The Ph¹ Chromosome in Childhood Leukaemia

Abstract

Ph1 positive leukaemia was diagnosed in eight of 123 (6.5%) consecutive new cases of childhood leukaemia. Four patients presented as typical chronic granulocytic leukaemia (CGL) and four as acute leukaemia, two of the four children with CGL have since developed blast crisis. Morphological and immunological characterization of the blasts in these six acute cases was suggestive of a lymphoid crisis in three, a mixed crisis in two, and a myeloid crisis in one. Remission was achieved in five of the six patients but lasted more than a year in only two; in one of these two patients the blasts at the time of crisis lacked the Ph1 chromosome and in the other, who presented in myeloid crisis, remission ended in a lymphoid crisis. Cytogenetic studies showed unusual translocations in two of the eight children. Serial examination showed that three children the proportion of Ph1 positive cells in the marrow was reduced to less than 6% at the time of remission from acute leukaemia. We conclude that Ph1 positive leukaemia in childhood presents frequently as acute leukaemia and may even mimic 'good risk' acute lymphoblastic leukaemia. The response to treatment cannot be predicted by morphological or immunological characterization of blast cells. Storage of bone marrow for possible autologous transplantation should be considered in children who achieve remission.

Chromosome banding studies in 106 cases of chronic myelogenous leukemia

“…This case was unusual in that 5 different chromosomes were involved in the formation of the complex translocation. Complex Ph 1 translocations involving chromosome 4 have been reported in 7 cases (Rowley et al, 1976;Geraedts et al, 1977;Chessells et al, 1979;Pasquali et al, 1979;Fraisse et al, 1980;Kessous et al, 1980;Sudries et al, 1980), but the break point in the affected no. 4 of t(4; 9;22) in Case 71 was different from those in the reported cases.…”

Clustering of aberrations to specific chromosomes in human neoplasms

“…CARBONELL pers. comm BLOOMFIELD et al 1977;FORMAN et al 1977;GIBBS et al 1977;MANDEL et al 1977;CHESSELLS et al 1979;PI'ITMAN et al 1979 cases with chromosome aberrations in addition to the Phl translocation, and in the others its incidence varies from 1 to 5 % of the cases. It has also been found in CA and MM.…”

“…comm. -BELLOMO 1978;KOHLER 1979 ENGEL et al 1975b; SONTA and SANDBERG 1977a;ROWLEY et al 1979ENGEL et al 1974ISHIHARA et al 1974ENGEL et al 1975aGERAEDTS et al 1977CHESSELLS et al 1979LAWLER et al 1976T. ISHIHARA pers.…”