A population study of severe aplastic anemia in children. Incidence, etiology and course.

The total number of registered cases of severe aplastic anemia in Denmark was 39 among children aged 0-14 years in 1967 to 1982 giving an annual incidence of 2.2 cases per 1 million children in a well defined and stable population. A probable cause of the aplastic anemia was found in 21 cases (54%). Exposure of the population to known etiological factors of aplastic anemia such as chloramphenicol, pesticides and hepatitis was constant in the study period, and accounted for 2, 5, and 2 cases, respectively. Other infections caused 6 cases, other drugs 2 cases, and organic diluents one case. Three children with constitutional aplasia developed severe aplastic anemia among a total of 6 Fanconi's anemias, 3 probable Fanconi's anemias, and 10 cases of erythrogenesis imperfecta diagnosed during the same 16 years. Thirteen patients (33%) died within 3 months after the diagnosis while on supportive treatment and low dose prednisolone supplemented with androgen treatment. Long term survival occurred in 10 patients (26%), of which 5 patients were in complete and 5 in partial remission.