Use of ropeginterferon in inducing graft versus myelofibrosis effect in post‐transplant myelofibrosis relapse

Abstract

Key Clinical Message Here, we describe a patient with post‐transplant myelofibrosis with chronic graft‐versus‐host disease (GVHD), who showed successful molecular remission with ropeginterferon with 100% donor chimerism without any flare up of GVHD. He was initially diagnosed with polycythemia vera (PV) which progressed to myelofibrosis after 6 years. The MYSEC (Myelofibrosis Secondary to PV and ET‐Prognostic Model) and MTSS (myelofibrosis transplant scoring system) scores were 13.1 and 4, respectively, and the patient was in intermediate risk group. He underwent an allogenic stem cell transplant; however, his disease gradually progressed and was administered two donor lymphocyte infusions with minimal response. A second allogeneic transplant was performed, which led to a persistent molecular remission for more than a decade, although he developed chronic skin graft‐versus‐host disease (GVHD). The JAK2 V617F levels started to increase 10 years post‐transplant with ongoing chronic GVHD and a corresponding decrease in donor chimerism levels. He was administered ropeginterferon, which led to a decrease in JAK 2617F to undetectable levels. A graft versus myelofibrosis effect was attained with reversal to 100% donor chimerism, and he has since maintained a molecular remission with undetectable JAK 2617F levels. Chronic GVHD made him ineligible for donor lymphocyte infusions later. Thus, ropeginterferon was successful in inducing graft versus myelofibrosis effect, leading to a molecular response with no flare up of GVHD. The use of ropeginterferon needs to be further evaluated in larger cohorts of post‐transplant myelofibrosis patients.

Keywords

• graft versus host disease
• graft versus myelofibrosis
• MTSS score (myelofibrosis transplant scoring system)
• myelofibrosis
• MYSEC score (myelofibrosis secondary to PV and ET‐prognostic model)
• ropeginterferon