Hodgkin's lymphoma, Epstein–Barr virus reactivation and fatal haemophagocytic syndrome

Abstract

Haemophagocytic syndrome is a serious disorder, often related to Epstein-Barr virus (EBV) or other infectious agents. Frequently an underlying immune abnormality or a T-cell lymphoma is present. The combination of haemophagocytosis and Hodgkin's lymphoma seems to be rare. A 70-yearold female with rheumatoid arthritis was admitted with constitutional symptoms, persistent fever, pancytopenia, deranged liver enzymes, lymphadenopathy and splenomegaly. A fatal coagulopathy supervened. The clinical picture and the bone marrow findings indicated a haemophagocytic syndrome and a lymph node biopsy disclosed an EBV-positive Hodgkin's lymphoma. EBV serology pointed at viral reactivation and a high EBV DNA content was detected in serum by real-time quantitative PCR analysis (5.5 · 10 6 copies per mL). The case history is presented and the literature is reviewed.

Hemoperfusion for Hodgkin Lymphoma-associated Hemophagocytic Lymphohistiocytosis

“…Those reports revealed that the mixed cellularity type and lymphocytedepleted type are two major types of Hodgkin lymphoma that accompany HLH. In addition, most cases showed Epstein-Barr virus to be present in the Hodgkin cells or Reed-Sternberg cells (4,5,10). Consistent with these findings, the pathological diagnosis of our patient was the mixed cellularity type in which Reed-Sternberg cells were positive for EBER1 staining.…”

“…Hodgkin lymphoma-associated HLH is rare with only a limited number of reported cases (3)(4)(5). Those reports revealed that the mixed cellularity type and lymphocytedepleted type are two major types of Hodgkin lymphoma that accompany HLH.…”

“…However, if such treatments show little or no effect on HLH, immediate mitigation of the cytokine storm is vital to save patients. In fact, patients with Hodgkin lymphoma and HLH have often shown poor prognosis due to the rapid progression of HLH even after chemotherapy (4,5).…”

Lymphocyte-depleted Hodgkin Lymphoma Complicating Hemophagocytic Lymphohistiocytosis as an Initial Manifestation: A Case Report and Review of the Literature

“…Both of these subtypes have significantly unfavorable prognoses in Western countries, as well as in Asia (15). In the past, the majority of patients with HLHassociated HL were observed to have the LD or MC subtype (11,(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28). These findings raise the possibility that EBV plays a key role in the pathogenesis of HLH.…”

Autoimmune-Associated Hemophagocytic Syndrome/Macrophage Activation Syndrome

“…In 1981, a case of T-cell lymphoma resembling MH was reported (Kadin, 1981). Following this report, many lymphoma cases associated with HPS have been reported worldwide (Han et al, 2007;Hasselblom et al, 2004;Ishii et al, 2007;Janka et al, 1998;Reiner & Spivak, 1988;Tong et al, 2008). In most of these cases it was proven that the proliferating cells were not of histiocytic origin, but that they were lymphoma cells.…”