Anatomic Pathology: Hematopathology

947) This cavity based lymphoproliferative disorder is associated with and attributable to which infectious agent?

• Primary effusion lymphoma (PEL) is associated with and attributable to Kaposi sarcoma herpes virus (KSHV), also known as human herpes virus 8 (HHV-8). PEL is the most common lymphoma primarily involving body cavity fluids and is typically seen in HIV positive patients. Large B-cell lymphoma arising in HHV-8-associated multicentric Castleman disease is another lymphoma associated with and attributable to KSHV.

• Classical Hodgkin lymphoma, Burkitt lymphoma (BL), posttransplant lymphoproliferative disorders, Epstein Barr virus (EBV) positive diffuse large B-cell lymphoma (DLBCL) of the elderly, and lymphomatoid granulomatosis (LYG) are associated with and attributable to EBV (A). None of these lymphomas are cavity based. PEL can be associated with EBV; however, it is thought that EBV is a coinfection and is not required in the pathogenesis of this lymphoma.

• Some cases of lymphoplasmacytic lymphoma (LPL) and splenic marginal zone lymphoma have been associated with hepatitis C (C). Patients infected with human immunodeficiency virus (HIV) (D) are at a much higher risk to develop lymphoma, especially primary central nervous system lymphoma, BL, and DLBCL.

• Several pathogenetic mechanisms are involved in the development of lymphoma in the setting of HIV infection. Patients with PEL are typically HIV infected; however, PEL can be seen in elderly patients without HIV infection from areas with high prevalence for HHV-8 infection.

• Adult T-cell leukemia/lymphoma (ATLL) (E) is associated with and attributable to human T-lymphotropic virus (HTLV). ATLL is not a cavity based lymphoproliferative disorder.

Swerdlow SH, Campo E, Harris NL, et al: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.Lyon, France: IARC, 2008.

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