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

Abstract

Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. A rare entity within this group is represented by hepatosplenic ␥␦ T cell lymphoma, which is characterized by primary extranodal disease with typical sinusoidal or sinusal infiltration of the liver and the spleen, respectively, by expression of the T cell receptor ␥␦ chain, and by a number of other frequent clinicopathological features including aggressive course of disease. In contrast to these common attributes some biologic characteristics such as expression of cytotoxic proteins and cytotoxic activity have been controversial. In this review, clinicopathological, immunophenotypical, molecular biological, cytogenetical and biological findings, and diagnostic and therapeutic difficulties in hepatosplenic ␥␦ T cell lymphoma are discussed. Leukemia (2000) 14, 991-997.

Hepatosplenic γδ T‐cell lymphoma in a 10‐year‐old boy successfully treated with hematopoietic stem cell transplantation

“…Most of the reported cases were in young adult males. Only three children under 15 years old were reported: 2 boys and 1 girl [3][4][5]. The 2 boys failed to respond to multi-agent chemotherapy and survived less than 24 months.…”

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

“…Solid organ transplant recipients account for a significant proportion of HSTL cases: 7 of 45 in a literature review [2] and 4 of 21 in a single institution report [3]. Chronic antigenic stimulation from transplanted allograft and the associated expansion of CD3 1 CD4 2 CD8 2 TCRab 2 T-cells may contribute to the pathogenesis [18].…”

“…Peripheral lymphadenopathy is rare. The disease is clinically aggressive: median survival was 10 months in a literature review of 45 cases [2] and 16 months in a single institution series of 21 cases [3].…”

Long-term remission in a patient with hepatosplenic γδ T cell lymphoma after cord blood stem cell transplantation following autologous peripheral blood stem cell transplantation

“…1 Despite a broad array of potential therapeutic approaches, HSgdTCL is very aggressive, with a median survival duration of o2 years. 2,3 We report successful treatment with an unrelated donor cord blood transplantation (UCBT) following autologous peripheral blood stem cell transplantation (APBSCT) in a young woman with HSgdTCL.…”