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.

Membranoproliferative Glomerulonephritis in Castleman's Disease: A Systematic Review of the Literature and 2 Case Reports

“…This was for the relapse of his primary disease and also with the aim to manage renal manifestations. But the outcome of CHOP therapy was not detailed in that case (7). However, in the other 5 case reports, the therapies were effective.…”

“…Because CD with renal complications is easily clinically misdiagnosed or ignored, nephrologists must remain vigilant. There have been only reports of seven MPGN/ MPGN-like cases associated with CD, among which six reports were in the English language and indexed by the PubMed database (2)(3)(4)(5)(6)(7). Together with the relevant literature review, we herein report 2 cases of MPGN lesion in multicentric type CD (MCD): one is MPGN of hyaline vascular variant (HVV) and the other is MPGN of mixed variant.…”

Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman’s disease: A case report

“…CD may be associated with Human Immunodeficiency Virus (HIV) or Human Herpes Virus-8 (HHV-8) infections [7]. Autoimmune disorders may be present as systemic manifestations of CD, and the occurrence of hematological disorders, such as hemolytic anemia [8] and Evan's syndrome [9,10] have been described.…”