B-cell acute lymphoblastic leukemia with mature phenotype and MLL rearrangement: report of five new cases and review of the literature

Autor:	Silvia Eandi-Eberle, Patricia L. Rubio, Mariela C. Coccé, Sophia Polychronopoulou, Vassilios Papadakis, Stefanos I. Papadhimitriou, George Paterakis, Marta S. Gallego, Cristina N. Alonso, Loizos Petrikkos, Andrea Bernasconi, Maria S. Felice, Jorge Rossi, Adrian P. Mansini, E.O. Sajaroff, Maria Ampatzidou
Rok vydání:	2016
Předmět:	Male Cancer Research Pathology medicine.medical_specialty Lineage (genetic) medicine.medical_treatment Hematopoietic stem cell transplantation Biology Immunoglobulin light chain Translocation Genetic Immunophenotyping 03 medical and health sciences Fatal Outcome 0302 clinical medicine Bone Marrow Precursor B-Cell Lymphoblastic Leukemia-Lymphoma hemic and lymphatic diseases medicine Humans Transplantation Homologous neoplasms Gene Rearrangement Hematopoietic Stem Cell Transplantation Infant Karyotype Hematology Gene rearrangement Phenotype Chromosome Banding Treatment Outcome Oncology 030220 oncology & carcinogenesis Disease Progression Myeloid-Lymphoid Leukemia Protein Female Neoplasm Grading 030215 immunology
Zdroj:	Leukemia & Lymphoma. 57:2289-2297
ISSN:	1029-2403 1042-8194
DOI:	10.3109/10428194.2016.1141407
Popis:	The association between mature-B phenotype and MLL abnormalities in acute lymphoblastic leukemia (ALL) is a very unusual finding; only 14 pediatric cases have been reported so far. We describe the clinical and biological characteristics and outcome of five pediatric cases of newly diagnosed B lineage ALL with MLL abnormalities and mature immunophenotype based on light chain restriction and surface Ig expression. Blasts showed variable expression of CD10/CD34/TdT. MLL abnormalities with no MYC involvement were detected in all patients by G-banding, FISH, and/or RT-PCR. Three patients were treated according to Interfant protocol, one to ALLIC-09, and one received B-NHL-BFM-2004. All patients achieved complete remission and three of them relapsed. Despite the small cohort size, it could be postulated that B lineage ALL with MLL abnormalities and mature phenotype is a distinct entity that differs both from the typical Pro B ALL observed in infants and mature B-ALL with high MYC expression.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5c46d178fa6afbeee8d90594fbdf937a https://doi.org/10.3109/10428194.2016.1141407 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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