UCD with MCD-like inflammatory state: surgical excision is highly effective

Blood Advances - Tập 5 - Trang 122-128 - 2021
Miao-yan Zhang1, Ming-nan Jia1, Jia Chen1, Jun Feng1, Xin-xin Cao1, Dao-bin Zhou1, David C. Fajgenbaum2, Lu Zhang1, Jian Li1
1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences–Peking Union Medical College, Beijing, China; and
2Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA

Tóm tắt

AbstractUnicentric Castleman disease (UCD) is a rare lymphoproliferative disorder presenting as a single nodal mass with characteristic histopathology. Patients with UCD are typically asymptomatic with normal laboratory markers, whereas patients with multicentric Castleman disease (MCD) demonstrate multicentric lymphadenopathy and cytokine storm–induced systemic inflammatory symptoms. This retrospective analysis of 116 UCD cases identified 19 (16.4%) cases with an MCD-like inflammatory state (UCD-MIS). We compared treatments and outcomes between cases of UCD-MIS and UCD–non-MIS to evaluate the role of surgery and illuminate biological behavior of UCD-MIS. There were differences in the distribution of histopathological subtypes (plasmacytic histopathology was more frequently seen, 52.6% vs 13.4%; P < .001) between the 2 groups. However, both groups demonstrated good responses to surgical treatment, suggesting that UCD-MIS in some patients still shared common biological behavior with UCD in other patients. Sixteen (94.2%) patients with UCD-MIS underwent complete surgical excision alone, and the systemic inflammation resolved completely in all of them. This high response rate suggests surgical treatment as a potential cure for this unique subset of patients. After a median follow-up duration of 64 months (range, 2-239 months), neither lymphadenopathy nor the inflammatory state recurred. However, inflammation may progress in patients with irresectable disease, and treatment options other than surgery should be considered in these patients.

Tài liệu tham khảo

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Blood Advances

Tập 5

122-128

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