CASE REPORT

Isolated EBV lymphoproliferative disease in a child with Wiskott-Aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy

N J Sebire¹, S Haselden¹, M Malone¹, E G Davies² and A D Ramsay¹

¹ Department of Histopathology, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK
² Department of Immunology, Great Ormond Street Hospital for Children

Correspondence to:
Correspondence to:
Dr A D Ramsay, Department of Histopathology, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK;
a.ramsay{at}ich.ucl.ac.uk

Patients with primary immunodeficiencies such as the Wiskott-Aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. Biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.

Keywords: Wiskott-Aldrich; lymphomatoid granulomatosis; Epstein-Barr virus; lymphoproliferative disorder; immunotherapy; anti-CD20; Rituximab

Abbreviations: EBER, Epstein-Barr encoded viral RNAs; EBV, Epstein-Barr virus; ISH, in situ hybridisation; LPD, lymphoproliferative disorders; LyG, lymphomatoid granulomatosis; PTLD, post-transplant lymphoproliferative disorder; WAS, Wiskott-Aldrich syndrome

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