Myelodysplastic syndromes and systemic manifestations. A non-casual association.

Purpose. - Myelodysplastic syndromes are clonal hematologic disorders, expa nded from myeloid stem cells. A primitive immunologic disorder is discussed . This hypothesis could explain a non-casual association with systemic dise ases. The aim of our study is to test this hypothesis. Methods. - We retrospectively investigated the data of 60 patients with mye lodysplastic syndromes (group I) hospitalized in our unit from 1990 to 1999 . The frequency of systemic disorders was screened and compared to controls (group II). Group II consisted of 120 patients matched for age and sex and hospitalized in the same hospital during the same period. Results. - Sixty patients were included (mean age: 83 years old). Myelodysp lastic syndrome subtypes were refractory anemia with excessive blasts (52%) , refractory anemia (43%) and sideroblastic anemia (5%). Fourteen cases of systemic manifestations were reported in group I (23%) and five in the cont rols (4%) (P < 0.0001). Systemic manifestations in group I included vasculi tis in six cases (42%), polyarthritis in three cases (21%), systemic amyloi dosis AA in two cases (14%), relapsing polychondritis in one case, pyoderma gangrenosum in one case and celiac disease associated with a systemic gran ulomatosis in one case. In the controls, vasculitis was present in four cas es and polyarthritis in one. Median age at onset of myelodysplastic syndrom e was not influenced by the association with systemic disorders which, in r eturn, have not influenced the myelodysplastic syndromes' subtypes. Myelody splastic syndromes succeeded to systemic manifestations in 71.4% of cases a nd could not be attributed to immunosuppressive therapy. Conclusions. - The association of myelodysplastic syndromes with systemic m anifestations seems not to be casual. It raises the hypothesis of a primiti ve immunological disorder in both diseases. Moreover, the description of tw o cases of systemic amyloidosis and one case of pyoderma gangrenosum might suggest an additional disorder of macrophages or granular cells. (C) 2001 E ditions scientifiques et medicales Elsevier SAS.