options may include a combination of chemotherapy, stem cell transplants, BTK inhibitors, targeted therapy, immunotherapy, or other medications such as JAYPIRCA.