Pediatric Nephrology

, Volume 26, Issue 8, pp 1317–1320

Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent

Authors

    • Renal Division, Department of Internal MedicineBaystate Medical Center
    • Tufts University School of Medicine
  • Chirag K. Vaidya
    • Renal Division, Department of Internal MedicineBaystate Medical Center
    • Tufts University School of Medicine
  • Jiuming Ye
    • Renal Division, Department of Internal MedicineBaystate Medical Center
    • Tufts University School of Medicine
  • Jonathan Freeman
    • Department of PathologyBaystate Medical Center
    • Tufts University School of Medicine
  • Christine McKiernan
    • Department of PediatricsBaystate Medical Center
    • Tufts University School of Medicine
  • Peter R. Blier
    • Department of PediatricsBaystate Medical Center
    • Tufts University School of Medicine
  • Chester AndrzejewskiJr
    • Department of PathologyBaystate Medical Center
    • Tufts University School of Medicine
  • Michael Germain
    • Renal Division, Department of Internal MedicineBaystate Medical Center
    • Tufts University School of Medicine
  • Gregory L. Braden
    • Renal Division, Department of Internal MedicineBaystate Medical Center
    • Tufts University School of Medicine
Brief Report

DOI: 10.1007/s00467-011-1862-5

Cite this article as:
Agrawal, V., Vaidya, C.K., Ye, J. et al. Pediatr Nephrol (2011) 26: 1317. doi:10.1007/s00467-011-1862-5

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 anti-nuclear 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 ANCA-associated vasculitis with TTP.

Keywords

Thrombotic thrombocytopenic purpuraMicroscopic polyangiitisMyeloperoxidase antibodiesAntineutrophil cytoplasmic antibodiesADAMTS13

Copyright information

© IPNA 2011