With the aim of reviewing critical concepts and producing recommendations for the management of chronic myelomonocytic
leukemia, key questions were selected according to the criterion of clinical relevance. Recommendations were produced using a Delphi process and four consensus conferences involving a panel of experts appointed by the Italian Society of Hematology and affiliated societies. This report presents the final statements and recommendations, covering patient evaluation at diagnosis, diagnostic criteria, risk classification, first-line
therapy, monitoring, second-line
therapy and allogeneic
stem cell transplantation. For the first-line
therapy, the panel recommended that patients with myelodysplastic-type
chronic myelomonocytic leukemia and less than 10% blasts in bone marrow should be managed with supportive
therapy aimed at correcting
cytopenias. In patients with myelodysplastic-type
chronic myelomonocytic leukemia with a high number of blasts in bone marrow (≥ 10%), supportive
therapy should be integrated with the use of
5-azacytidine. Patients with myeloproliferative-type
chronic myelomonocytic leukemia with a low number of blasts (<10%) should be treated with cytoreductive
therapy.
Hydroxyurea is the
drug of choice to control cell proliferation and to reduce organomegaly. Patients with myeloproliferative-type
chronic myelomonocytic leukemia, and a high number of blasts should receive
polychemotherapy. Both in myelodysplastic-type and myeloproliferative-type
chronic myelomonocytic leukemia, allogeneic
stem cell transplantation should be offered within clinical trials in selected patients.