title: Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency.
abstract: OBJECTIVE
  To describe the ophthalmic and histopathological features of a female with granulomatous common variable immunodeficiency (CVID) who presented with upper-lid swelling.
  
  
  METHODS
  The patient underwent a biopsy of the right upper lid/palpebral lacrimal gland with imaging showing a left-sided nasopharyngeal mass, multiple lymph nodes within the mediastinum, bilateral lung nodules and a peritoneal nodule in the right iliac fossa. The right upper-lid swelling progressed and was subject to a second biopsy.
  
  
  RESULTS
  The first right upper-lid biopsy revealed a diffuse large B-cell lymphoma (DLBCL), confirmed with clonal IgH gene rearrangement with PCR. The nasopharyngeal mass and lymph nodes were suspected clinically to be DLBCL. However, a biopsy of the nasopharyngeal mass showed Kaposi sarcoma (KS). The second biopsy of the right upper lid/palpebral lacrimal gland revealed KS with no evidence of DLBCL.
  
  
  CONCLUSIONS
  This is the first documentation of periocular/orbital metachronous DLBCL and KS in a patient with granulomatous CVID. We discuss the role of fluctuating immunity in CVID to explain the spontaneous regression of the DLBCL and the varying clinical picture.
authors.affiliation: Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK.
authors.forename: Sophie
authors.initials: S
authors.lastname: Stenton
authors.affiliation: Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK.
authors.forename: Malee
authors.initials: M
authors.lastname: Fernando
authors.affiliation: Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, UK.
authors.forename: Zanna
authors.initials: Z
authors.lastname: Currie
authors.affiliation: National Specialist Ophthalmic Pathology Service (NSOPS), Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK.
authors.forename: Hardeep Singh
authors.initials: HS
authors.lastname: Mudhar
keywords: Common variable immunodeficiency; Kaposi sarcoma; Lacrimal gland; Lymphoma