TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange
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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2019-01-01
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| Series: | Leukemia Research Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213048919300421 |
| _version_ | 1818999331482501120 |
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| author | Sreenath Kodali Preethi Ramachandran Ivan N. Richard Jen-Chin Wang |
| author_facet | Sreenath Kodali Preethi Ramachandran Ivan N. Richard Jen-Chin Wang |
| author_sort | Sreenath Kodali |
| collection | DOAJ |
| description | 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 |
| first_indexed | 2024-12-20T22:15:43Z |
| format | Article |
| id | doaj.art-7485ae6bed354e6981f92db8cf550b19 |
| institution | Directory Open Access Journal |
| issn | 2213-0489 |
| language | English |
| last_indexed | 2024-12-20T22:15:43Z |
| publishDate | 2019-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Leukemia Research Reports |
| spelling | doaj.art-7485ae6bed354e6981f92db8cf550b192022-12-21T19:25:03ZengElsevierLeukemia Research Reports2213-04892019-01-0112TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchangeSreenath Kodali0Preethi Ramachandran1Ivan N. Richard2Jen-Chin Wang3Division of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, 11212 NY, USADivision of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, 11212 NY, USADivision of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, 11212 NY, USACorresponding author.; Division of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, 11212 NY, USABackground: 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, TMAhttp://www.sciencedirect.com/science/article/pii/S2213048919300421 |
| spellingShingle | Sreenath Kodali Preethi Ramachandran Ivan N. Richard Jen-Chin Wang TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange Leukemia Research Reports |
| title | TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange |
| title_full | TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange |
| title_fullStr | TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange |
| title_full_unstemmed | TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange |
| title_short | TTP-like syndrome associated with hemoglobin SC disease treated successfully with plasma and red cell exchange |
| title_sort | ttp like syndrome associated with hemoglobin sc disease treated successfully with plasma and red cell exchange |
| url | http://www.sciencedirect.com/science/article/pii/S2213048919300421 |
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