Transient Abnormal Myelopoiesis: A Varied Spectrum of Clinical Presentation

Abstract

Transient myeloproliferative disorder (TMD) is a condition seen almost exclusively in newborns with Down syndrome (DS). It can have a spectrum of clinical presentation ranging from being asymptomatic with incidental detection to a stormy course and fatal outcome. We describe three cases of TMD having different clinical presentation, course, complications and outcome. All but one had Down's phenotype; one of them had a severe disease with tumor lysis syndrome and died of liver failure, while the other one had pericardial effusion and cardiac failure but survived. The third patient had a very benign course of illness requiring only supportive care. Newborns with DS should be screened for TMD by a complete blood count during their first month of life, irrespective of symptoms. With increasing knowledge about the natural history and management guidelines, the prognosis of this rare and unique entity has improved in recent years.




J Hematol. 2017;6(1):25-28
doi: https://doi.org/10.14740/jh306w