Hepatosplenic gamma/delta T-Cell Lymphoma in Immunocompromised Patients

Abstract

We describe 2 male patients in whom hepatosplenic gamma/delta T-cell lymphoma (HSTL) developed 6 and 10 years after renal transplantation. The onset was abrupt with systemic symptoms, cytopenia, and hepatosplenomegaly. The histologic examination of the spleen (case 1), liver, and bone marrow revealed sinusoidal infiltrates of markedly abnormal lymphocytes. The neoplastic cells in these cases were CD2+, CD3+, CD4-, CD5-, CD7+, CD8+, CD16+, CD56+, beta F1-negative, and TIA-1-negative. Both cases displayed clonal rearrangement of the T-cell receptor (TCR) delta gene and the TCR beta gene. The spleen in case 1 was positive for Epstein-Barr virus genome and showed TCR-gamma gene rearrangement by polymerase chain reaction. Isochromosome 7 [i(7)(q10)] was found in each case. Both patients died within 4 months of diagnosis. HSTL has been reported in only 5 renal transplant recipients. HSTL may be relatively more frequent in immunocompromised patients compared with the general population.

Post‐transplant hepatosplenic T‐cell lymphoma successfully treated with hyperCVAD regimen

“…In 2 of 13 post-transplant HSTL, however, EBV could be detected in a minority of tumor cells [10,12]. In addition, EBV infection may play an indirect pathogenic role by stimulating and expanding gd T-cells.…”

“…However, in aggressive monomorphic PTLD, reducing immunosuppression alone is rarely sufficient and anthracycline-containing chemotherapy is instituted early, often as part of frontline treatment. Long-term disease-free survival rates of 50-60% can be achieved in monomorphic B-cell PTLD [21,12], but the prognosis of T-cell PTLD is generally worse. In one single institution report of four cases of T-cell PTLD, all patients died within 4 months of presentation [16].…”

Hepatosplenic T-cell lymphoma and TNF-α inhibitors

“…Although the majority of these cases seem to have developed de novo, approximately a third have been associated with immunosuppressive therapy for various reasons, mostly for organ transplantations and other conditions that require immune-modifying agents; most cases involving thiopurines [5,23,24]. Several immunocompromised conditions described in the literature include renal transplantation, heart transplantation, Hodgkin lymphoma, acute myelogenous leukemia, inflammatory bowel disease and malaria infection [4][5][6]13,[23][24][25][26][27][28][29][30][31][32].…”

Gamma/Delta T Cell Lymphoma

“…Early studies in GDTL did not demonstrate the relation to Epstein-Barr virus (EBV) infection (7,15), however GDTL had been reported recently in an immunocompromised host (16) and in posttransplant lymphoproliferative disorder (PTLD) (17) in relation to EBV infection demonstrating EBV nuclear antigen (EBNA)-2, latent membrane protein (LMP)-1 and EBVencoded small RNA (EBER) transcripts proved by in situ hybridization. In the present patient, EBV genomic DNA was detected in the spleen cells by Southern blot analysis suggesting the relation to EBV infection for the pathogenesis of GDTL.…”