Congenital transient myeloproliferative disorder progressing to acute myeloid leukemia in Down's syndrome: a case report

Abstract

Chromosomal abnormalities like monosomies and trisomies predispose to various malignancies, hematopoietic or non hematopoietic. Patients with Trisomy 21(Down's syndrome) are prone to acute leukemias during childhood, but congenital leukemia in such children is rare (17%) and should be differentiated from a similar condition -Transient Myeloproliferative Disorder (TMD) which does not necessitate any treatment other than follow up. We report a patient of Down's syndrome with TMD in neonatal period which had spontaneous remission at 3 weeks but later died of acute myeloid leukemia at 6 months. The blasts in our case during the TMD episode were Myeloperoxidase(MPO) positive unlike other cases of TMD reported in literature. To the best of our knowledge we have not come across a case of TMD (MPO positive) later progressing to leukemia in Indian literature. Hence we report this case.