A high sensitivity to targeted therapy with imatinib (IM) is expected... A long-term experience of rare PDGFRB –positive diseases observation reveals that FISH is an optimal method for detection of various PDGFRB gene rearrangements and it should be done in all pts with HES and previously excluded PDGFRA (including those with unchanged karyotype). The IM effectiveness in PDGFRB -positive neoplasms is ambiguous which may be due to the partner gene of PDGFRB. The choice of IM dose should be 400 mg QD.
The treatments and outcomes varied greatly depending on the type of disease, although tyrosine kinase inhibitors (TKIs) were not effective. In contrast to neoplasms with ETV6::PDGFRB fusion, myeloid neoplasms with ETV6::ACSL6 fusion have unique characteristics.