Intravascular large B-cell lymphoma of the nasal turbinate presenting with hemophagocytic lymphohistiocytosis: a case report and literature review
10.3760/cma.j.cn121090-20250303-00108
- VernacularTitle:鼻甲血管内大B细胞淋巴瘤伴噬血细胞淋巴组织细胞增生症1例报告并文献复习
- Author:
Xinyu ZHANG
1
;
Lu HE
;
Shuying MA
;
Yanping LIU
;
Chongyang DING
;
Lei FAN
;
Jianyong LI
;
Yi MIAO
Author Information
1. 南京医科大学第一附属医院（江苏省人民医院）血液科，南京 210029
- Publication Type:Journal Article
- From:
Chinese Journal of Hematology
2025;46(11):1064-1067
- CountryChina
- Language:Chinese
-
Abstract:
Intravascular large B cell lymphoma (IVLBCL) is a rare, aggressive subtype of diffuse large B cell lymphoma, with nasal turbinate involvement being uncommon. We report a 51-year-old woman with a 1-month history of fever of unknown origin. Laboratory findings showed cytopenia, hypertriglyceridemia, elevated ferritin, increased soluble CD25, and bone marrow hemophagocytosis. No infectious cause was identified. PET-CT revealed abnormal 18F-fluorodeoxyglucose (FDG) uptake in the nasal turbinates. Turbinate biopsy revealed tumor cells localized predominantly within vascular lumens, positive for CD20, BCL6, PAX5, and MUM1, with a Ki-67 index >60%, confirming a diagnosis of IVLBCL with hemophagocytic lymphohistiocytosis (HLH). The patient received one cycle of the DEP regimen (liposomal doxorubicin, etoposide, and methylprednisolone) for HLH, followed by five cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) and consolidation with auto-HSCT, achieving sustained complete remission. IVLBCL outcomes are heterogeneous; early diagnosis and prompt treatment improve survival, and R-CHOP plus auto-HSCT may be an effective strategy.
