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

• 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

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