특발성골수섬유증에서 Hydroxyurea 치료 중 아세포 증식과 함께 17번 단완 결손이 관찰된 1례

Abstract

The recently described ´17p deletion syndrome´ is a clonal hematologic disease which has characteristic dysgranulopoietic features, such as pseudo-Pelger-Huet hypogranulation and small vacuoles in neutrophils and is strongly associated with p53 mutation. The cases with 17p deletion are seen in 3~4% of myelodysplastic syndrome and acute myelogenous leukemia and about 30% of them are therapy-related. Hydroxyurea, which is considered to have relatively low leukemogenic potential, has therefore been widely used in chronic myeloproliferative disease. But the recent study has found that hydroxyurea administration is a considerable risk for later leukemic transformation and is closely associated with development of 17p deletion. We report one case of idiopathic myelofibrosis which developed 17p deletion with blast increment after hydroxyurea therapy for 3 years.