ABSTRACT
We report the therapeutic results obtained in 28 children, aged less than 16 years, suffering from novo acute myeloid leukaemia [AML], collected and followed up during a period of 7 years [1996-2002]. Diagnosis and cytologic type were established according the Franco-Americano-British [FAB] cooperative group criteria. All of the patients received the same induction therapy, the so-called 7 + 3 regimen which associates aracytine for 7 days to an anthracyclin [daunorubicine or idarubicine] for three days. When a complete remission [CR] was obtained, a consolidation therapy was administered, consisting of two courses of aracytine associated to daunorubicin in the first course and to vepeside in the second one. Complete remission was obtained in 60 percent of the cases. Death rate during the induction phase was of 28 percent. Among the CR patients, 71 percent relapsed. Relapse was rapid [< 12 months] in 67 percent of the cases; it was medullary and neuro meningeal in two patients. Median survival, disease-free survival and event-free survival at 5 years were respectively of 35 percent, 11 percent and 13 percent. Event-free survival at 5 years was better in patients treated with high dose aracytine [20 percent] than in those treated with low dose [12 percent]. However, in the first [high dose] group, hematologic and extrahematologic toxicities were more important
ABSTRACT
The auto-immune haemolytic anaemia [AHAI] is a scare entity in child. So the positive diagnosis is in easy rule. The therapeutically treatment remains the mainly difficulty because of the less age of some patients and often because of the unforeseenable variability of the diseases evolution. The prognosis is regularly different according to the forms if they are acute with a good prognosis or chronic with unfavourable evolution owing to the fact that a subjacent pathology is often associated. We report the results of a retrospective study of 21 child and teenagers affected by AHAI collected in a period of 16 years old [1986 - 2001]. The average age of these patients was 7 years 4 months [extreme 5 months and 18 years]. So 8 are aged less than 4 years. The outset was Brutal in 4 cases [19 per cent] and progressive in 17 cases [81 percent]. So the anaemic syndrome associating cutano-mucous pallor and asthenia, the haemolytic syndrome developed by icterus and dark urine constituted the most frequently consultation motives respectively observed in 76, 2 per cent and 42, 8 per cent of cases. However digestive signs [abdominal pain and/or diarrhea and/or vomiting] and signs linked to the associated disease [arthralgia, purpura] are revealing of AIHA in respectively 28, 6 and 1 9per cent of cases. The clinical triad of haemolytic anaemia [pallor, icterus and SMG] was noted in 47, 6 percent of cases. An haemogram revealed severe anemia in 17 cases [81 per cent], normochrome anaemia in 95, 2 per cent and macrocytary in the half of cases relating to an important hyper reticulosytosis. So it was regenerative in 20 cases and aregenerative in only one child relating with fierce appearing severe haemolysis. The anaemia has been associated with a thrombopenia in 9 cases and with leucooenia in 2 cases. The direct combs test was positive in 19 cases [90, 5 percent] in the two others cases, where it was negative the cold agglutinin research was positive [> 1/32]. The etiologic balance permitted to place the AIHA in 17 cases of secondary AIHA [81 percent] and in 4 cases of AIHA probably idiopathic [19 percent]. So the viral infections, the systemic diseases and the immunitary deficiency constitute the most frequently associated affections in our series found respectively in 23, 8 per cent, 33, 3 percent and 19 percent of cases. The prescribed corticoid treatment in 19 child permitted the obtainment an initial remission in 13 cases. The splenectomy realized in 4 child was effective in two cases. The prescribed immuno-suppresseur treatment in 4 child was effective in only one case. After a middle recession of 1 year and 9 months, the evolution was favorable [recovery] in 5 cases [23,8per cent]. Six infants were dead and 10 were lost sight