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 hyperrea...

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Bibliographic Details
Published in:Pathology international 2006-11, Vol.56 (11), p.668-673
Main Authors: Hassan, Rocío, Franco, Sergio A L, Stefanoff, Claudio Gustavo, Romano, Sergio O, Diamond, Hilda R, Franco, Luiz G P, Seuánez, Héctor N, Zalcberg, Ilana R
Format: Article
Language:English
Subjects:
DNA, Neoplasm - analysis
Fatal Outcome
Gene Rearrangement, delta-Chain T-Cell Antigen Receptor
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
Humans
Immunocompromised Host
Immunophenotyping
Liver Neoplasms - genetics
Liver Neoplasms - immunology
Liver Neoplasms - pathology
Lymphoma, T-Cell - genetics
Lymphoma, T-Cell - immunology
Lymphoma, T-Cell - pathology
Malaria - immunology
Malaria - pathology
Male
Middle Aged
Receptors, Antigen, T-Cell, gamma-delta - biosynthesis
Receptors, Antigen, T-Cell, gamma-delta - genetics
Splenic Neoplasms - genetics
Splenic Neoplasms - immunology
Splenic Neoplasms - pathology
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Summary: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.
ISSN:1320-5463
1440-1827