TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange. (2019)

• Record Type: Journal Article
• Authors: Kodali, Sreenath; Ramachandran, Preethi; Richard, Ivan N.; Wang, Jen-Chin
• Is Part Of: Leukemia research reports. Volume 12(2019)
• Publication Date: 2019
• Subjects: Sickle cell disease -- Red cell exchange -- Plasma exchange -- TMA; 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; Leukemia -- Periodicals; Lymphomas -- Periodicals; Multiple myeloma -- Periodicals; Leukemia -- Periodicals; Lymphoma -- Periodicals; Multiple Myeloma -- Periodicals; Hematologic Neoplasms -- Periodicals; Leukemia; Lymphomas; Multiple myeloma; Electronic journals; Periodicals; 616.9941905
• DOI: 10.1016/j.lrr.2019.100179
• Languages: English
• ISSNs: 2213-0489

Abstract

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.