Management of Hyperhemolysis in β-thalassemia With Multiple Immunosuppressives, Including Complement Blockade
| Autor: | Kristen Romanelli, Thomas C Newton, Lauren M Vasta, Richard C Zanetti |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male medicine.medical_treatment Thalassemia Antibodies Monoclonal Humanized Hemolysis Young Adult medicine Humans business.industry beta-Thalassemia Immunosuppression Hematology Eculizumab Prognosis medicine.disease Hematologic Diseases Blockade Complement Inactivating Agents Oncology Pediatrics Perinatology and Child Health Immunology Transfusion dependence Erythropoiesis Drug Therapy Combination Transfusion therapy business Immunosuppressive Agents medicine.drug |
| Zdroj: | Journal of Pediatric Hematology/Oncology. 43:e1145-e1147 |
| ISSN: | 1077-4114 |
| DOI: | 10.1097/mph.0000000000002059 |
| Popis: | Hyperhemolysis is a life-threatening condition of exaggerated hemolysis of red blood cells which occurs in patients receiving chronic transfusion therapy. We present a 19-year-old male with the β-thalassemia major with an episode of hyperhemolysis. Hemolysis was initially unresponsive to immunosuppression but responded after the addition of eculizumab. Several weeks after stabilization, hemolysis returned; which was also managed with immunosuppression and eculizumab. Hyperhemolysis presents significant challenges in β-thalassemia due to the underlying dysfunctional erythropoiesis and transfusion dependence. Aggressive immunosuppression combined with eculizumab successfully slowed the hemolysis and allowed for the resumption of transfusions. |
| Databáze: | OpenAIRE |
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