Abstract:
:ALK-positive large B-cell lymphoma is a rare subtype of lymphoma, and most cases follow an aggressive clinical course with a poor prognosis. We examined an ALK-positive large B-cell lymphoma case showing an anti-ALK immunohistochemistry pattern distinct from those of 2 known ALK fusions, CLTC-ALK and NPM-ALK, for the presence of a novel ALK fusion; this led to the identification of SQSTM1-ALK. SQSTM1 is an ubiquitin binding protein that is associated with oxidative stress, cell signaling, and autophagy. We showed transforming activities of SQSTM1-ALK with a focus formation assay and an in vivo tumorigenicity assay using 3T3 fibroblasts infected with a recombinant retrovirus encoding SQSTM1-ALK. ALK-inhibitor therapies are promising for treating ALK-positive large B-cell lymphoma, especially for refractory cases. SQSTM1-ALK may be a rare fusion, but our data provide novel biological insights and serve as a key for the accurate diagnosis of this rare lymphoma.
journal_name
Haematologicajournal_title
Haematologicaauthors
Takeuchi K,Soda M,Togashi Y,Ota Y,Sekiguchi Y,Hatano S,Asaka R,Noguchi M,Mano Hdoi
10.3324/haematol.2010.033514subject
Has Abstractpub_date
2011-03-01 00:00:00pages
464-7issue
3eissn
0390-6078issn
1592-8721pii
haematol.2010.033514journal_volume
96pub_type
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