title: Treatment of rare co-occurrence of Epstein-Barr virus-driven post-transplant lymphoproliferative disorder and hemophagocytic lymphohistiocytosis after allogeneic stem cell transplantation.
abstract: In both conditions, post-transplant lymphoproliferative disorder (PTLD) and hemophagocytic lymphohistiocytosis (HLH), infection with Epstein-Barr virus (EBV) is a key mechanism: almost all PTLD in allogeneic stem cell transplantation (alloSCT) is caused by EBV-related neoplastic lymphoproliferation, and secondary HLH is most frequently triggered by EBV infection. Therefore, concomitant EBV-driven PTLD and HLH early after alloSCT require an approach to eliminate EBV and balance immune activation simultaneously. We report on a patient who developed simultaneous PTLD and signs of HLH on day 64 after alloSCT. Treatment was comprised of stopping cyclosporine, short-course dexamethasone, and 3 courses of rituximab. The patient showed full recovery and complete remission of lymphadenopathy. This result indicates that immediate reduction in EBV-carrying B cells by rituximab, suppression of general inflammation, and parallel support of reconstitution of long-term T-cell function, might be an appropriate therapeutic approach in this rare situation.
authors.affiliation: Department of Hematology and Oncology, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany.
authors.forename: T
authors.initials: T
authors.lastname: Weber
authors.forename: C
authors.initials: C
authors.lastname: Wickenhauser
authors.forename: A
authors.initials: A
authors.lastname: Monecke
authors.forename: C
authors.initials: C
authors.lastname: Gläser
authors.forename: M
authors.initials: M
authors.lastname: Stadler
authors.forename: M
authors.initials: M
authors.lastname: Desole
authors.forename: K
authors.initials: K
authors.lastname: Ligeti
authors.forename: C
authors.initials: C
authors.lastname: Behrmann
authors.forename: C
authors.initials: C
authors.lastname: Müller-Tidow
authors.forename: L P
authors.initials: LP
authors.lastname: Müller
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000821:virology*; D008297:Male; D000069283:Rituximab; D033581:Stem Cell Transplantation / Q000009:adverse effects*; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D003907:Dexamethasone
keywords: allogeneic; allogeneic stem cell transplantation; hemophagocytic lymphohistiocytosis; post-transplant lymphoproliferative disorder; rituximab