Daily Health Advices

Symptomatic management of pancytopenia in children with Fanconi anemia is similar to that used in children with acquired aplastic anemia and includes transfusion of red cells and platelets when they are listed.

In studying the effect of treatment with androgens has been observed that over 75% of patients have some degree of early hematologic improvement: increasing the number of reticulocytes followed by increased hemoglobin levels by one or two months and happens also rise somewhat later the numbers of leukocytes and platelets. However, it is very difficult to maintain a sustained response to treatment. This explains the paucity of data that indicate the maintenance of remission after stopping the drug.On the other hand, has observed the development of leukemia and liver tumors during androgen treatment, or a proportion of patients are refractory to this treatment and die from bleeding complications. It has been suggested the association of androgens with corticosteroids, indicating that the accelerating effect of the early growth is offset by the effect of the second decelerator. Alter and Young suggest a treatment regimen that includes administering a dose of oxymetholone 5 mg/kg/day and prednisone 2 to 5 to 10 mg every 48 hours if no response, the dose is gradually reduced to the minimum level of androgen maintenance possible.

Finally, bone marrow transplantation in children with Fanconi anemia, offers the only chance of cure for aplastic anemia. However, there is a risk that the chemotherapy and / or irradiation can accelerate the development of secondary malignancies in these patients.

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