Catalog

Primary effusion lymphoma

2A81.9

Link to WHO Definition

Primary effusion lymphoma – http://id.who.int/icd/entity/697911710

An aggressive non-Hodgkin B-cell lymphoma composed of large cells, presenting as a serous effusion without detectable tumour masses. It is universally associated with human herpes virus 8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV) [HHV-8/KSHV]. It mostly occurs in the setting of immunodeficiency; most cases have been reported in HIV positive patients. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. The prognosis is extremely unfavorable.

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