Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent
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Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent Varun Agrawal & Chirag K. Vaidya & Jiuming Ye & Jonathan Freeman & Christine McKiernan & Peter R. Blier & Chester Andrzejewski Jr & Michael Germain & Gregory L. Braden
Received: 26 August 2010 / Revised: 7 February 2011 / Accepted: 9 February 2011 / Published online: 16 April 2011 # IPNA 2011
Abstract 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 signifV. Agrawal (*) : C. K. Vaidya : J. Ye : M. Germain : G. L. Braden Renal Division, Department of Internal Medicine, Baystate Medical Center, 759 Chestnut Street, Springfield, MA 01199, USA e-mail: [email protected] J. Freeman : C. Andrzejewski Jr Department of Pathology, Baystate Medical Center, Springfield, MA, USA C. McKiernan : P. R. Blier Department of Pediatrics, Baystate Medical Center, Springfield, MA, USA V. Agrawal : C. K. Vaidya : J. Ye : J. Freeman : C. McKiernan : P. R. Blier : C. Andrzejewski Jr : M. Germain : G. L. Braden Tufts University School of Medicine, Boston, MA, USA
icant for positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA=1:640) and decreased von Willebrand factor cleaving protease activity (100 U/ml) and p-ANCA (titer>1:640) were reported (serum samples were collected prior to plasma exchange). Extractable nuclear antigens (anti-Smith, anti-Ribonucleoprotein, anti-La, and anti-Ro) were negative. Additionally, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) enzyme activity level prior to TPE was severely depressed (
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