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

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

Andrew J Gangemi  1 , Peter V Pickens  2
Affiliations
Case Reports

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

Andrew J Gangemi et al. Clin Case Rep. 2015 Jul.

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.

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Figures

Figure 1
Figure 1
Peripheral smear in acute TTP crisis. Note the presence of schistocytes (arrows), classic markers of microangiopathic hemolysis, along with the almost complete absence of platelets. Also note the presence of Howell-Jolly bodies (arrowheads).
See this image and copyright information in PMC

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