Immunosuppressive therapy with horse anti-thymocyte globulin and cyclosporine as treatment for fulminant aplastic anemia in children
Autor: | Hideki Muramatsu, Yoshiyuki Takahashi, Shouichi Ohga, Yoshitoshi Ohtsuka, Hiroyuki Shimada, Hiromasa Yabe, Kazuko Hamamoto, Ryoji Kobayashi, Seiji Kojima, Masami Inoue, Akira Ohara, Hiroshi Yagasaki, Hiroyuki Shichino |
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Rok vydání: | 2013 |
Předmět: |
Male
medicine.medical_specialty Adolescent Neutrophils Anemia Fulminant medicine.medical_treatment Hematopoietic stem cell transplantation Severity of Illness Index Gastroenterology Internal medicine Animals Humans Medicine Horses Prospective Studies Aplastic anemia Child Antilymphocyte Serum Immunosuppression Therapy Hematology business.industry Histocompatibility Testing Hematopoietic Stem Cell Transplantation Anemia Aplastic Immunosuppression General Medicine medicine.disease Survival Analysis Transplantation Treatment Outcome Child Preschool Acute Disease Immunology Cyclosporine Absolute neutrophil count Female business Immunosuppressive Agents |
Zdroj: | Annals of Hematology. 93:747-752 |
ISSN: | 1432-0584 0939-5555 |
Popis: | Patients with severe aplastic anemia (SAA) and an absolute neutrophil count (ANC) of 0 typically have fatal outcomes. We defined fulminant AA (FAA) as ANC = 0 for at least 2 weeks prior to and after immunosuppressive therapy (IST). We analyzed the outcomes of 35 children with FAA among 288 children who enrolled in a prospective study for AA (AA-97 study). AA was classified as FAA (n = 35), very SAA (vSAA; n = 129), or SAA (n = 124). All of the children received the IST with horse anti-thymocyte globulin (ATG) and cyclosporine (CsA). A significantly lower response rate at 6 months was seen in children with FAA when compared to those with vSAA or SAA (40.0, 63.6, and 63.7 %, respectively; p = 0.027). Of 20 nonresponder patients in the FAA group, 11 were rescued by alternative donor transplantation, and 5 patients showed a late response after 6 months. Consequently, no significant difference was noted in overall survival when comparing the FAA, vSAA, and SAA groups (88.5, 95.8, and 96.8 %). These findings indicate that IST with ATG and CsA is justified as a first-line treatment for children with FAA who lack a human leukocyte antigen-matched sibling donor. |
Databáze: | OpenAIRE |
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