Diffuse large B-cell lymphoma with Clathrin-ALK fusion product

Clinical features

The three cases in one paper were in children and young adults1 A second paper reported cases in adults with a male predominance2.

Histopathology

There is a proliferation of monomorphic large cells. The adult cases showed plasmablastic differentiation.

Immunohistochemistry

CD2	0/21
CD3	0/31, 0/62
CD4	0/21, 2/6(weak positivity)2
CD5	0/31
CD15	0/21
CD20	0/31, 0/62
CD30	1/31, 0/62
CD43
CD45	0/21
CD57	1/62
CD79a	2/21, 0/62
CD138	3/31, 6/62
ALK1 (monoclonal antibody against the intracytoplasmic portion of the ALK receptor)	3/31, 6/62
EMA	1/21, 6/62
bcl-2	0/42
IgA heavy chain	3/62
demonstrable light chain restriction	4/62

Cytogenetics

The translocation t(2;17) has been reported2. FISH shows ALK gene rearrangement to produce a Clathrin-ALK fusion product.

References

1 De Paepe P, Baens M, van Krieken H, et al. ALK activation by the CLTC-ALK fusion is a recurrent event in large B-cell lymphoma. Blood 2003; 102:2638-41 FULL TEXT

2 Gascoyne RD, Lamant L, Martin-Subero JI, et al. ALK-positive diffuse large B-cell lymphoma is associated with Clathrin-ALK rearrangements: report of 6 cases. Blood 2003; 102:2568-73 FULL TEXT

3 Chikatsu N, Shinagawa A, Mori N, Suzukawa K, Nagasawa T, Kojima N. ALK-positive large B-cell lymphoma containing ALK fused to clathrin heavy chain gene, CLTC [abstract]. Blood 2002.

This page last revised 14.10.2006.