Erythropoiesis stimulating agents have limited therapeutic activity in transfusion-dependent patients with primary myelofibrosis regardless of serum erythropoietin level.

摘要

Erythropoiesis stimulating agents (ESA) are widely used for the treatment of anemia associated with both lym-phoid and myeloid neoplasms (1). The suggestion that ESA work better in the context of lymphoid as opposed to myeloid malignancies and the fact that they might exacerbate splenomegaly in the latter was recognized almost two decades ago (2). In general, serum erythro-poietin (Epo) levels in patients with myelofibrosis are appropriately elevated for the degree of anemia and it was therefore suggested that exogenous administration of ESA might have limited value in this disease (3). Subsequent studies reported either positive (4) or negative (5) results in terms of treatment response to ESA in myelofibrosis and suggested limited activity in the presence of transfusion-dependency, marked splenomegaly, serum Epo level of > 125 U/L or homozygosity for JAKZV 617F (6-8). In a recent communication, we reported an unexpected and significant association between ESA use and leukemic transformation in primary myelofibrosis (PMF) (9), an observation that is consistent with current concerns in the use of the drug in patients with head and neck cancer, breast cancer and non-small cell lung cancer (10). We therefore sought to revisit with our experience in the use of ESA in patients with PMF.