Current status of treatment for primary effusion lymphoma.

[primary effusion lymphoma]

Primary effusion lymphoma (PEL ) is a rare and aggressive B-cell non-Hodgkin 's lymphoma that usually presents with malignant effusions without tumor masses . An extracavitary or solid variant of PEL has also been described . Human herpes virus 8 /Kaposi sarcoma -associated herpes virus (HHV-8 /KSHV ) is universally associated with the pathogenesis of PEL . More than 70 % of cases occur with concurrent Epstein-Barr virus infection , but its relation to the pathogenesis is unknown . Patients are found in the context of immunosuppressive states (HIV-1 infection , post-organ transplantation ). PEL is usually treated with CHOP (cyclophosphamide , doxorubicin , vincristine , and prednisone )-like chemotherapy with antiretroviral therapy if HIV-1 is positive . However , it is generally resistant to chemotherapy with a short median survival of less than 6 months . The optimal treatment for PEL has not been established yet . More intensive chemotherapy , such as dose-adjusted EPOCH (DA-EPOCH ; etoposide , prednisone , vincristine , cyclophosphamide and doxorubicin ) and CDE (cyclophosphamide , doxorubicin , etoposide ) are expected to show a favorable prognosis . Recently , the molecular steps in KSHV /HHV-8 -driven oncogenesis have begun to be revealed , and molecular targeting therapies such as proteasome , NF-κB , cytokines and surface antigens would provide evidence for their clinical use .

Diseases
Validation

Diseases presenting "lymphoma" symptom

adrenal incidentaloma

alpha-thalassemia

carcinoma of the gallbladder

cushing syndrome

dedifferentiated liposarcoma

erdheim-chester disease

erythropoietic protoporphyria

esophageal adenocarcinoma

esophageal carcinoma

familial hypocalciuric hypercalcemia

focal myositis

hirschsprung disease

hodgkin lymphoma, classical

kabuki syndrome

liposarcoma

locked-in syndrome

monosomy 21

oculocutaneous albinism

primary effusion lymphoma

severe combined immunodeficiency

systemic capillary leak syndrome

waldenström macroglobulinemia

wiskott-aldrich syndrome

This symptom has already been validated