TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange

Background: Sickle cell hemoglobinopathies are associated with end organ damage but very rarely present with a clinical and laboratory picture of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, characteristic of thrombotic microangiopathy (TMA). Case presentation: We present a patient...

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Bibliographic Details
Main Authors: Sreenath Kodali, Preethi Ramachandran, Ivan N. Richard, Jen-Chin Wang
Format: Article
Language:English
Published: Elsevier 2019-01-01
Series:Leukemia Research Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2213048919300421
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Summary:Background: Sickle cell hemoglobinopathies are associated with end organ damage but very rarely present with a clinical and laboratory picture of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, characteristic of thrombotic microangiopathy (TMA). Case presentation: We present a patient with HbSC disease who developed thrombotic microangiopathy, needing both RBC exchange transfusion and therapeutic plasma exchange (TPE) for complete clinical recovery. Conclusion: Although literature showed therapeutic plasma exchange alone can abrogate a similar clinical scenario, we did an in-depth review which concluded that in most of the TMA cases secondary to sickle cell disease, treatment with both with plasma exchange and red cell exchange transfusion are necessary. Keywords: Sickle cell disease, Red cell exchange, Plasma exchange, TMA
ISSN:2213-0489