Philadelphia chromosome-positive mature B-cell (Burkitt cell) leukaemia.
Philadelphia chromosome-positive (Ph+) acute leukaemia usually shows lymphoblastic morphology and a B-precursor phenotype. The bone marrow aspirate of a 9-year-old boy showed a L3 blast cell morphology in 90% of cells; immunophenotyping revealed a mature B-blast population. The translocation t(9;22) (q34;q11) was seen in 45 out of 50 metaphases, and expression of the corresponding bcr1/abl fusion transcripts, but no IgH/myc co-localization or splitting of c-myc, was demonstrated. Chemotherapy according to the Berlin-Frankfurt-Munster non-Hodgkin's lymphoma (NHL-BFM 95) protocol with maintenance according to the BFM acute lymphoblastic leukaemia (ALL-BFM 90) protocol resulted in continuing complete remission of 54 months. The occurrence of Ph+ Burkitt's leukaemia might reflect multiple-step cancer development.
Mann
Georg
G
Children's Cancer Research Institute, St. Anna Children's Hospital, Kinderspitalgasse 6, 1090 Vienna, Austria. mann@ccri.univie.ac.at
Trebo
Monika M
MM
Haas
Oskar A
OA
Grümayer-Panzer
Eva R
ER
Dworzak
Michael N
MN
Lion
Thomas
T
Gadner
Helmut
H
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002051:Burkitt Lymphoma / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology; D002648:Child; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D015328:Gene Rearrangement, B-Lymphocyte, Light Chain; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D007621:Karyotyping / Q000379:methods; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D014178:Translocation, Genetic
D016044:Fusion Proteins, bcr-abl