2A81.9

ICD-11 MMS 2026-01

Primary effusion lymphoma

Description

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.

Index terms

  • Primary effusion lymphoma
  • Primary effusion lymphoma associated with the human immune deficiency virus infection

Exclusions from above levels

  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2A81.0 – Primary mediastinal large B-cell lymphoma
  • 2A81.1 – Intravascular large B-cell lymphoma
  • 2A81.2 – Plasmablastic lymphoma
  • 2A81.3 – Lymphomatoid granulomatosis
  • 2A81.4 – T-cell/histiocyte rich large B-cell lymphoma
  • 2A81.5 – Primary diffuse large B-cell lymphoma of central nervous system
  • 2A81.6 – Epstein-Barr Virus-positive diffuse large B-cell lymphoma of the elderly
  • 2A81.7 – Diffuse large B-cell lymphoma associated with chronic inflammation
  • 2A81.8 – ALK-positive large B-cell lymphoma
  • 2A81.A – Primary cutaneous diffuse large B-cell lymphoma, leg type
  • 2A81.Y – Other specified diffuse large B-cell lymphomas
  • 2A81.Z – Diffuse large B-cell lymphoma, not otherwise specified

Foundation entity

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

Allowed postcoordination axes

Part of: 2A81: Diffuse large B-cell lymphomas › 02: Neoplasms