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J Pediatr Hematol Oncol ; 33(1): 68-70, 2011 Jan.
Article in English | MEDLINE | ID: mdl-20881870

ABSTRACT

Diamond-Blackfan anemia (DBA) is a rare, congenital, pure red blood cell aplasia owing to gene defects affecting the function of ribosomal proteins, essential for erythroid maturation. Iron overload is a serious complication of chronic transfusions, which may lead to cardiac toxicity and endothelial damage. We report a case of pulmonary embolism, observed after viper bite in a transfusion-dependent child with DBA without known inherited thrombophilic factors. Embolic events are uncommon after viper bites, which they usually cause consumption coagulopathy, resulting in hypocoagulable state. DBA has not been earlier correlated with thrombotic episodes. In our patient, we suggest an iron overload-induced hypercoagulability state, which in the presence of a procoagulant substance lead to the development of a thromboembolic event.


Subject(s)
Anemia, Diamond-Blackfan/complications , Pulmonary Embolism/complications , Snake Bites/complications , Adrenal Cortex Hormones/therapeutic use , Anemia, Diamond-Blackfan/genetics , Anemia, Diamond-Blackfan/therapy , Blood Transfusion, Autologous , Child , Erythrocyte Transfusion , Hematopoietic Stem Cell Transplantation , Humans , Iron Overload/complications , Iron Overload/genetics , Iron Overload/therapy , Male , Mutation , Pulmonary Embolism/genetics , Pulmonary Embolism/therapy , Ribosomal Proteins/genetics , Ribosomal Proteins/metabolism , Snake Bites/genetics , Snake Bites/therapy , Transplantation, Homologous
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