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Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome
  1. Hariklia Gakiopoulou1,
  2. Penelope Korkolopoulou1,
  3. Helen Paraskevakou1,
  4. Smaragdi Marinaki2,
  5. Michael Voulgarelis3,
  6. Anastasios Stofas1,
  7. Maria Lelouda1,
  8. Andreas C Lazaris1,
  9. John Boletis2,
  10. Efstratios Patsouris1
  1. 1Department of Pathology, School of Medicine, The National & Kapodistrian, University of Athens, Athens, Greece
  2. 2Department of Nephrology, Laikon Hospital, Athens, Greece
  3. 3Department of Pathophysiology, School of Medicine, The National & Kapodistrian, University of Athens, Athens, Greece
  1. Correspondence to Dr Hariklia Gakiopoulou, Department of Pathology, School of Medicine, The National & Kapodistrian University of Athens, 5 Iras str, Athens 15452, Greece; chgakiop{at}med.uoa.gr

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.

  • Castleman disease
  • membranoproliferative glomerulonephritis type I
  • Evan syndrome
  • cytokines
  • autoantibodies
  • autoimmunity
  • histopathology
  • immunofluorescence
  • lymphoid lesions

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Footnotes

  • HG & PK have equally contributed to this paper.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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