Abstract
Introduction & Objectives: Pathogenesis of primary cutaneous CD30+ T cell lymphoproliferative disorders is not clearly elucidated. Viral infection has been proposed as causative cofactors in these diseases. We report here a unique case of concurrent primary cutaneous CD30+ T-cell lymphoproliferative disorder and chronic hepatitis E virus (HEV) infection, its clinical course paralleling the viral response.
Materials & Methods: Retrospective study
Results: A 62-year old Caucasian male was diagnosed with a typical type B lymphomatoid papulosis in 2005 and subsequently with chronic HEV (genotype 3c) infection and cirrhosis in 2009. He was treated chronologically in March 2010 with ribavirin for 12 weeks, in February 2011 with ribavirin for 48 weeks then with ribavirin and interferon (as an add-on) for 40 weeks, and in May 2013 with ribavirin and interferon for 48 weeks. Remarkably, skin lesions and hematological responses paralleled the virological response. The patient remained free of any cutaneous lesion after achieving a sustained virological response. Skin biopsies and a complete hematological workup were carried out between the second and the third treatment.
The immunological workup did not detect any common cause of primary or acquired immune deficiency. Cutaneous T cell proliferation consisted of scattered large CD30+ lymphocytes surrounding blood vessels within a predominant small CD8+ infiltrate. Restricted usage of TCR V-beta of infiltrating cells was consistent with oligoclonal expansion. HEV core protein and RNA were readily detected in endothelial cells by immunofluorescence and in-situ hybridization, respectively. Furthermore, viral-like particles were detected in endothelial cells using electronic microscopy.
Conclusions: The data show for the first time a possible role of HEV infection in the pathogenesis of lymphomatoid papulosis. Extrahepatic replication sites, including skin endothelial cells, will be discussed as well as its association with CD30+ T-cell lymphoproliferative disorders. HEV screening should be part of the workup of any lymphoproliferative disorder.
Pol:Abbvie: Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Membership on an entity's Board of Directors or advisory committees; Boehringer Ingelheim: Membership on an entity's Board of Directors or advisory committees; BMS: Membership on an entity's Board of Directors or advisory committees, Research Funding; Roche: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees; Sanofi: Membership on an entity's Board of Directors or advisory committees; Gilead: Membership on an entity's Board of Directors or advisory committees, Research Funding; MSD: Membership on an entity's Board of Directors or advisory committees, Research Funding. Hermine:AB science: Consultancy, Equity Ownership, Membership on an entity's Board of Directors or advisory committees, Patents & Royalties, Research Funding, Speakers Bureau; Celgene: Research Funding; Novartis: Research Funding; Alexion: Research Funding.
Author notes
Asterisk with author names denotes non-ASH members.
