20  Classical Phenotype-Genotype Causations

20.1 Overview of genes associated with inherited predisposition to myeloid neoplasms

20.2 Germline predisposition syndromes with increased risk of myeloid neoplasm development

Germline predisposition syndromes with increased risk of myeloid neoplasm development

20.3 Inherited Thrombocytiopenia

Immune Thrombocytopenias to consider which may also present with thrombocytopenia with normal platelet size include

  • RUNX1-related thrombocytopenia (RUNX1-RT)–> 30-40% lifetime risk of developing MDS/AML
  • ETV6-related thrombocytopenia (ETV6-RT)–> 20% lifetime risk of B-ALL with a 30% overall lifetime risk of hematological malignancy
  • ANKRD26-RT
  • CYCS-related thrombocytopenia (CYCS-RT)

20.4 Familial CLL

  • It is common

  • POT1 (~6% of familial cases)

  • DDX41

  • TERF2IP

  • TP53