1. Intravascular large B-cell lymphoma as a covert trigger for hemophagocytic lymphohistiocytosis complicated with capillary leak syndrome: a case report and literature review.
- Author
-
Wen J, Xu J, Ji J, Zhang W, Zheng Q, Liu T, Zheng Y, and Ma H
- Subjects
- Humans, Vincristine therapeutic use, Male, Cyclophosphamide therapeutic use, Prednisone therapeutic use, Doxorubicin therapeutic use, Doxorubicin administration & dosage, Rituximab therapeutic use, Middle Aged, Lymphohistiocytosis, Hemophagocytic diagnosis, Lymphohistiocytosis, Hemophagocytic etiology, Lymphohistiocytosis, Hemophagocytic complications, Lymphohistiocytosis, Hemophagocytic drug therapy, Capillary Leak Syndrome etiology, Capillary Leak Syndrome diagnosis, Capillary Leak Syndrome therapy, Lymphoma, Large B-Cell, Diffuse complications, Lymphoma, Large B-Cell, Diffuse drug therapy, Lymphoma, Large B-Cell, Diffuse diagnosis, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Antineoplastic Combined Chemotherapy Protocols adverse effects
- Abstract
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma. Patients with hemophagocytic lymphohistiocytosis (HLH)-associated IVLBCL variants exhibit significantly poor survival. Cytokines play pivotal roles in malignancy-associated HLH as well as in capillary leak syndrome (CLS). The pathogenesis of CLS involves hyperpermeability and transient endothelial dysfunction. Here, we report the first case of HLH-associated IVLBCL variant complicated with CLS. The patient presented with fever, refractory hypoproteinemia, hypotension and severe edema, followed by telangiectasias. Treatment with etoposide and dexamethasone and hydroxyethyl starch-based artificial colloid led to transient improvement. The diagnosis of IVLBCL was confirmed after the sixth bone marrow biopsy. Subsequently, the R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone) regimen was administered and resulted in prompt alleviation of CLS and HLH symptoms. The patient has survived for more than 6 years after combination of immunochemotherapy and autologous peripheral stem-cell transplantation. This case provides some insights into the mechanism and clinical management of IVLBCL complicated with HLH and CLS. Similar cases concerning lymphoma-associated CLSs were also reviewed., Competing Interests: The authors declare the research was conducted in the absence of any commercial or financial relationships that could be construed as potential conflicts of interest., (Copyright © 2024 Wen, Xu, Ji, Zhang, Zheng, Liu, Zheng and Ma.)
- Published
- 2024
- Full Text
- View/download PDF