Hepatosplenic T-cell lymphoma

Definition

An extranodal neoplasm of T-cells of gd receptor type, which involves the spleen, liver and bone marrow.

Synonyms

Epidemiology

Rare, representing <5%of all peripheral T-cell lymphomas

Clinical features

There is marked hepatosplenomegaly without lymphadenopathy. Bone marrow involvement results in anaemia and thrombocytopenia.

Histopathology

The cells are medium size and monotonous. The liver and spleen show sinusoidal infiltration. Bone marrow involvement may be subtle and require immunohistochemistry to demonstrate.

Immunohistochemistry

CD1a
CD2	positive
CD3	positive
CD4	negative
CD5	negative
CD7	positive
CD8	negative
CD11b
CD16	positive
CD25
CD30
CD56	variable
CD57
TCR	usually ab-negative / d1-positive; rarely ab-positive
TdT
TIA-1	positive
granzyme B	negative
perforin	usually negative
ALK
EBV	negative

Differential diagnosis

• Other gd T-cell lymphomas appear not to be closely related to hepatosplenic T-cell lymphoma. See gd cutaneous lymphoma.

Prognosis

There may be initial response to chemotherapy, but relapse is the norm with a median survival of less than 2 years.

References

World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

This page last revised 19.12.2002.

©SMUHT/PW Bishop