This strategy was very effective for treating B-NHL in a developing country. The results were comparable to those of the BFM 90 study and other contemporary groups and represented an increase in the cure rates in childhood B-NHL in Brazil.
Highlights
CRLF2
overexpression associates with
IKZF1
deletions that lead to a dominant-negative effect and with
IKZF1
plus.
Paediatric patients with a high load expression of IK4 isoform presented higher
CRLF2
transcript levels.
CRLF2
overexpression and
IKZF1
deletions conferred poorer prognosis both to paediatric patients treated with RELLA05 protocol as well as to adult patients.
Chronic myeloid leukemia (CML) is a rare disease in children. Different from that in adults, childhood CML involves transformative events occurring over a short time period. CML transformation to lymphoid blast phase (BP) is associated with copy number abnormalities, characteristic of BCR‐ABL1 positive acute lymphoblastic leukemia, but not of CML in the chronic phase. Here, we present an unusual case of CML progressing to BP in a 1.6‐year‐old child, harboring IKZF1, PAX5, CDKN2A, and ETV6 deletions at diagnosis. It remains to be addressed whether distinct mechanisms might account for CML pathogenesis in early childhood.
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