Primary effusion lymphoma
Primary effusion lymphoma
Classification & external resources
Primary effusion lymphoma (PEL) is a malignancy of B cells that is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8). In about 80% of cases, the lymphoma cells are also infected with Epstein Barr virus (EBV). PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the pericardium; another name for PEL is "body cavity lymphoma". It was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.
Additional recommended knowledge
PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS. It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a very poor prognosis.
- Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM. Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. N Engl J Med 1995;332(18):1186-91.
- Boshoff C, Weiss R. AIDS-related malignancies. Nat Rev Cancer 2002;2(5):373-82.
- Yarchoan R, Tosato G, Little RF. Therapy insight: AIDS-related malignancies - the influence of antiviral therapy on pathogenesis and management. Nature Clin Prac Oncology 2005;2(8):406-15.
- Staudt MR, Kanan Y, Jeong JH, Papin JF, Hines-Boykin R, Dittmer DP. The tumor microenvironment controls primary effusion lymphoma growth in vivo. Cancer Res 2004;64(14):4790-9.
|Hematological malignancy histology (ICD-O 9590-9989)|
|Lymphomas (9590-9759)||Hodgkin's lymphoma vs. Non-Hodgkin lymphoma - Diffuse lymphoma vs. Follicular lymphoma|
B-cell lymphoma (Small cell, Primary effusion, Diffuse large, ,Burkitt's, Splenic marginal zone, MALT)
mast cell tumor (Mast-cell sarcoma, Malignant mastocytosis, Malignant histiocytosis, Langerhans cell histiocytosis)
T-cell lymphoma (Cutaneous , Mycosis fungoides/Sézary's disease, Angioimmunoblastic, Anaplastic large cell, Hepatosplenic)
plasma cell (Plasmacytoma, Multiple myeloma)
|Immunoproliferative disorders (9760-9799)||Waldenström macroglobulinemia - Lymphomatoid granulomatosis|
|Lymphoid leukemias (9800-9839)||ALL - CLL - T-cell leukemia (Adult, Large granular lymphocyte, Prolymphocytic, Acute lymphoblastic) - B-cell leukemia (Prolymphocytic)|
|Myeloid leukemias (9840-9939, 9963)||AML (M2, APL/M3, AMoL/M5, Erythroleukemia/M6) - CML (CMoL, CNL, Philadelphia chromosome) - Granulocytic sarcoma|
|Other leukemias (9940-9949)||Hairy cell leukemia - Aggressive NK-cell leukemia|
|Myeloproliferative disease (9950-9961)||Polycythemia vera - Essential thrombocytosis - Myelofibrosis|
|Other (9964-9989)||Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome|