University of Virginia Health System

Gamma-Delta Hepatosplenic T Cell Lymphoma/Leukemia

Gamma-delta hepatosplenic T cell lymphoma- Peripheral blood smear, Wright-Giemsa stain, 1000x

Gamma-delta hepatosplenic T cell lymphoma- Bone marrow aspirate smear, Wright Giemsa stain, 1000x

Description:

The size of the blasts resembles the L2 subtype. They are rather monotonous in size with relatively scant cytoplasm that is grey to light blue in color. The nucleus may be round or oval and indentations are frequent. The nuclear chromatin is immature with some clumping, and one or more prominent nucleoli are usually visible.

Immunophenotype:

Gamma-delta TCR+, CD3+, CD56+, double negative (CD4-, CD8-), and CD5- T cells

Cytogenetics:

Isochrome 7q present in most cases, often associated with trisomy 8.

Clinical Features:

Occurs in children and young adults, and more in males. Is characterized by marked hepatosplenomegaly and absence of peripheral lymphadenopathy.

Prognosis:

Aggressive with very poor prognosis despite a good initial response to chemotherapy.

General References:

• Jaffe ES, Harris NL, Stein H, Vardiman JW (Eds). World Health Organization Classification of Tumours. Pathology and Genetics of Tuours of Haematopoietic and Lymphoid Tissues. IRAC Press: Lyon 2001
• Cooke CB, Krenacs M, Stetler-Stevenson M, et al. Hepatosplenic gamma/edlta T cell lymphoma: A distinct clinicopathologic entity of cytotoxic gamma/delta T cell origin. Blood 88: 4265, 1996
• Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gamma-delta T cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood 102:4261, 2003