Hepatosplenic Gamma/Delta T-Cell Lymphoma: A Report of Two Cases in Immunocompromised Patients, Associated with Isochromosome 7q

Abstract

Two cases of peripheral T-cell lymphoma, characterized by hepatosplenic presentation and gamma/delta T-cell receptor phenotype on malignant cells, are reported. Little is known about the chromosomal changes in these peculiar lymphomas. We report the cytogenetic analysis of these two patients. Isochromosome 7q and trisomy 8 were observed. These abnormalities were reported previously in five cases of gamma/delta T-cell lymphoma. These two patients had lymphomatous infiltration of the spleen, liver, bone marrow, and (in one case) lymph nodes. These abnormalities occurred in immunocompromised patients (i.e., immunosuppressive therapy for kidney transplantation and chemotherapy for Hodgkin's disease), without Epstein-Barr virus infection stigmata in tumor cells.

Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients

“…55 Eight of our patients had remarkable medical histories. Four had received long-term immunosuppressive therapy for kidney transplantation, an observation in accordance with previous reports of HS␥␦TCL as a late-onset posttransplantation lymphoproliferative disorder 7,14,15,19,22,24,56,57 of host origin. 20 Two other patients had falciparum malaria, 1 was monitored for systemic lupus erythematosus, and 1 was found to have HS␥␦TCL during therapy delivered for EBV-positive Hodgkin disease.…”

“…Parts of immunomorphologic, genotypic, and cytogenetic features of some patients in this series have been reported previously. 4,14,32,38,39 …”

“…The identification of this lymphoma subtype, recognized as a provisional entity in the Revised European American Lymphoma (REAL) classification, 26 has been further supported by its cytotoxic phenotype 11,27 and its strong association with the isochromosome arm 7q cytogenetic abnormality. 12,14,25,[28][29][30][31][32] More recently, a few cases of HSTCL with sinusoidal infiltration and an ␣␤ T-cell receptor phenotype have been reported. [33][34][35] This is now considered an immunophenotypic variant of the same disease entity in the World Health Organization (WHO) classification.…”

Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990

“…1,2,5,28 Bone marrow, if affected, is usually hypercellular with a sinusoidal distribution of the T cells. [15][16][17][18]20 Malignant hepatosplenic ␥␦ T cells are usually of small to medium size with relatively regular or folded nuclei, mostly inconspicuous nucleoli, and with a mature dispersed chromatin and a pale blue, not granulated, somewhat abundant cytoplasm. Blast-like appearance at diagnosis is rare, 18 however, terminal transformation to blast-like cells with large nucleoli has been reported several times (Refs 6, 9, 11, 20 and case 45).…”

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]. Cases of HSTCL are also associated with viral infections, including human herpes virus 6, hepatitis B virus, and Epstein-Barr virus [33,34].…”