Acute myeloid leukemia with t(X;6)9p11;q23);MYB-GATA1 and female sex: GATA1 insufficiency may be insufficient for pathogenesis.


Journal

Cancer genetics
ISSN: 2210-7762
Titre abrégé: Cancer Genet
Pays: United States
ID NLM: 101539150

Informations de publication

Date de publication:
01 2022
Historique:
received: 14 06 2021
revised: 27 09 2021
accepted: 30 11 2021
pubmed: 17 12 2021
medline: 17 2 2022
entrez: 16 12 2021
Statut: ppublish

Résumé

Pediatric acute myeloid leukemia (AML) is genetically heterogenous (Olsson et al., 2016). t(X;6)(p11;q23) is a rare but recurrent chromosomal translocation in infant AML thought to be associated with male sex and basophilic differentiation (Dastugue et al., 1997). Here we report molecular characterization of AML with t(X;6)(p11;q23);MYB-GATA1 in two female infants and demonstrate preserved GATA1 expression in the sample tested. These findings further debunk a concept that this fusion was restricted to males, in whom it disrupts the only copy of the X-linked GATA1 gene, causing presumable complete loss of GATA1 function. Our data also demonstrate the power and efficiency of RNA sequencing for subclassification of leukemia on a clinically relevant timeline.

Identifiants

pubmed: 34915342
pii: S2210-7762(21)00233-7
doi: 10.1016/j.cancergen.2021.11.007
pii:
doi:

Substances chimiques

GATA1 Transcription Factor 0
GATA1 protein, human 0
MYB protein, human 0
Oncogene Proteins, Fusion 0
Proto-Oncogene Proteins c-myb 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

37-40

Informations de copyright

Copyright © 2021 Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare no conflict of interest.

Auteurs

Alexandra E Kovach (AE)

Division of Laboratory Medicine, Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, 4650 Sunset Blvd., Mailstop #32, Los Angeles, CA 90027, United States. Electronic address: akovach@chla.usc.edu.

Elena Zerkalenkova (E)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Ludmila Zemtsova (L)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Aleksandra Borkovskaya (A)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Marina Gaskova (M)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Marat Kazanov (M)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Alexander Popov (A)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Liudmila Baidun (L)

Russian Children's Clinical Hospital, Moscow, Russian Federation.

Michael Maschan (M)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Alexey Maschan (A)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Paul S Gaynon (PS)

Division of Hematology/Oncology, Department of Pediatrics, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States.

Deepa Bhojwani (D)

Division of Hematology/Oncology, Department of Pediatrics, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States.

Galina Novichkova (G)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Yulia Olshanskaya (Y)

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation.

Gordana Raca (G)

Division of Laboratory Medicine, Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, 4650 Sunset Blvd., Mailstop #32, Los Angeles, CA 90027, United States.

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Classifications MeSH