To the Editor: A 2-y-old female presents with a 2-mo his-tory of quotidian fever, rash, lymphadenopathy, and arthritis. After excluding infections and malignancies, systemic juvenile idiopathic arthritis (sJIA) was diagnosed… Click to show full abstract
To the Editor: A 2-y-old female presents with a 2-mo his-tory of quotidian fever, rash, lymphadenopathy, and arthritis. After excluding infections and malignancies, systemic juvenile idiopathic arthritis (sJIA) was diagnosed and treated with methotrexate and corticosteroids. Four weeks later, macrophage activation syndrome (MAS) appeared, thus methylprednisolone and tocilizumab (TCZ) were administered. Urticaria developed during TCZ infusions, progress-ing to anaphylaxis, and was, therefore, discontinued. A reduced-intensity conditioning (RIC) regimen autologous stem-cell transplant (SCT) was considered. Based on a study in type 1 diabetes conducted by our group stem cells were mobilized with cyclophosphamide (1.5 g/m 2 /d) for 2 d and granulocyte colony-stimulating factor (G-CSF) (10 mcg/kg/d) for 6 d. On day 10 after chemo-stimulation, 9 × 10 6 CD34/kg were recovered by apheresis. RIC con-sisted of cyclophosphamide (500 mg/m 2 /d) and fludarabine (25 mg/m 2 /d) from day −4 to
               
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