An Adult Patient with Early Pre-B Acute Lymphoblastic Leukemia with t(12;17)(p13;q21)/ZNF384-TAF15.
In Vivo
; 32(5): 1241-1245, 2018.
Article
in En
| MEDLINE
| ID: mdl-30150451
ABSTRACT
This is a case report of a 46-year-old man diagnosed with early pre-B acute lymphoblastic leukemia (ALL), bearing the translocation t(12;17)(p13;q21) as the sole chromosomal abnormality. This is a rare chromosomal abnormality that has been reported in approximately 25 cases worldwide. FISH analysis revealed a rearrangement of ZNF384 (12p13) and TAF15 (17q12) genes, which is usually associated with a pre-B ALL phenotype with co-expression of the myeloid markers CD13 and/or CD33. ZNF384 encodes a zinc finger protein, which acts as a transcription factor, regulating the expression of several matrix metalloproteinases and TAF15 belongs to the FET (FUS, EWS, and TAF15) family, consisting of RNA and DNA-binding proteins. Unlike most of the cases where CD10 expression was absent or weak, in our case CD10 was highly expressed. The prognostic significance of ZNF384/TAF15 fusion is not very clear since several reports support a generally good prognosis, while others support a poor clinical outcome. Our patient was treated with the German multicenter ALL (GMALL) protocol for B-ALL, but experienced a fulminant gram-negative sepsis and eventually died during induction therapy.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Translocation, Genetic
/
Chromosomes, Human, Pair 12
/
Chromosomes, Human, Pair 17
/
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma
/
Oncogene Proteins, Fusion
/
Trans-Activators
/
TATA-Binding Protein Associated Factors
Type of study:
Diagnostic_studies
/
Guideline
/
Prognostic_studies
Limits:
Humans
/
Male
/
Middle aged
Language:
En
Journal:
In Vivo
Journal subject:
NEOPLASIAS
Year:
2018
Document type:
Article
Affiliation country:
Grecia