Plasma exchange in refractory autoimmune anemia in a child with systemic vasculitis associated with homozygote beta thalassemia.
A four-year-old girl presenting with fever and purpuric lesions was diagnosed with systemic vasculitis based on her clinical and laboratory findings. She also had homozygote beta thalassemia. Oral steroids were administered and during the course of her treatment she developed necrotizing lesions on her extremities along with severe myalgia and an autoimmune refractory anemia. Autoantibodies against the Rh antigen causing a persistent hemolysis of her erythrocytes were detected in her serum. Since no improvement in her skin lesions and autoimmune hemolytic anemia was achieved with bolus methylprednisolone therapy and cyclophosphamide, plasma exchange was performed. After three sessions of plasma exchange, her immune complex and autoantibody levels gradually declined and a remission in her clinical and laboratory findings was achieved. We suggest the use of plasma exchange along with conventional therapy for similar cases with ongoing immunologic injury.
Beşbaş
N
N
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Ozen
S
S
Bakkaloğlu
A
A
Gürgey
A
A
Kanra
T
T
Saatçi
U
U
D000753:Anemia, Refractory / Q000150:complications* / Q000276:immunology / Q000628:therapy*; D001327:Autoimmune Diseases / Q000150:complications / Q000628:therapy*; D002675:Child, Preschool; D005260:Female; D006720:Homozygote; D006801:Humans; D010951:Plasma Exchange; D014657:Vasculitis / Q000150:complications*; D017086:beta-Thalassemia / Q000150:complications* / Q000235:genetics