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

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.

• Authors: Sreenath Kodali, Preethi Ramachandran, Ivan N Richard, Jen-Chin Wang
• Journal: Leukemia research reports (Leuk Res Rep) Vol. 12 Pg. 100179 ( 2019) ISSN: 2213-0489 [Print] England
• PMID: 31388486 (Publication Type: Case Reports)