title: Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome.
abstract: Systemic Castleman's disease is a lymphoproliferative disorder with various clinical presentations and incompletely understood aetiology. The authors report on a rare case of the plasma cell variant of Castleman's disease associated with autoimmune haemolytic anaemia and autoimmune thrombocytopenia (Evan's syndrome) and complicated by mixed nephrotic-nephritic syndrome and acute renal failure due to an underlying glomerulopathy with microscopic and immunofluorescence findings suggestive of membranoproliferative glomerulonephritis (MPGN) type I. Immunocomplexed glomerulonephritis is rare in Castleman's disease, while, to the best of our knowledge, constellation of all these autoimmune phenomena is reported for the first time suggesting that apart from the putative role of VEGF and IL-6 in the pathogenesis of the disease, a more generalised immunological disturbance occurs, probably through autoantibodies induced by active polyclonal B cells raised from Castleman's disease tumour.
authors.affiliation: Department of Pathology, School of Medicine, The National & Kapodistrian, University of Athens, Athens, Greece. chgakiop@med.uoa.gr
authors.forename: Hariklia
authors.initials: H
authors.lastname: Gakiopoulou
authors.forename: Penelope
authors.initials: P
authors.lastname: Korkolopoulou
authors.forename: Helen
authors.initials: H
authors.lastname: Paraskevakou
authors.forename: Smaragdi
authors.initials: S
authors.lastname: Marinaki
authors.forename: Michael
authors.initials: M
authors.lastname: Voulgarelis
authors.forename: Anastasios
authors.initials: A
authors.lastname: Stofas
authors.forename: Maria
authors.initials: M
authors.lastname: Lelouda
authors.forename: Andreas C
authors.initials: AC
authors.lastname: Lazaris
authors.forename: John
authors.initials: J
authors.lastname: Boletis
authors.forename: Efstratios
authors.initials: E
authors.lastname: Patsouris
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology; D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology*; D005871:Castleman Disease / Q000150:complications*; D015432:Glomerulonephritis, Membranoproliferative / Q000209:etiology*; D006801:Humans; D008297:Male; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology*; D013577:Syndrome