Coagulopathy and functional hyposplenism during an episode of thrombotic thrombocytopenic purpura in a HgbS/β (+)-thalassemia patient

Andrew J Gangemi; Peter V Pickens

doi:10.1002/ccr3.202

Coagulopathy and functional hyposplenism during an episode of thrombotic thrombocytopenic purpura in a HgbS/β (+)-thalassemia patient

Clin Case Rep. 2015 Jul;3(7):521-6. doi: 10.1002/ccr3.202. Epub 2015 May 5.

Authors

Andrew J Gangemi¹, Peter V Pickens²

Affiliations

¹ Department of Internal Medicine, Drexel University College of Medicine/Hahnemann University Hospital 245 N. 15th Street, Mail Stop 427, Philadelphia, Pennsylvania, 19102.
² Division of Hematology and Oncology, Department Medicine, Abington Memorial Hospital 1200 Old York Road, Abington, Pennsylvania, 19001.

Abstract

We report a case of TTP in a sickle cell/β+-thalassemia heterozygote with nonspecific complaints and a evidence of hemolysis, initially attributed to sickle crisis. Included in this case is a discussion of the development of functional hyposplenism, a rarely reported complication, limitation of ADAMTS-13 in diagnosis, and the use of platelet transfusion.

Keywords: Hyposplenism; sickle cell disease; thrombotic thrombocytopenic purpura; β+-thalassemia.

Publication types

Case Reports