Epstein-Barr virus-driven B Cell Proliferation with CD4+ T Cell Expansion: A Lymphomatoid Granulomatosis-like Disease Related to Hyperinterleukin-10 Secretion of Remarkably Favourable Outcome with Rituximab.

Abstract

Granulomatous lymphomatosis is an Epstein-Barr virus (EBV)-driven B cell proliferation associated with an exuberant CD4(+) T cell reaction with usually histopathological pictures of angiocentrism. So far, the characteristics of CD4(+) T cells in granulomatous lymphomatosis and the mechanism leading to their expansion remain poorly explored. We report a 56-year-old female with a past history of cold agglutinin disease, which was successfully treated with 4 weekly infusions of rituximab. She presented one year later with features of granulomatous lymphomatosis that resulted in severe lung and bone marrow infiltration. We provide evidence that CD4(+) T cell expansion was oligoclonal, involved anergic cells and did not result from an EBV-driven stimulation. Rather, it resulted possibly from a high production of interleukin-10 by immunoblastic EBV-positive B cells. The outcome was remarkably favourable with rituximab and steroids. Our results suggest that an EBV-driven B cell proliferation should be investigated in patients presenting with a CD4(+) T cells alveolitis or other systemic manifestations resulting from a CD4(+) T cell expansion. These features should prompt to introduce an immunosuppressive therapy including steroids and rituximab. Our results deserve further investigations to confirm our pathophysiological hypotheses in CD4(+) T cell expansions associated with EBV-driven B cell proliferations and to assess whether granulomatous lymphomatosis could result from comparable mechanisms.

• Authors: P Cervera, A Guihot, G Gorochov, K Lassoued, P Coppo
• Journal: Scandinavian journal of immunology (Scand J Immunol) Vol. 82 Issue 6 Pg. 532-8 (Dec 2015) ISSN: 1365-3083 [Electronic] England
• PMID: 26332210 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2015 The Foundation for the Scandinavian Journal of Immunology.

Chemical References

• Antineoplastic Agents
• IL10 protein, human
• Interleukin-10
• Rituximab

Topics

• Anemia, Hemolytic, Autoimmune (drug therapy)
• Antineoplastic Agents (therapeutic use)
• B-Lymphocytes (pathology, virology)
• CD4-Positive T-Lymphocytes (pathology, virology)
• Cell Proliferation
• Female
• Herpesvirus 4, Human (physiology)
• Humans
• Interleukin-10 (immunology)
• Lymphocyte Activation (immunology)
• Lymphomatoid Granulomatosis (immunology, pathology, virology)
• Middle Aged
• Rituximab (therapeutic use)