An extranodal histopathological analysis of idiopathic multicentric Castleman disease with and without TAFRO syndrome

Abstract

Thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly (TAFRO) syndrome, a poor prognostic clinical condition showing similar histopathological findings to idiopathic multicentric Castleman disease (iMCD), has been reported in Japan. In our previous report, a clinicopathological analysis was performed on 70 nodal cases of iMCD with/without TAFRO. iMCD is classified into three types based on histopathology: (i) plasmacytic (PC), (ii) mixed, and (iii) hypervascular (hyperV). In this report, extranodal histopathological changes of iMCD with/without TAFRO were analyzed. Regarding the kidney pathology, we observed the proliferation of mesangial cells with positive staining of interleukin-6 (IL-6), consistent with membranoproliferative glomerulonephritis, in two cases of iMCD with TAFRO. The number of megakaryocytes per high-powered fields was not significantly different between iMCD cases with and without TAFRO. In conclusion, extranodal lesions of iMCD with/without TAFRO showed various interesting histopathological findings. These lesions may therefore be related to the clinical condition of TAFRO. Obtaining further knowledge about TAFRO will require the observation of nodal as well as extranodal lesions.

Keywords: Extranodal lesion; Idiopathic multicentric Castleman disease; Membranoproliferative glomerulonephritis; Syndrome; TAFRO.