Immunologic abnormalities in myelofibrosis with activation of the
complement system
BR Gordon, M Coleman, P Kohen and NK Day
Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were
studied for clinical and laboratory evidence of immunologic dysfunction.
Clinical findings included the presence of arthritis, vasculitis, and
erythema nodosum. Laboratory abnormalities included the presence of
circulating immune complexes, antinuclear antibodies, positive direct
Coombs tests, elevated latex fixations, and a circulating lupus type
anticoagulant. Total hemolytic complement was markedly depressed in four
patients. Analysis of complement (C) components C1-C9 and factor B
demonstrated significant reduction of only C3 and factor B. By
crossed-immunoelectrophoresis, both C3 and factor B, but not C4, were
cleaved, indicating that C activation was occurring predominantly via the
alternative pathway. The control proteins beta 1H and C3b inactivator were
decreased in three of four patients with hypocomplementemia. These data
suggest that immunologic mechanisms associated with activation of the
complement system play an important role in the disease process of some
patients with agnogenic myeloid metaplasia with myelofibrosis.
Volume 58,
Issue 5,
pp. 904-910,
11/01/1981
Copyright © 1981 by The American Society of Hematology
