Transformation of Polycythemia Vera to Pure Erythroid Leukemia

Pure erythroid leukemia (PEL) is an aggressive and exceedingly rare form of acute leukemia characterized as a neoplastic proliferation of immature cells committed to the erythroid lineage. It has a poor overall median survival of two to three months. To our knowledge, there are currently only a handful of reports of PEL arising from polycythemia vera. Most reported cases have been associated with radiation therapy or chemotherapeutic alkylating agents. Here we report a rare occurrence of polycythemia vera treated with phlebotomy and hydroxyurea that underwent leukemic transformation to pure erythroid leukemia.