TTP-like Syndrome Associated with Hemoglobin SC Disease Treated Successfully with Plasma and Red Cell Exchange

Authors

Sreenath Kodali
Preethi Ramachandran
Ivan N. Richard
Jen-Chin Wang, New York Medical College

DOI

10.1016/j.lrr.2019.100179

Journal Title

Leukemia Research Reports

First Page

100179

Last Page

100179

Document Type

Article

Publication Date

7-1-2019

Department

Medicine

Abstract

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.

Recommended Citation

Kodali, S., Ramachandran, P., Richard, I. N., & Wang, J. (2019). TTP-like Syndrome Associated with Hemoglobin SC Disease Treated Successfully with Plasma and Red Cell Exchange. Leukemia Research Reports, 12, 100179-100179. https://doi.org/10.1016/j.lrr.2019.100179