Adult T-cell lymphoma/leukaemia (ATLL) with angioimmunoblastic T-cell lymphoma (AILT)-like features

Definition

Like typical ATLL, there are atypical cells in the peripheral blood and there is positivity for anti-HTLV1 antibody. The AILT-like features consist of arborising high endothelial venules, clear neoplastic cells and a mixed inflammatory infiltrate. Unlike the usual AILT, there is negativity for BCL and CD10 with a lack of CD21-positive FDCs.

Epidemiology

This variant occurs across a wide adult age range with a mean of 61 years.

Clinical features

There is commonly lymphadenopathy and may be hepatosplenomegaly. Other AILT-related features such as skin rashes and polyclonal hypergammaglobulinaemia are lacking. Hypercalcaemia is uncommon. Many patients show circulating atypical "flower" cells.

Histopathology

Lymph nodes show AILT-like features: effaced architecture, infiltration by small lymphocytes and blasts, clear cells, plasma cells and histiocytes with a prominent vascular network. The clear cells may form sheets around venules. Infiltration by eosinophils is limited or absent. Sequential biopsies may show progression from AILT-like to typical ATLL morphology.

Immunohistochemistry

	ATLL with AILT-like features	typical ATLL	typical AITL
CD4	11/111
CD8	0/111
CD10	0/111	0/101	7/101
CD21	0/111	0/101	8/101
CD25	10/111
BLC lymphocyte chemokine	0/111	0/101	6/101
CCR4	9/111
CXCR3	2/111
TIA-1	positive in reactive small lymphocytes1

Prognosis

This is an aggressive lymphoma with poor prognosis, much worse than is usual for typical AILT.

References

1 Karube K, Suzumiya J, Okamoto M, et al. Adult T-cell lymphoma/leukemia with angioimmunoblastic T-cell lymphomalike features: Report of 11 cases. Am J Surg Pathol 2007; 31:216-23

This page last revised 17.2.2007.