title: Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent.
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/mm(3)), 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.
authors.affiliation: Renal Division, Department of Internal Medicine, Baystate Medical Center, 759 Chestnut Street, Springfield, MA 01199, USA. varunagrawal1996@yahoo.com
authors.forename: Varun
authors.initials: V
authors.lastname: Agrawal
authors.forename: Chirag K
authors.initials: CK
authors.lastname: Vaidya
authors.forename: Jiuming
authors.initials: J
authors.lastname: Ye
authors.forename: Jonathan
authors.initials: J
authors.lastname: Freeman
authors.forename: Christine
authors.initials: C
authors.lastname: McKiernan
authors.forename: Peter R
authors.initials: PR
authors.lastname: Blier
authors.forename: Chester
authors.initials: C
authors.lastname: Andrzejewski
authors.forename: Michael
authors.initials: M
authors.lastname: Germain
authors.forename: Gregory L
authors.initials: GL
authors.lastname: Braden
mesh_terms: D000293:Adolescent; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000503:physiopathology* / Q000628:therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010951:Plasma Exchange; D011696:Purpura, Thrombocytopenic / Q000150:complications* / Q000503:physiopathology* / Q000628:therapy
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents