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Childhood near‐tetraploid acute lymphoblastic leukemia: an EGIL study on 36 cases |
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| Authors: | Petr Lemež Andishe Attarbaschi Marie C. Béné Yves Bertrand Gianluigi Castoldi Erik Forestier Richard Garand Oskar A. Haas Sandrine Kagialis‐Girard Wolf‐Dieter Ludwig Estella Matutes Ester Mejstříková Marie‐Pierre Pages Winfried Pickl Anna Porwit Alberto Orfao Richard Schabath Jan Starý Herbert Strobl Pascaline Talmant Mars B. Van′t Veer Zuzana Zemanová |
| Affiliation: | 1. Department of Hematology and Blood Transfusion, Hospital Jihlava, Jihlava, Czech Republic;2. St. Anna Children’s Hospital, Vienna, Austria;3. GEIL‐Laboratoire d’Immunologie du CHU & Faculté de Médecine, Nancy, France;4. Department of Pediatric Hematology, Hopital Debrousse, Lyon, France;5. Institute of Hematology, University of Ferrara, Ferrara, Italy;6. Pediatric Unit, Department of Clinical Sciences, University of Umea, Umea, Sweden;7. Laboratory of Hematology, University Hospitals, Nantes, France;8. Hematological Laboratory, Hopital Debrousse, Lyon, France;9. Department of Hematology‐Oncology‐Tumor Biology, HELIOS Clinic, Berlin‐Buch, Germany;10. Department of Haematological Oncology, Royal Marsden Hospital, London, UK;11. Department of Pediatric Hematology and Oncology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic;12. Institute of Immunology, University of Vienna, Vienna, Austria;13. Department of Pathology, Karolinska University Hospital and Institute, Stockholm, Sweden;14. Servicio di Citometria, University of Salamanca, Salamanca, Spain;15. Daniel den Hoed Cancer Center, Rotterdam, The Netherlands;16. Center of Oncocytogenetics, Institute of Clinical Biochemistry and Laboratory Diagnostics, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic. |
| Abstract: | Objectives: Patients with near‐tetraploid (karyotype: 81 – 103 chromosomes) acute lymphoblastic leukemia (NT‐ALL) constitute about 1% of childhood ALL and data reported on them are limited and controversial. The aim of the study was to enlarge the knowledge on these rarely occurring ALL. Methods: The members of the European Group for Immunophenotyping of Leukemias (EGIL) searched retrospectively their databases for NT‐ALL patients. Results: We collected data of 36 European children from seven European countries with NT‐ALL diagnosed since 1992. All patients reached complete remission (CR) after induction chemotherapy. Their blasts were negative for peroxidase and BCR‐ABL1. Ten children were diagnosed as T‐cell ALL (T‐ALL) EGIL categories (T‐I n = 2, T‐II n = 2, T‐III n = 3, T‐IV n = 3) and four displayed various structural chromosomal abnormalities. Eight of 10 T‐ALL remained in 1st CR; one died in CR from sepsis and one is alive in 2nd CR. Median survival was 88 (7–213) months. B‐cell precursor (BCP) ALL was diagnosed in 26 children. Thirteen were positive for ETV6‐RUNX1 and are alive in 1st CR for 32–147 months. Ten children were ETV6‐RUNX1 negative and remained in 1st CR for 16–163 months. One girl with hypodiploid and NT metaphases and ETV6‐RUNX1‐negative BCP‐ALL and one of two boys with NT‐BCP‐ALL not examined for ETV6‐RUNX1 died of infection after stem cell transplantation in 2nd/3rd CR. Secondary myelodysplastic syndrome developed in two patients with NT‐BCP‐ALL. Conclusions: Our data demonstrate immunophenotypic, cytogenetic, and molecular heterogeneity of NT‐ALL and favorable prognosis of most NT‐ALL across different immunophenotypic and/or genetic ALL subtypes. |
| Keywords: | Tetraploid acute lymphoblastic leukemia polyploidy childhood classification immunophenotyping molecular genetics prognosis |