Immunodeficiency-Associated Lymphoproliferative Disorders

Abstract

Immunodeficiency- associated lymphoproliferative disorders are special group of lymphoid disorders that occur in people with primary or secondary immunodeficiency. This group includes lymphoproliferative diseases associated with primary immune disorders, lymphomas associated with HIV infection, post-transplant lymphoproliferative disorders, and other iatrogenic immunodeficiency-associated lymphoproliferative disorders. Most lymphomas that occur is HIV-positive patients are aggressive in nature and patients present with advanced disease. The most frequent lymphoma subtypes in HIV, such as Burkitt lymphoma and diffuse large B-cell lymphoma also occur in immunocompetent patients. Other rare NHL lymphomas, such as primary effusion lymphoma, plasmablastic lymphoma and HHV-8-positive DLBCL are characteristically seen in HIV. Post-transplant lymphoproliferative disorders (PTLD) develop in recipients of a solid organ or stem cell allograft. Morphologically, PTLD represent a spectrum of diseases from non-destructive, reactive appearing lesions to frank lymphomas. One of the most important etiologic factors associated with PTLD is Epstein-Barr virus. Iatrogenic immunodeficiency-associated lymphoproliferative disorders occur in patients who are treated with immunosuppressive/immunomodulatory agents, most commonly for autoimmune disease. Agents associated with development of lymphoproliferative disorders include methotrexate, TNF-α inhibitors, azathioprine, 6-mercaptopurine, tacrolimus, mycophenolate mofetil, and fludarabine. Morphologically, iatrogenic lymphoproliferative disorders are a spectrum ranging from PTLD-like polymorphic proliferations to frank B- and T-cell non-Hodgkin lymphomas, as well as classic Hodgkin lymphoma, and Hodgkin-like lesions.