ACUTE LEUKEMIA AND CONSTITUTIONAL ABNORMALITIES

Experience of a hematology and pediatric oncology center Amzal.K, Kili. A, El kababri .M, Hessissen .L, El khorassani. M, Khattab. M

Hematology and Pediatric Oncology Center, children’s hospital, Rabat.

kkThe genetic predisposition to tumor diseases exists by children, the acute leukemia represents the major pediatric tumor associated with constitutional abnormalities. These include Trisomy 21: 1.7% of all children with acute lymphatic leukemia and 5.3% of children with acute myeloid leukemia have the Down syndrome (Narod et al). So trisomy 21 constitutes a predisposition to acute leukemias. Leukemias generally respond well to current treatment provided that they are adapted to the field. The relationship between quantitative chromosomal aberration and carcinogenesis is obscure. The observations we propose here concern children with Down's syndrome (9 cases) or carriers of other types of constitutional abnormalities, who develop acute leukemias.

INTRODUCTION

RESULTS

MATERIALS AND METHODS

CONCLUSIONS

Certain congenital anomalies by children predispose to cancers and more particularly to the acute leukemias, other associations are fortuitous. In all cases the adaptation of the treatment is necessary according to the field.

The acute leukemia and constitutional abnormalities association is certainly a rare situation but not an exceptional one in pediatrics. The trisomy of chromosome 21 is the most frequent abnormality associated with acute leukemia by children. Barely 5% of all tumor diseases by children are due to a genetic predisposition. Among constitutional abnormalities associated with acute leukemia (542 LAL et 185 LAM), there are 9 cases of trisomy 21 (or 1.2%), one case of Ataxia telangiectasia, one case of Fanconi's anemia, one sickle-cell anaemia, one bloom syndrome, one Ollier Maffucci syndrome and one case of deficiency at G6PD. This association may be fortuitous or etiological in some cases of cancer. The treatment of leukemia remain to be adapted according to the field.

MATERIALS AND METHODS