Complete or partial deletions of chromosome 7 (-7/del7q) represent the most frequent chromosomal abnormalities observed in myeloid neoplasms (MNs) and are associated with a poor prognosis. -7/del7q are observed in 10-15% of adult patients with myelodysplasia (MDS) or with acute myeloid leukemia (AML). The occurrence of -7/del7q is particularly frequent in pediatric MDS, often associated with germline mutations of .GATA2 or SAMD9/SAMD9L genes. The disease biology of -7/del7q and the genes driving leukemic development have not been completely elucidated, hapoloinsufficiency of tumor suppressor genes located in chromosome 7 deleted regions, seems to play a relevant role. The response to standard treatments based either on chemotherapy or to hypomethylating agents plus Venetoclax is limited. No approved targeted therapies exist for patients with -7/del7q; however, some recent studies have discovered some vulnerabilities of these myeloid neoplasms than can be efficiently targeted.
Testa et al. (Fri,) studied this question.