Journal
ANNALS OF HEMATOLOGY
Volume 102, Issue 1, Pages 45-53Publisher
SPRINGER
DOI: 10.1007/s00277-022-05081-6
Keywords
Chronic active EBV infection; Hemophagocytic lymphohistiocytosis; Gastrointestinal involvement; Therapeutics
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This case series study summarizes the clinical features, treatment, and prognosis of gastrointestinal chronic active EBV infection (CAEBV) with hemophagocytic lymphohistiocytosis (HLH). The study highlights the importance of early recognition and the possibility of salvage therapy combining surgery, novel therapeutic agents, and/or autologous hematopoietic stem cell transplantation (HSCT).
Chronic active EBV infection (CAEBV) is a lymphoproliferative disorder of T- or NK-cell type in Asian countries. CAEBV involving the gastrointestinal tract (GI CAEBV) is a rare condition with poor prognosis that may rapidly progress with hemophagocytic lymphohistiocytosis (HLH) and life-threatening complications such as GI bleeding and/or perforation. The approach to CAEBV with GI tract involvement (GI CAEBV) is still an unmet clinical need. In this case series study, we summarized the clinical features, treatment, and prognosis of seven cases of GI CAEBV with HLH, particularly focusing on its prognosis and the possible salvage therapy combining surgery, novel therapeutic agents, and/or autologous(auto-) hematopoietic stem cell transplantation (HSCT) based on successful cases from our center. GI CAEBV is often misdiagnosed as inflammatory bowel diseases and certain infections. The key to its early recognition is the integrative consideration of its systemic manifestation, serum virology, endoscopic, and imaging findings along with pathology. Surgical intervention should not be hesitated when life-threatening GI complications occur. Resection of the involved bowel segment is an effective way of controlling bleeding and reducing tumor burden. In addition to upfront allogeneic HSCT, new therapeutic modalities including PD-1 antibody and auto-HSCT may be effective in certain patients.
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