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A new subtype of large B-cell lymphoma expressing the ALK kinase and
lacking the 2; 5 translocation
G Delsol, L Lamant, B Mariame, K Pulford, N Dastugue, P Brousset, F Rigal- Huguet, T al Saati, DP Cerretti, SW Morris and DY Mason
Department of Pathology, CHU Purpan, Toulouse, France.
Seven cases of large B-cell lymphoma which define a previously unrecognized
subgroup are reported. Morphologically they are comprised of monomorphic
large immunoblast-like cells, containing large central nucleoli, which tend
to invade lymphatic sinuses. Superficially they resemble anaplastic large
cell lymphoma (ALCL) but they lack CD30. These lymphomas express epithelial
membrane antigen (as do ALCL), but also contain intracytoplasmic IgA of a
single light chain type (five cases) and an endoplasmic
reticulum-associated marker detected by antibody VS38. They lack
lineage-associated leukocyte antigens with the exception of CD4 (5 of 5
cases) and CD57 (5 of 7 cases). They are labeled by antibodies detecting
both the intracytoplasmic and extracellular regions of the ALK receptor
kinase, suggesting that they express the full-length form of this molecule.
This was confirmed by Western blotting (in the one case tested) which
showed a band of 200 kD in tumor cell lysates, and by polymerase chain
reaction (PCR) amplification of mRNA encoding intracellular and
extracellular ALK sequences (in the two cases tested). There was no
evidence by cytogenetics (one case analyzed) or reverse transcriptase-PCR
(three cases tested) of the 2; 5 translocation or the resultant NPM-ALK
gene, as is commonly found in ALCL. All but one of the patients were male
and all but one were adults, and in all but the latter case the disease
followed an aggressive course.
Volume 89,
Issue 5,
pp. 1483-1490,
03/01/1997
Copyright © 1997 by The American Society of Hematology

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