Transformation of Castleman Disease into Angioimmunoblastic T-Cell Lymphoma Complicated by Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review.

[INTRODUCTION] Castleman disease (CD) is a disease characterized by chronic lymphoid reactive hyperplasia. CD transforming into angioimmunoblastic T-cell lymphoma (AITL) complicated by hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal disease with rapid progression, poor treatment, and poor prognosis.

[CASE PRESENTATION] We present a case of idiopathic multicentric CD-transformed AITL complicated by HLH that achieved good therapeutic outcomes after treatment with ruxolitinib combined with CHOP regimen. The Epstein-Barr virus (EBV) in the peripheral blood of this patient remained positive throughout the course of the disease.

[CONCLUSION] EBV could be involved in the mechanism of CD transforming into AITL complicated with HLH. Ruxolitinib combined with CHOP regimen may be a potentially effective treatment for this disease.