Onkologie. 2016:10(3):127-130 | DOI: 10.36290/xon.2016.027

Mixed myelodysplastic/myeloproliferative syndromes

Jaroslav Čermák

Ústav hematologie a krevní transfuze, Praha

Mixed myelodysplastic/myeloproliferative neoplasms (MDS/MPNs) are defined according to the current WHO classification as

„clonal myeloid neoplasms that at the time of initial presentation have some clinical, laboratory or morphology findings that

support a diagnosis of myelodysplastic syndromes (MDS) and other findings more consistent with myeloproliferative neoplasm

(MPN)“. These disorders comprise chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia (aCML), juvenile

myelomonocytic leukemia (JMML) and MDS/MPNs unclassifiable (MDS/MPN, U). In the latter subgroup, refractory anemia with

ring sideroblasts (RARS) associated with thrombocytosis (RARS-T) represents the most precisely defined entity. At present time,

allogeneic stem cell transplantation (SCT) is the only curative treatment for MPS/MPN patiens with overall survival (OS) > 5 years

in approximately 50 % of patients. Administration of hypomethylating agents may be effective in elderly patients with CMML who

are not indicated for SCT, but its efficiency is limited and OS of these patients is < 2 years. Introduction of new disease markers

(karyotype abnormalities, driver genes mutations) should improve prognostic stratification of these patients and more precisely

define subgroups in which intensive and targeted treatment is indicated.

Keywords: myelodysplastic/myeloproliferative sydromes, chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia,

Published: June 1, 2016  Show citation