Javier Pinilla-Ibarz, MD, PhD: Principle Treatment Options for Patients With a 17p Deletion

What are the principle treatment options in a patient with a 17p deletion?

Patients with CLL and 17p deletions are always a challenge for the practicing oncologist. These patients tend to require therapy at diagnosis or shortly after. At the same time, classically and historically, they respond poorly to chemotherapy and purine analogs. Considering this problem, a clinical trial is the best therapy for these patients, as well as incorporation of the new BCR inhibitor to the clinical practice. Also, the approval of ibrutinib as a front-line therapy for 17p is somewhat improving the outcomes of those in the front-line.

Case 1: Relapsed and Refractory CLL

Robert is a 63-year-old retired civil engineer from Houston, Texas. His medical history is notable for mild hypertension and for an acute appendicitis and appendectomy in 2010. He presented to his PCP in September 2012 with symptoms of intermittent fatigue and abdominal discomfort.

On physical examination, Robert showed moderate splenomegaly (12 cm), lymphadenopathy, and CBC showed elevated WBC count of 98 x 109/L, with 80% lymphocytes, and anemia (Hb 11 g/dL).

He was referred to an oncologist for further evaluation and was subsequently diagnosed with (CLL); peripheral blood flow cytometry showed mature B lymphocytes CD5+/CD23+.

Interphase cytogenetic analysis showed 17p13.1 deletion

He was initiated on chemoimmunotherapy with fludarabine, cyclophosphamide, and rituximab (FCR) in October 2012

After 5 cycles he displayed a complete response, with disappearance of palpable disease, normalization of blood counts, and no evidence of disease on bone marrow biopsy and CT scans.

In January 2015, he presented to his oncologist with symptoms of worsening fatigue and abdominal distension.

• On relapse he shows bulky disease 5.5 cm; Hb 12 g/dL, platelets 120,000 cells/mm3, lymphocytes 39,000/mm3, and beta 2 microglobulin of 4.1 mg/L
• His ECOG performance status at the time of recurrence was 1, and liver and kidney function were within normal limits