Immunodeficiency-Associated Lymphoproliferative Disorders

Immunodeficiency-associated lymphoproliferative disorders include a spectrum of lymphomas that are associated with immunodeficiency states such as HIV infections, post-transplant immune suppression, and primary immune deficiency disorders. HIV-associated lymphoproliferative disorders are most common among these in India. Amongst HIV-associated lymphomas diffuse large B cell is the most common histology. These patients tend to present with advanced-stage and ‘B’ symptoms. They also frequently have extra-nodal and CNS involvement. Plasmablastic and immunoblastic lymphomas, which are seen more commonly in patients with low CD4 counts, have poor outcomes, while Burkitt lymphomas and Hodgkin lymphomas, which are usually seen in patients with higher CD4 counts, have better outcomes. Lymphomas associated with primary immune disorders and post-transplant immune suppression are usually associated with EBV infections. The use of combined anti-retroviral therapy and combination chemotherapy along with Rituximab has led to improved outcomes in patients with HIV-associated lymphomas.