EBV-Associated Hodgkin's Disease in an HIV-Infected Child Presenting with a Hemophagocytic Syndrome

Abstract

An 8-years-old boy was admitted with fever of unknown origin, cervical lymphadenopathy and hepatosplenomegaly and weight loss. His mother's HIV infection was diagnosed two weeks before his hospitalization, so he was diagnosed as perinatally acquired AIDS. Serology and serial cultures were negative for viral infections, toxoplasmosis, chagas, tuberculosis and atypical mycobacterium. The patient met clinical and laboratory criteria for hemophagocytic syndrome (HS) that was confirmed on bone marrow aspirate and biopsy. A cervical lymph node biopsy was performed which was diagnosed as Hodgkin's disease (HD) diffuse fibrosis lymphocyte depletion subtype. EBERs in situ hybridization and LMP-1 immunohistochemistry on the lymph node biopsy established the EBV association. On the basis of a sequence of appearance of the clinical, laboratory and histological signs, HIV, EBV or HD may have triggered HS as the last fatal event in this pediatric patient.

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

“…In these lymphomas, EBV plays an important role in the pathogenesis of HPS. LMP-1 is considered to induce proinflammatory cytokines leading to the hemophagocytic process (11,17). In contrast, HLH is rarely associated with either B-cell lymphomas or HL.…”

“…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.…”

Hodgkin Lymphoma–Associated Hemophagocytic Syndrome: A Disorder Strongly Correlated with Epstein‐Barr Virus

“…Such an association has been reported mainly with diffuse large B cell lymphoma [5]. Case reports describing Hodgkin lymphoma (HL)-associated HPS (HL-HPS) have also been published [6][7][8][9][10]. HL is associated with EBV in 20%-80% of cases, depending on histological subtype.…”

Epstein-Barr virus associated with primary CNS lymphoma and disseminated BCG infection in a child with AIDS

“…32,33 When EBV infects B-lymphocytes, it induces cell proliferation. Such lymphoproliferative action may enable oncogenesis by increasing the probability of genetic alterations (e.g.…”