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

Abstract

Key Clinical MessageWe 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.

Refractory acquired thrombotic thrombocytopenic purpura in a patient with sickle cell trait successfully treated with caplacizumab

“…In our literature review there have been six reported cases of patients with sickle cell disease or sickle cellhemoglobin C disease also presenting with TTP [9][10][11][12][13][14]. There have also been three cases of TTP diagnosed in a patient with sickle cell thalassemia [15][16][17]. However, there has only been one reported case of TTP in a patient with sickle cell trait [18].…”