Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Varun Agrawal & Chirag K. Vaidya & Jiuming Ye & Jonathan Freeman & Christine McKiernan & Peter R. Blier & Chester Andrzejewski Jr & Michael Germain &
- چاپ و سال / کشور: 2011
Description
Thrombotic thrombocytopenic purpura (TTP) rarely occurs with systemic vasculitis. A 17-year-old girl presented with non-bloody diarrhea, menorrhagia, and syncope. She had severe anemia (hemoglobin=3.8 g/dl), thrombocytopenia (platelet=7,000/mm3), and acute kidney injury (serum creatinine, Cr=2.3 mg%). Peripheral smear examination confirmed the presence of microangiopathic hemolytic anemia. Additionally, she had a positive antinuclear antibody (1:1600) and normal complement levels. We considered the diagnosis of TTP, possibly associated with systemic lupus erythematosus, and promptly initiated pulse methylprednisolone and daily 3–4 l of plasma exchange therapy. Following resolution of her thrombocytopenia in 48 h, we performed a kidney biopsy that revealed diffuse proliferative, focal crescentic, and necrotizing glomerulonephritis with mild IgG immunofluorescence staining. Concomitantly, autoimmune work-up was significant for positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA=1:640) and decreased von Willebrand factor cleaving protease activity (<5%). A final diagnosis of TTP with microscopic polyangiitis (p-ANCA-mediated) was made and treatment with daily oral cyclophosphamide and prednisone resolved her renal injury over 2 months (follow-up Cr=1.0 mg%). Our case highlights the importance of identifying systemic disorders such as ANCAassociated vasculitis with TTP.
Pediatr Nephrol (2011) 26:1317–1320 DOI 10.1007/s00467-011-1862-5 Received: 26 August 2010 / Revised: 7 February 2011 / Accepted: 9 February 2011 / Published online: 16 April 2011
