Hepatosplenic gammadelta T-cell lymphoma following seven malaria infections.
Hepatosplenic gammadelta T-cell lymphoma (HSTL) is a clinicopathological entity associated with an immunocompromised status in approximately 25% of patients. Herein is described a case of HSTL in a 53-year-old Brazilian man with seven previous malaria infections, initially misdiagnosed as a hyperreactive splenomegaly due to chronic malaria. A characteristic lymphoid infiltrate was observed in spleen, liver and bone marrow sinusoids/sinuses. Neoplastic cells had a CD45RO+, CD2+, CD7+, CD3+, CD5-, CD8+, CD56+, perforin+, FasL-negative, T-cell receptor (TCR)alphabeta-negative, TCRgammadelta+ profile. Analyses of gamma and delta TCR rearrangements confirmed diagnosis of gammadelta T-cell lymphoma by detecting VgammaI/Vdelta1-Jdelta1 clonal rearrangements. Sensitive polymerase chain reaction (PCR) for Plasmodium falciparum, Epstein-Barr virus and herpesvirus-8 failed to demonstrate infection. The disease progressed to a fatal outcome following cutaneous infiltration and leukemic proliferation. The authors also comment on the association of lymphoma and infection, focusing on PCR diagnosis of TCRgamma and delta clonal rearrangements and the presumed pathogenic events leading to HSTL in the context of chronic malaria infection. Initial lymphomagenic stages might not be direct consequences of antigenic stimulation of Vdelta1 T-cells, but might depend on interactions between gammadelta T and B cells during cooperative or regulatory responses to Plasmodium sp.
Hassan
Rocío
R
Bone Marrow Transplantation Center, National Institute of Cancer, Rio de Janeiro, RJ, Brazil. biomol@inca.gov.br
Franco
Sergio A L
SA
Stefanoff
Claudio Gustavo
CG
Romano
Sergio O
SO
Diamond
Hilda R
HR
Franco
Luiz G P
LG
Seuánez
Héctor N
HN
Zalcberg
Ilana R
IR
D004273:DNA, Neoplasm / Q000032:analysis; D017809:Fatal Outcome; D016694:Gene Rearrangement, delta-Chain T-Cell Antigen Receptor; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016867:Immunocompromised Host; D016130:Immunophenotyping; D008113:Liver Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008288:Malaria / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000096:biosynthesis* / Q000235:genetics; D013160:Splenic Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*
D004273:DNA, Neoplasm; D016692:Receptors, Antigen, T-Cell, gamma-delta