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The bone marrow fibrosis of hairy-cell leukemia is caused by the synthesis
and assembly of a fibronectin matrix by the hairy cells
J Burthem and JC Cawley
Department of Haematology, University of Liverpool, UK.
Hairy-cell leukemia (HCL) is a proliferation of clonal B-lymphocytes with
features of activation. The disease has a number of distinctive
characteristics, prominent among which is the fine reticulin fibrosis
invariably present in the bone marrow. However, fibroblast infiltration has
never been noted in the marrow and the origin of the fibrosis has not been
established. The present studies show that the hairy cells (HCs) of HCL
produce an insoluble matrix of fibronectin (FN) in vitro. FN synthesis was
shown by the appearance of cellular FN on the surface of cells cultured in
serum-free medium and by immunoprecipitation of the metabolically labeled
protein from HC aggregates. Moreover, the HCs were shown to assemble FN
into disulphide-bonded multimers. This assembly was blocked by a 70-kD
amino-terminal fragment of the molecule that blocks FN multimer formation
by fibroblasts. HCs expressed abundant VLA-5, an FN receptor not present on
normal circulating B lymphocytes, but important in matrix formation.
Furthermore, HCs were shown to adhere to an FN fragment containing the
VLA-5 binding site. It is therefore suggested that the VLA-5 of HCs is
implicated in their assembly of FN matrix. The in vivo relevance of the
findings was established by the demonstration of FN in association with
infiltrating HCs in bone marrow sections from patients with HCL. It is
concluded that the HCs synthesise and assemble an FN matrix and that this
is at least partly responsible for the bone marrow fibrosis so
characteristic of the disease.
Volume 83,
Issue 2,
pp. 497-504,
01/15/1994
Copyright © 1994 by The American Society of Hematology
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