title: Primary Central Nervous System Immunomodulatory Therapy-Induced Lymphoproliferative Disorder in a Patient with Ulcerative Colitis: A Case Report and Review of the Literature.
abstract: BACKGROUND
  Immunosuppression and immunomodulatory therapy-induced lymphoproliferative disorders (ILPD) represent a heterogeneous group of lymphoid cell disorders that occur secondary to iatrogenic immune dysfunction, best described in the post-transplant setting.
  
  
  METHODS
  We describe a case of a primary central nervous system ILPD in a patient with ulcerative colitis treated chronically with the immunomodulatory agents infliximab and azathioprine. This 52-year-old woman presented with a 1-month history of left-side weakness and paresthesias. Neuroimaging identified multiple heterogeneously enhancing lesions in her cerebrum. Extensive systemic infectious and malignancy-related investigations were negative, prompting neurosurgical referral to obtain a tissue diagnosis. Pathologic assessment of her open excisional biopsy specimen confirmed the diagnosis of a polymorphic lymphoproliferative disorder. She was treated by withdrawal of infliximab and azathioprine, along with a prolonged course of prednisone. At her 6-month follow-up, she demonstrated both clinical and radiologic improvement.
  
  
  CONCLUSIONS
  ILPD should be considered in the differential diagnosis in patients with iatrogenic immunodeficiency presenting with neurologic symptoms and intra-axial mass lesions on neuroimaging investigations. A standard treatment regimen for ILPD remains to be determined, however withholding the immunomodulatory agents and trial of corticosteroids may be tried as a first-line option before the use of more aggressive chemotherapy and/or radiotherapy.
authors.affiliation: Division of Neurosurgery, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada. Electronic address: abdullah.alobaid@medportal.ca.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Alobaid
authors.affiliation: Department of Laboratory Medicine and Pathology, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Emina
authors.initials: E
authors.lastname: Torlakovic
authors.affiliation: Department of Laboratory Medicine and Pathology, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Paul
authors.initials: P
authors.lastname: Kongkham
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use; D003093:Colitis, Ulcerative / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000139:chemically induced / Q000188:drug therapy; D007155:Immunologic Factors / Q000009:adverse effects* / Q000627:therapeutic use; D056747:Immunomodulation; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D000069285:Infliximab / Q000009:adverse effects; D008232:Lymphoproliferative Disorders / Q000139:chemically induced* / Q000473:pathology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069285:Infliximab; D001379:Azathioprine; D011241:Prednisone
keywords: Central nervous system lymphoma; Immunosuppression; Inflammatory bowel disease; Lymphoproliferative disorders; Post-transplant lymphoproliferative disorder