NTDTNew Tang Dynasty Television
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La magnitud de este desequilibrio es el determinante principal del fenotipo de la enfermedad, la cual oscila entre pacientes asintomaticos ([beta]-talasemia NTDT o portador), hasta los pacientes que dependen de transfusiones regulares para vivir TDT [1].
[beta]-talasemia no dependiente de transfusiones (NTDT).
A lo largo de los anos las clasificaciones clinicas de la enfermedad han cambiado, anteriormente se clasificaba segun la gravedad del fenotipo clinico en [beta]-Talaasemia mayor, in termedia y menor, sin embargo en la actualidad se ha simplificado un poco y se clasifica con respecto a la necesidad o no de trasfusiones en TDT o NTDT
HbH which is a form of NTDT has a diverse phenotypic presentation depending on the degree of alpha globin chain deficiency which in turn relates to the underlying a-thalassemia mutations with hemolysis being the dominant clinical symptom [1].
Thromboembolic events occur in both b-thalassemia major and NTDT patients [6].
In our study, pregnancy was complicated with portal vein thrombosis, a generally uncommon event in NTDT. The patient underwent an extended investigation for the putative underlying cause of thrombosis that included hereditary and acquired thrombophilia tests along with tests for the exclusion of a latent myeloproliferative disorder and paroxysmal nocturnal hemoglobinemia because of the site of thrombosis (visceral).
Chronic iron overload can also occur in patients with NTDT, due to increased iron absorption in the stomach and intestines.