title: Childhood secondary ALL after ALL treatment.
abstract: Data on secondary acute lymphoblastic leukaemia (sALL) following ALL treatment are very rare. However, the incidence might be underestimated as sALLs without a significant lineage shift might automatically be diagnosed as relapses. Examination of immunoglobulin and T-cell receptor gene rearrangements brought a new tool that can help in discrimination between relapse and sALL. We focused on the recurrences of childhood ALL to discover the real frequency of the sALL after ALL treatment. We compared clonal markers in matched presentation and recurrence samples of 366 patients treated according to the Berlin-Frankfurt-Munster (BFM)-based protocols. We found two cases of sALL and another three, where the recurrence is suspicious of being sALL rather than relapse. Our proposal for the 'secondary ALL after ALL' diagnostic criteria is as follows: (A) No clonal relationship between diagnosis and recurrence; (B) significant immunophenotypic shift--significant cytogenetic shift--gain/loss of a fusion gene. For the sALL (A) plus at least one (B) criterion should be fulfilled. With these criteria, the estimated frequency of the sALL after ALL is according to our data 0.5-1.5% of ALL recurrences on BFM-based protocols. Finally, we propose a treatment strategy for the patients with secondary disease.
authors: CLIP (Childhood Leukaemia Investigation Prague), Department of Paediatric Haematology and Oncology, Charles University, 2nd Medical School, Prague, Czech Republic. jan.zuna@lfmotol.cuni.cz
authors: J
authors: J
authors: Zuna
authors: H
authors: H
authors: Cavé
authors: C
authors: C
authors: Eckert
authors: T
authors: T
authors: Szczepanski
authors: C
authors: C
authors: Meyer
authors: E
authors: E
authors: Mejstrikova
authors: E
authors: E
authors: Fronkova
authors: K
authors: K
authors: Muzikova
authors: E
authors: E
authors: Clappier
authors: D
authors: D
authors: Mendelova
authors: P
authors: P
authors: Boutard
authors: A
authors: A
authors: Schrauder
authors: J
authors: J
authors: Sterba
authors: R
authors: R
authors: Marschalek
authors: J J M
authors: JJ
authors: van Dongen
authors: O
authors: O
authors: Hrusak
authors: J
authors: J
authors: Stary
authors: J
authors: J
authors: Trka
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D005803:Genes, Immunoglobulin; D006801:Humans; D016130:Immunophenotyping; D015994:Incidence; D008297:Male; D025202:Molecular Diagnostic Techniques / Q000379:methods*; D016609:Neoplasms, Second Primary / Q000139:chemically induced / Q000175:diagnosis*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000139:chemically induced / Q000175:diagnosis* / Q000188:drug therapy; D012008:Recurrence
chemical_list: D000970:Antineoplastic Agents