Progression of romiplostim myelofibrosis to myeloproliferative neoplasm

摘要

A 65-year-old man received a diagnosis of idiopathic thrombocytopenic purpura (FTP) 2 years ago based on an isolated thrornbocytopenia (55 X 103/|xL); however, bone marrow showed only increased megakaryocytes, including naked ones (panel A), and cytogenetic studies were unremarkable. The patient failed improvement with steroids, intravenous immunoglobulin, and Rituxan therapy. Also, romiplostim therapy was administered weekly for 4 weeks without improvement. Repeat bone marrow during romiplostim treatment showed hypercellular marrow with 60% to 70% cellularity with increased megakaryocytes and 3/4 reticulin fibrosis (panel B). Repeat marrow was done after the patient was off romiplostim for 6 weeks.. The marrow showed progression of the process to a picture of myelodysplastic/myeloproliferative neoplasms (MDS/MPN) with cellularity of 90% with dysplastic megakaryocytes, granulocytosis/ myeloid hyperplasia, and 4/4 reticulin fibrosis (panel C). The blood shows leukoerythroblastosis, Pelgeroid polymorphonuclear neutrophils, and blasts (5%) (panel D).