Haploidentical hematopoietic stem cell transplantation for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Abstract

To assess the efficacy of haploidentical hematopoietic stem cell transplantation (HSCT) in adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), we retrospectively analyzed 30 cases that presented at our institution. At the time of HSCT, 20 patients (66.7%) had achieved a response after receiving HLH-94 or salvage therapies. All patients underwent myeloablative conditioning followed by peripheral blood HSCT from their related, haploidentical donors. Twenty-six patients (86.7%) achieved donor cell engraftment. Of these, 23 (88.5%) achieved complete chimerism and three (11.5%) demonstrated mixed chimerism. Reactivated EBV infection was found in 25 (96.2%). Acute graft-versus-host disease occurred in 18 (69.2%), with grade I-II in 11 patients and grade III-IV in seven. Chronic graft-versus-host disease occurred in six (23.1%). Nineteen patients survived until the end of follow-up. The three-year overall survival rate was 63.3%. Our results indicate that haploidentical HSCT is an effective treatment for adult patients with EBV-HLH.

• Authors: Zhihui Li, Yini Wang, Jingshi Wang, Jia Zhang, Zhao Wang
• Journal: Leukemia & lymphoma (Leuk Lymphoma) Vol. 59 Issue 1 Pg. 77-84 (01 2018) ISSN: 1029-2403 [Electronic] United States
• PMID: 28573910 (Publication Type: Journal Article)

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Immunosuppressive Agents
• R-CHOP protocol
• Rituximab
• Vincristine
• Doxorubicin
• Cyclophosphamide
• Prednisone

Topics

• Adolescent
• Adult
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Cause of Death
• Combined Modality Therapy
• Cyclophosphamide (therapeutic use)
• Doxorubicin (therapeutic use)
• Epstein-Barr Virus Infections (complications)
• Female
• Graft Survival
• Graft vs Host Disease (etiology, prevention & control)
• Hematopoietic Stem Cell Mobilization
• Hematopoietic Stem Cell Transplantation (adverse effects, methods)
• Histocompatibility Testing
• Humans
• Immunosuppressive Agents (therapeutic use)
• Living Donors
• Lymphohistiocytosis, Hemophagocytic (diagnosis, etiology, mortality, therapy)
• Male
• Middle Aged
• Prednisone (therapeutic use)
• Rituximab
• Transplantation Chimera
• Transplantation, Homologous
• Treatment Outcome
• Vincristine (therapeutic use)
• Virus Diseases (etiology)
• Young Adult