Multicentric Castleman disease with high IgG4 level: report of one case and review of literature / 白血病·淋巴瘤

ABSTRACT

Objective:To investigate the diagnosis and treatment of multicentric Castleman disease (MCD).Methods:The diagnosis and treatment of one MCD patient admitted in Hangzhou First People's Hospital in July 2020 was analyzed and related literatures were reviewed.Results:The patient was a 55-year-old male with anemia, elevated globulin levels and IgG4 > 10 g/L, and enlarged lymph nodes. He was undiagnosed for 7 years. Lymph node biopsy revealed a large number of polyclonal plasma cell hyperplasia, and the ratio of IgG4/IgG was less than 0.40; the serum interleukin (IL)-6 was more than 6 000 pg/ml and then he was eventually diagnosed as MCD (plasma cell type). Rituximab + cyclophosphamide + dexamethasone (RCD) regimen was not effective, and it was changed to anti-IL-6 receptor antibody tocilizumab for 2 courses and then the patient obtained good results.Conclusions:Castleman disease is a rare disease with a poor prognosis. It has high heterogeneity and is easy to be misdiagnosed clinically. The diagnosis requires pathological examination. IL-6 is considered to be closely related to the onset of Castleman disease and has become an effective target for treatment.