Immune disorders in agnogenic myeloid metaplasia: relations to myelofibrosis

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Abstract
Tests for a dysimmune state were done in an unselected group of 67 patients with agnogenic myeloid metaplasia (AMM). The results were compared to those of 56 patients with polycythemia vera (PV). A total of 75% of AMM patients vs. 32% of PV patients had various abnormalities. The most frequent disorders among AMM patients were serum antinuclear and anti-smooth muscle autoantibodies (10.3% each), a positive test for rheumatoid factor (21.7%), a polyclonal increase in serum Ig levels (46.8%) or a serum monoclonal component (9.7%), a positive direct Coombs'' test (19%) and an anti-I autoantibody (30%). In AMM patients there was no relationship between age, sex, importance of splenic enlargement, time from diagnosis or treatment and the present of a dysimmunity. In AMM patients, but also in PV patients, it seems that the more frequent and numerous these abnormalities the more severe is the myelofibrosis. Like other previous studies, these results suggest a lymphoid cell involvement in AMM and a role for these immune disorders in the pathogenesis of myelofibrosis.