Epstein barr virus hemophagocytic lymphohistiocytosis related to rituximab use and immunopathogenetic insights

Sotirios G Papageorgiou; Sotirios Tsiodras; Georgios Siakallis; Efthimia Bazani; Aris Spathis; Garyfalia Poulakou; Penelope Korkolopoulou; Ioannis Panayiotides; Vasiliki Pappa

doi:10.1016/j.prp.2016.10.003

Epstein barr virus hemophagocytic lymphohistiocytosis related to rituximab use and immunopathogenetic insights

Pathol Res Pract. 2016 Dec;212(12):1194-1198. doi: 10.1016/j.prp.2016.10.003. Epub 2016 Oct 17.

Authors

Sotirios G Papageorgiou¹, Sotirios Tsiodras², Georgios Siakallis², Efthimia Bazani³, Aris Spathis⁴, Garyfalia Poulakou², Penelope Korkolopoulou⁵, Ioannis Panayiotides⁴, Vasiliki Pappa³

Affiliations

¹ 2nd Department of Internal Medicine, Propaedeutic, Hematology Unit, School of Medicine, National and Kapodistrian University of Athens, "Attikon" University General Hospital, Haidari, Greece. Electronic address: sotirispapageorgiou@hotmail.com.
² 4th Department of Internal Medicine, University General Hospital "Attikon", Medical School, National and Kapodistrian University of Athens, Athens, Greece.
³ 2nd Department of Internal Medicine, Propaedeutic, Hematology Unit, School of Medicine, National and Kapodistrian University of Athens, "Attikon" University General Hospital, Haidari, Greece.
⁴ Department of Pathology, University General Hospital "Attikon", Medical School, National and Kapodistrian University of Athens, Athens, Greece.
⁵ 1st Department of Pathology, "Laikon" General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

PMID: 28340951
DOI: 10.1016/j.prp.2016.10.003

Abstract

Anti-CD20-based chemo-immunotherapeutic regimens have been suggested to assist in the management of Epstein-Barr virus (EBV)-induced hemophagocytic lymphohistiocytosis (HLH) and EBV-associated post-transplant lymphoproliferative disorders (EBV-PTLD), by reducing EBV viral load and EBV-induced inflammation. Herein we report a fatal EBV-related HLH in the context of Hodgkin lymphoma (HL)-like Richter's transformation of B chronic lymphocytic leukemia (B-CLL), two months after rituximab treatment. The complex balance between EBV driven T-cell stimulation and immunosuppressive therapy in the context of multiple immune deficits is discussed.

Keywords: Anti-CD 20; EBV; Epstein barr virus; Hemophagocytic lymphohistiocytosis; Hodgkin lymphoma; Richter’s; Rituximab.

Publication types

Case Reports

MeSH terms

Aged
Antineoplastic Agents / adverse effects*
Epstein-Barr Virus Infections / drug therapy
Epstein-Barr Virus Infections / pathology*
Hodgkin Disease / drug therapy
Hodgkin Disease / pathology*
Humans
Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy
Leukemia, Lymphocytic, Chronic, B-Cell / pathology*
Lymphohistiocytosis, Hemophagocytic / complications
Lymphohistiocytosis, Hemophagocytic / pathology*
Lymphohistiocytosis, Hemophagocytic / virology
Male
Neoplasms, Second Primary / drug therapy
Neoplasms, Second Primary / pathology
Rituximab / adverse effects*
Virus Activation

Substances

Antineoplastic Agents
Rituximab