Oncogenetic Risk Classification of Trinquand et al for Adult T-Cell Acute Lymphoblastic Leukemia (T-ALL)

 

Patient selection: adult T-cell acute lymphoblastic leukemia (T-ALL)

 

Oncogenes:

(1) NOTCH1 (at 9q34.3)

(2) FBXW7 (at 4q31.3)

(3) RAS: K-RAS (at 12p12.1) and/or N-RAS (at 1p13.2)

(4) PTEN (at 10q23.3)

 

A good outcome was seen in patients with BOTH:

(1) NOTCH1 and/or FBXW7 (N/F) mutations AND

(2) absence of RAS and PTEN mutations

 

A poor prognosis was seen in patients with EITHER:

(1) absence of NOTCH1 and FBXW7 mutations OR

(2) presence of RAS and/or PTEN mutations

 

where:

• The occurrence of RAS and PTEN mutations tend to be mutually exclusive (one or the other may be present but raraly both).