Human herpesvirus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of 54 cases in the literature.

[primary effusion lymphoma]

To report a patient with primary effusion lymphoma who was negative for human herpesvirus-8 (HHV-8 ), human immunodeficiency virus , Epstein-Barr virus , hepatitis C virus , and hepatitis B virus , as well as review 54 reported cases of HHV-8 -unrelated primary effusion lymphoma (PEL )-like lymphoma in the literature to clarify the nature of this entity .The patients ' characteristics , clinical presentation , pathogenesis , morphologic-immunophenotypic features , clinical management , and prognosis were studied .H HV-8 -negative PEL -like lymphomas often occur in immunocompetent and elderly patients , are sometimes associated with chronic inflammation -related fluid overload , are mostly large B-cell or large B-cell with plasmacytic differentiation type , and are associated with a better prognosis .In various aspects , HHV-8 -unrelated PEL -like lymphoma is a different entity from HHV-8 -related PEL . Immunophenotype , morphology , and c-myc /8 q 24 status should be included for differential diagnosis . A test for c-myc or 8 q 24 abnormalities should be recommended for subdividing HHV-8 -unrelated PEL -like lymphoma , which may have benefits in patient management .

Diseases
Validation

Diseases presenting "immunodeficiency" symptom

adrenal incidentaloma

allergic bronchopulmonary aspergillosis

cushing syndrome

dracunculiasis

hirschsprung disease

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

kabuki syndrome

legionellosis

malignant atrophic papulosis

oculocutaneous albinism

omenn syndrome

papillon-lefÃ¨vre syndrome

primary effusion lymphoma

primary hyperoxaluria type 1

pyomyositis

severe combined immunodeficiency

sneddon syndrome

werner syndrome

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

This symptom has already been validated