ABSTRACT
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.
