Allogeneic Hematopoietic Cell Transplant for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome
Autor: | Hemalatha G. Rangarajan, Nicole Davidson, Rolla Abu-Arja, Rajinder P.S. Bajwa, Veronika Polishchuk, Kyla Driest |
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Rok vydání: | 2021 |
Předmět: |
030203 arthritis & rheumatology
0301 basic medicine business.industry Hepatosplenomegaly Arthritis Case Report Diseases of the musculoskeletal system General Medicine medicine.disease Rash 03 medical and health sciences 030104 developmental biology 0302 clinical medicine RC925-935 Refractory Macrophage activation syndrome Immunology medicine Juvenile medicine.symptom Complication business Serositis |
Zdroj: | Case Reports in Rheumatology, Vol 2021 (2021) Case Reports in Rheumatology |
ISSN: | 2090-6897 2090-6889 |
DOI: | 10.1155/2021/9323141 |
Popis: | 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. |
Databáze: | OpenAIRE |
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