단기간의 저용량 Cytosine arabinoside 치료에 반응하였던 Down 증후군에 병발한 급성골수성백혈병 1례 = A Case of Acute Myelogenous Leukemia Which Developed in a Paint with Down's Syndrpme Showed Response to a Short-course Low-dose Cytosine Arabinoside Therapy

다국어 초록 (Multilingual Abstract)

The incidence of hematologic disorders in patients with Down's syndrome is significantly increased, about 14∼30 - fold higher than in general population and includes neonatal transient abnormal myelopoieis and acute leukemias. The age of onset of leukemia in Down's syndrome is peaking first in the newborn period and then under 4 years of age. Down's syndrome with acute leukemia above the age of 20 is very rare and it's treatment oucome is unclear. The treatment of Down's syndrome with leukemia has been controversial because of toxicity and associated congenital cardiac and other abnormalities. But if treated adequately, children with Down's syndrome show a favorable response to anti-leukemia therapy.
A 24-year-old man with Down's syndrome was first seen for the evaluation of anemia and thrombocytopenia. The peripheral blood morphology and bone marrow study revealed acute myelogenous leukemia, cytogenetic study of bone marrow showed trisomy 21. Beacuse of his sicioeconomic condition and medical abnormalities including deafness, visual loss, he was treated with low dose subcutaneous cytosine arabinoside(Ara-C) for 11 days. Complete remission was obtained after 37 days. The complete remission lasted for 5 months. He subsequently relapsed, and died 6 months later.