Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

• Published in: International journal of hematology Vol. 90; no. 1; pp. 94 - 98
• Main Authors: Tsagarakis, Nikolaos J., Argyrou, Aspasia, Gortzolidis, Georgios, Kentrou, Nektaria, Papadhimitriou, Stefanos I., Tzanetou, Konstantina, Kakiopoulos, Georgios, Papadimitriou, Konstantinos A., Skoumi, Dimitra, Paterakis, Georgios
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01.07.2009; Springer; Springer Nature B.V
• Subjects: Aged; Antigens, CD - metabolism; Antineoplastic Combined Chemotherapy Protocols - administration & dosage; B-Lymphocytes - metabolism; Biological and medical sciences; Case Report; CD30 antigen; CD38 antigen; Chemotherapy; Cytoplasm; Epstein-Barr virus; Flow cytometry; Fluorescence in situ hybridization; Hematologic and hematopoietic diseases; Hematology; Herpes simplex; Herpesvirus; Herpesvirus 4, Human; Herpesvirus 8, Human; HIV; HIV and HHV-8; Human herpesvirus 8; Human immunodeficiency virus; Human viral diseases; Humans; Idiopathic T4 lymphocytopenia; Immunodeficiency; Infectious diseases; Kaposi's sarcoma-associated herpesvirus; KSHV negative; Lymphocytes B; Lymphoma, Primary Effusion - complications; Lymphoma, Primary Effusion - drug therapy; Lymphoma, Primary Effusion - virology; Lymphopenia - complications; Lymphopenia - drug therapy; Lymphopenia - virology; Male; Medical sciences; Medicine; Medicine & Public Health; Nuclei; Oncology; Other diseases. Hematologic involvement in other diseases; Primary effusion lymphoma; Remission; Sarcoma; Trisomy; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids; Idiopathic T4 lymphocytopenia; Primary effusion lymphoma; HIV and HHV-8/KSHV negative; Gammaherpesvirinae; Immunopathology; Leukopenia; Hematology; Herpesviridae; Idiopathic; Retroviridae; AIDS; B cell neoplasm; Malignant hemopathy; Non Hodgkin lymphoma; Immune deficiency; Lentivirus; Infection; Virus; Human herpesvirus 8; Lymphoproliferative syndrome; Viral disease; Human immunodeficiency virus; Cancer; Lymphocytopenia
• ISSN: 0925-5710; 1865-3774; 1865-3774
• DOI: 10.1007/s12185-009-0343-0

Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.