Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome
Systemic juvenile idiopathic arthritis (sJIA) is characterized by arthritis, fever, rash, lymphadenopathy, hepatosplenomegaly, and serositis. Macrophage activation syndrome is the most feared complication of sJIA with a high risk of mortality. We report a 16-year-old female diagnosed with refractory...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2021/9323141 |
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| author | Nicole Davidson Hemalatha G. Rangarajan Kyla Driest Rajinder P. S. Bajwa Veronika Polishchuk Rolla F. Abu-Arja |
| author_facet | Nicole Davidson Hemalatha G. Rangarajan Kyla Driest Rajinder P. S. Bajwa Veronika Polishchuk Rolla F. Abu-Arja |
| author_sort | Nicole Davidson |
| collection | DOAJ |
| description | Systemic juvenile idiopathic arthritis (sJIA) is characterized by arthritis, fever, rash, lymphadenopathy, hepatosplenomegaly, and serositis. Macrophage activation syndrome is the most feared complication of sJIA with a high risk of mortality. We report a 16-year-old female diagnosed with refractory systemic juvenile idiopathic arthritis (sJIA) complicated by recurrent macrophage activation syndrome (MAS), severe joint disease, and lung involvement requiring prolonged immunosuppressive therapy. She received a matched unrelated allogeneic hematopoietic cell transplant (Allo-HCT) using a reduced-intensity conditioning regimen and is now, 3 years after the transplant, with complete resolution of sJIA symptoms, off immunosuppressants, and with significant improvement in the quality of life. |
| format | Article |
| id | doaj-art-70d22ca73803420fbadc94f0358d99de |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-70d22ca73803420fbadc94f0358d99de2025-08-20T03:33:57ZengWileyCase Reports in Rheumatology2090-68892090-68972021-01-01202110.1155/2021/93231419323141Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation SyndromeNicole Davidson0Hemalatha G. Rangarajan1Kyla Driest2Rajinder P. S. Bajwa3Veronika Polishchuk4Rolla F. Abu-Arja5Pediatric Residency Program, Nationwide Children’s Hospital, Columbus, OH, USAPediatric Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USADepartment of Pediatrics, The Ohio State University, OH, USAPediatric Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USAPediatric Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USAPediatric Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USASystemic juvenile idiopathic arthritis (sJIA) is characterized by arthritis, fever, rash, lymphadenopathy, hepatosplenomegaly, and serositis. Macrophage activation syndrome is the most feared complication of sJIA with a high risk of mortality. We report a 16-year-old female diagnosed with refractory systemic juvenile idiopathic arthritis (sJIA) complicated by recurrent macrophage activation syndrome (MAS), severe joint disease, and lung involvement requiring prolonged immunosuppressive therapy. She received a matched unrelated allogeneic hematopoietic cell transplant (Allo-HCT) using a reduced-intensity conditioning regimen and is now, 3 years after the transplant, with complete resolution of sJIA symptoms, off immunosuppressants, and with significant improvement in the quality of life.http://dx.doi.org/10.1155/2021/9323141 |
| spellingShingle | Nicole Davidson Hemalatha G. Rangarajan Kyla Driest Rajinder P. S. Bajwa Veronika Polishchuk Rolla F. Abu-Arja Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome Case Reports in Rheumatology |
| title | Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome |
| title_full | Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome |
| title_fullStr | Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome |
| title_full_unstemmed | Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome |
| title_short | Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome |
| title_sort | allogeneic hematopoietic cell transplant for systemic juvenile idiopathic arthritis and macrophage activation syndrome |
| url | http://dx.doi.org/10.1155/2021/9323141 |
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