Abstract
Multicentric Castleman's disease (MCD) is lymphoproliferative disorder characterized by systemic inflammatory symptoms such as fever and weight loss. Human herpes virus-8 (HHV-8) is thought to be a causable pathogen in all HIV-positive and some HIV-negative MCD patients. Furthermore, the term idiopathic MCD (iMCD) was recently proposed to represent a group of HIV-negative and HHV-8-negative patients with unknown etiologies. Although the international diagnostic criteria for iMCD require exclusion of infection-related disorders, autoimmune/autoinflammatory diseases and malignant/lymphoproliferative disorders to make an iMCD diagnosis, the relationships and differences between these disorders and MCD have not yet been clarified. We recently reported the first case of MCD with autoimmune lymphoproliferative syndrome (ALPS). Although ALPS was included in the iMCD exclusion criteria as an autoimmune/autoinflammatory disease according to the international diagnostic criteria, there is a lack of evidence on the association between MCD and ALPS. In this study, we review the recent understanding of MCD and discuss the possible association between MCD with ALPS.
Highlights
Multicentric Castleman’s diseaseDr Benjamin Castleman first reported Castleman’s disease (CD) in 1956 as unicentric lymphadenopathy with hyperplastic lymphoid tissue and hyalinized germinal centers.[1]
Our case of multicentric Castleman’s disease (MCD) with impaired lymphocyte apoptosis We recently reported the first case of idiopathic MCD (iMCD) with impaired T cell apoptosis.[22]
Diagnostic criteria of iMCD, both major histopathological criteria and seven minor criteria, including two clinical criteria, were met. These results suggest that our patient had iMCD
Summary
Dr Benjamin Castleman first reported Castleman’s disease (CD) in 1956 as unicentric lymphadenopathy with hyperplastic lymphoid tissue and hyalinized germinal centers.[1] This type of CD is referred to as unicentric Castleman’s disease (UCD) according to the number of affected lymph nodes. The etiology of MCD needs more exploration, because MCD patients with low serum IL-6 levels and who did not respond to anti-IL-6 therapy have been reported. Some other mediators, such as vascular endothelial growth factor (VEGF), IL-1, and tumor necrosis factora, may be involved in the pathogenesis of MCD.[9,15]
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