Forty-four cases of childhood myelodysplasia with cytogenetics, documented by the Groupe Français de Cytogénétique Hématologique

Forty-four cases of myelodysplasia in children were studied. A third of the cases were unclassifiable according to the FAB system, Twenty-five cases were 'idiopathic' myelodysplastic syndromes (MDS), lour were secondary to chemo- and/or radiotherapy, and 15 (1/3) were found associated with a congenital genetic disease (such as familial myelodysplasia, chromosome syndrome, or various dysmorphic features). Polyclonality was retained in some cases, even in the 'idiopathic MDS' group, demonstrating that non-malignant myelodysplasia does exist. Sex ratio was well balanced in this study; mean age at diagnosis was 4 years; median survival was 3 years; prognosis was better in girls and in young children; normal karyotypes were associated with a better outcome, monosomy 7 with intermediate survival, and other chromosomal findings, including trisomy 8 and 19, with a worse prognosis; refractory anaemia with excess of blasts in transformation, and cases of. secondary MDS were associated with a bad prognosis. Childhood MDS are extremely heterogeneous conditions deserving more studies

Hypodiploidy is a major prognostic factor in multiple myeloma.

Conventional karyotypes performed before any treatment in 208 patients with multiple myeloma were reviewed by the Groupe Français de Cytogénétique Hématologique. A total of 138 patients displayed complex chromosomal abnormalities (CCAs). According to the chromosome number pattern, a first group of 75 patients had a hyperdiploid karyotype. A second group of 63 patients referred to as the hypodiploid group had either pseudodiploid, hypodiploid, or near-tetraploid karyotypes. Of 159 treated patients available for survival analysis, 116 had an abnormal karyotype. The comparison of overall survival (OS) between hyperdiploid and hypodiploid patients showed a highly significant difference (median OS 33.8 vs 12.6 months, respectively, P <.001). The presence of 14q32 rearrangements (36 of 116 patients) worsened the prognosis (median OS 17.6 vs 29.9 months, P <.02). The presence of chromosome 13q abnormalities (13qA, 63 patients) did not modify OS in CCA patients (median OS 20.6 vs 27.8 months, P <.59). However, taking into account the whole series including normal karyotypes, 13qA (63 of 159 patients) had a significant impact on OS (median 20.6 vs 37.1 months, P <.04). In the same way, the presence of a hypodiploid karyotype (52 of 159 patients) had a strong prognostic value (OS 12.8 vs 44.5 months, P <.000 01). A multivariate analysis including stage, beta2-microglobulin, bone marrow plasmocytosis, treatment type, 13qA, and hyperdiploidy and hypodiploidy showed that a hypodiploid karyotype was the first independent factor for OS (P <.001), followed by treatment approach. These results confirm that the chromosome number pattern of malignant plasma cells is a very powerful prognostic factor in newly diagnosed multiple myeloma patients