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
Rok vydání: 2021
Předmět:
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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