Epstein-Barr virus related hemophagocytic syndrome in a T-cell rich B-cell lymphoma.
We report the case of a 30-year-old woman who presented with an EBV related hemophagocytic syndrome. After a few months she developed a T-cell rich B-cell non-Hodgkin's lymphoma with liver involvement. Serological data demonstrated a reactivation of the EBV infection. Tumor progression with liver involvement occurred during treatment with conventional chemotherapy. Tumor reduction and disappearance of all masses was seen after starting high-dose sequential chemotherapy, followed by an autologous peripheral blood progenitor transplantation LMP-1 could be amplified in the tumor material by PCR technology, but no LMP-1 expression could be found in the few malignant B-cells with Reed-Sternberg morphology. Sequence analysis of the carboxy terminal of the LMP-1 region revealed the naturally occurring 30 bp deletion variant of the LMP-1 with multiple point mutations within the NF kb region. Since LMP-1 was not expressed in the malignant tumor cells, no evidence could be found, that EBV participated in the tumorigenesis of this case.
Mitterer
M
M
Department of Hematology and Bone Marrow Transplantation, Bozen, Italy. mitman@dnet.it
Pescosta
N
N
McQuain
C
C
Gebert
U
U
Oberkofler
F
F
Coser
P
P
Knecht
H
H
D000328:Adult; D002352:Carrier Proteins / Q000032:analysis; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications*; D006801:Humans; D016393:Lymphoma, B-Cell / Q000209:etiology*; D014412:Tumor Virus Infections / Q000150:complications*; D014763:Viral Matrix Proteins / Q000032:analysis
D002352:Carrier Proteins; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D014763:Viral Matrix Proteins