NYMC Faculty Publications
TTP-like Syndrome Associated with Hemoglobin SC Disease Treated Successfully with Plasma and Red Cell Exchange
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
Keywords
ADMSTS13, A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, NF-Κb, nuclear factor kappa-light-chain-enhancer of activated B cells, Plasma exchange, Red cell exchange, Sickle cell disease, TMA
Disciplines
Hematology | Medicine and Health Sciences
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
