Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia.
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.
Tsagarakis
Nikolaos J
NJ
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece. nikolaostsagarakis@gmail.com.
Argyrou
Aspasia
A
Department of Clinical Hematology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Gortzolidis
Georgios
G
Department of Clinical Hematology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Kentrou
Nektaria
N
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece.
Papadhimitriou
Stefanos I
SI
Department of Laboratory Hematology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Tzanetou
Konstantina
K
Department of Microbiology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Kakiopoulos
Georgios
G
Department of Pathology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Papadimitriou
Konstantinos A
KA
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece.
Skoumi
Dimitra
D
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece.
Paterakis
Georgios
G
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece.
D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001402:B-Lymphocytes / Q000378:metabolism; D006678:HIV; D004854:Herpesvirus 4, Human; D019288:Herpesvirus 8, Human; D006801:Humans; D054685:Lymphoma, Primary Effusion / Q000150:complications* / Q000188:drug therapy / Q000821:virology; D008231:Lymphopenia / Q000150:complications* / Q000188:drug therapy / Q000821:virology; D008297:Male
D015703:Antigens, CD