<unk>  <unk>  is a type of leukemia. It is a heterogeneous disorder belonging to the group of<unk> ysplastic/myeloproliferative (MDS/MPN) syndromes. In<unk>  many clinical features (splenomegaly,<unk>  predominance in the bone marrow with some dysplastic features but without a differentiation block) and laboratory abnormalities<unk>  proliferation, low leukocyte alkaline phosphatase values) suggest the diagnosis of<unk>  myelogenous leukemia (CML). However the lack of the pathognomonic Philadelphia chromosome and of the resulting BCR-ABL1 fusion point to a different pathogenetic process. Since no specific recurrent genomic or karyotypic abnormalities have been identified in<unk> , the molecular pathogenesis of this disease has remained elusive and the outcome dismal (median survival 37 months) with no improvement over the last 20 years. This sharply contrasts with the outcome for CML, for which the prognosis was dramatically improved by the development of imatinib as a specific inhibitor of the BCR-ABL protein and in particular for CML. In 2012 SETBP1 was identified as a novel oncogene in<unk> ; specific somatic mutations of this gene were discovered in people with<unk>  and related diseases. These mutations, which are identical to the ones present in SGS as germline mutations, impair the degradation of SETBP1 and therefore cause increased cellular levels of the protein.
Atypical chronic myeloid leukemia
Aggressive NK-cell leukemia
Accelerated phase chronic myelogenous leukemia
B-cell prolymphocytic leukemia
Adult T-cell leukemia/lymphoma
Minimally differentiated acute myeloblastic leukemia
T-lymphoblastic leukemia/lymphoma
Precursor T-lymphoblastic lymphoma