Genetic characterization of B-cell prolymphocytic leukemia: a prognostic model involving MYC and TP53

Abstract

Key Points B-PLL is tightly linked to MYC aberrations (translocation or gain) and 17p (TP53) deletion. Cases of B-PLL with MYC aberration and 17p (TP53) deletion have the worst prognosis.

Chronic lymphocytic leukaemia and prolymphocytic leukaemia. Two coins or two sides of the same coin?

“…11 However, we did not find any expression of genes related with MCL in our patient who had instead MYC rearrangement, a frequent feature in B-PLL and rare in MCL. 12 In agreement with the previously described data documenting that 50-80% of B-PLL have mutated IGHV, 3,13 our case showed a mutated IGHV pattern in the B-PLL clone. These previous studies also described that the IGHV rearrangements were predominantly involving IGHV3 or IGHV4 in most cases (89-100%).…”

“…In agreement with the previously described data documenting that 50-80% of B-PLL have mutated IGHV , 3 , 13 our case showed a mutated IGHV pattern in the B-PLL clone. These previous studies also described that the IGHV rearrangements were predominantly involving IGHV3 or IGHV4 in most cases (89-100%).…”

“…These previous studies also described that the IGHV rearrangements were predominantly involving IGHV3 or IGHV4 in most cases (89-100%). 3,13 However, the neoplastic B-PLL cells in our case were IGHV5, until now not described in B-PLL. In CLL, the mutational status of the IGHV genes allow the stratification of patients into two prognostic groups and the cases with unmutated IGHV genes having a worse prognosis.…”

“…Laura Magnano, 1,2* Alba Navarro, 2,3* Mónica López-Guerra, 1,2,3 Guillem Clot,2,3 Sílvia Beà, 1,2,3 Gabriela Bastidas, 4 Dolors Costa, 1,2,3 Andrea Rivero, 4 Marta Garrote, 4 Eva Giné, 2,3,4 María Rozman, 1,2,3 Marta Aymerich, 1,2,3 Dolors Colomer, 1,2,3 Armando López-Guillermo, 2,3,4 Elías Campo, 1,2,3…”

An illustrative case of B-cell prolymphocytic leukemia

“…Peripheral lymphadenopathy is uncommon [ 2 ]. Cytogenetic abnormalities, c-myc aberration, and deletion 17p/TP53 mutation are seen in about 75%, 60%, and 40% cases, respectively [ 5 ]. Based on the c-myc aberration, and deletion 17/TP53 mutation, B-PLL is classified into three prognostic groups; low-risk (myc-activation- and deletion 17p - ), intermediate-risk (myc-activation + and deletion 17p - ), and high-risk (myc-activation + and deletion 17p + ) [ 6 ].…”