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An Adult Patient with Early Pre-B Acute Lymphoblastic Leukemia with t(12;17)(p13;q21)/ZNF384-TAF15.
In Vivo 2018 Sep-Oct; 32(5):1241-1245V

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.

Authors+Show Affiliations

Department of Cytogenetics and Molecular Pathology, Locus Medicus S.A, Athens, Greece.Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece pandiamantopoulos@gmail.com.Section for Cancer Cytogenetics, Institute for Cancer Genetics and Informatics, Oslo University Hospital, Oslo, Norway.Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.Department of Immunology and Histocompatibility, Laikon General Hospital, Athens, Greece.Hematology Unit, First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

30150451

Citation

Georgakopoulos, Nikolaos, et al. "An Adult Patient With Early Pre-B Acute Lymphoblastic Leukemia With T(12;17)(p13;q21)/ZNF384-TAF15." In Vivo (Athens, Greece), vol. 32, no. 5, 2018, pp. 1241-1245.
Georgakopoulos N, Diamantopoulos P, Micci F, et al. An Adult Patient with Early Pre-B Acute Lymphoblastic Leukemia with t(12;17)(p13;q21)/ZNF384-TAF15. In Vivo. 2018;32(5):1241-1245.
Georgakopoulos, N., Diamantopoulos, P., Micci, F., Giannakopoulou, N., Zervakis, K., Dimitrakopoulou, A., & Viniou, N. A. (2018). An Adult Patient with Early Pre-B Acute Lymphoblastic Leukemia with t(12;17)(p13;q21)/ZNF384-TAF15. In Vivo (Athens, Greece), 32(5), pp. 1241-1245. doi:10.21873/invivo.11371.
Georgakopoulos N, et al. An Adult Patient With Early Pre-B Acute Lymphoblastic Leukemia With T(12;17)(p13;q21)/ZNF384-TAF15. In Vivo. 2018;32(5):1241-1245. PubMed PMID: 30150451.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An Adult Patient with Early Pre-B Acute Lymphoblastic Leukemia with t(12;17)(p13;q21)/ZNF384-TAF15. AU - Georgakopoulos,Nikolaos, AU - Diamantopoulos,Panagiotis, AU - Micci,Francesca, AU - Giannakopoulou,Nefeli, AU - Zervakis,Konstantinos, AU - Dimitrakopoulou,Aglaia, AU - Viniou,Nora-Athina, PY - 2018/04/23/received PY - 2018/05/29/revised PY - 2018/06/05/accepted PY - 2018/8/29/entrez PY - 2018/8/29/pubmed PY - 2018/12/12/medline KW - Pre-B acute lymphoblastic leukemia KW - ZNF384/TAF KW - t(12;17)(q13;q21) SP - 1241 EP - 1245 JF - In vivo (Athens, Greece) JO - In Vivo VL - 32 IS - 5 N2 - 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. SN - 1791-7549 UR - https://www.unboundmedicine.com/medline/citation/30150451/An_Adult_Patient_with_Early_Pre_B_Acute_Lymphoblastic_Leukemia_with_t_12/ZNF384_TAF15_ L2 - http://iv.iiarjournals.org/cgi/pmidlookup?view=long&pmid=30150451 DB - PRIME DP - Unbound Medicine ER -