Secondary (AA) Amyloidosis Associated With Castleman’s Disease:Report of Two Cases and Review of the Literature

Abstract

This article describes two patients with localized Castleman's disease (CD) of the mixed hyaline vascular and plasma cell type located at the mesentery of the small bowel, associated with systemic symptoms, anemia, an increased plasma level of acute phase reactants, and systemic amyloidosis. There were amyloid deposits on the vascular walls of the liver, spleen, and mesenteric lymphoid mass. On immunohistochemical studies, amyloid was shown to be of the AA type in both cases. After surgical resection of the mesenteric mass, clinical and laboratory manifestations improved, and the amyloidosis showed no progression in these patients 23 years and 1 year later. The authors' review of the literature shows a striking similarity of CD cases associated with amyloidosis in terms of the abdominal localization of the lymphoid mass, secondary pattern (AA type) of amyloidosis, and improvement of clinical manifestations after removal of the tumor, without progression of amyloid deposition.

Treatment of castleman’s disease

“…• Castleman's disease is associated with a number of autoimmune phenomena ( Table 2) including paraneoplastic mucocutaneous disease (paraneoplastic pemphigus [8,9] and Bechet's disease [10], secondary amyloidosis [11][12][13], lymphoid interstitial pneumonitis [14], bronchiolitis obliterans [15][16][17], and a multitude of renal disorders [18][19][20]. Though CD can occur in the context of POEMS syndrome, it can also exist without the hallmark features of POEMS but with a number of other associated "paraneoplastic" symptoms.…”

Remission of Nephrotic Syndrome due to AA Amyloidosis and Initiation of Glomerular Repair after Surgical Resection of Localized Castleman’s Disease

“…Renal amyloidosis has hitherto been reported in only 11 CD patients in whom a renal biopsy was done. Amyloid deposition in other sites has been described in a further 4 CD patients without renal involvement [24, 25]and in 1 patient with mild proteinuria, in whom renal biopsy was not performed [3]. The two main types of amyloid (AA and AL) have both been described in association with CD.…”

“…The two main types of amyloid (AA and AL) have both been described in association with CD. Amyloid deposits most frequently occur in the presence of an intra-abdominal CD mass, where later diagnosis results in a longer period of fibril deposition [2, 24]. It is currently unknown whether amyloid deposits that have accumulated prior to surgical treatment of CD can be spontaneously reabsorbed.…”

Castleman’s disease imitating adrenal mass in the retroperitoneal area

“…[13][14][15] Secondary amyloidosis is more frequent especially in multicentric CD. 14,15 Biopsy is obligatory in diagnosis of the disease. Excisional biopsy is neccesary because cell samples taken in thin needle aspiration biopsy have normal appearance and may hinder the diagnosis.…”