Zittoun R, Debain P, James J M, Bilski-Pasquier G
Sem Hop. 1978;54(33-36):1011-20.
The main hematological manifestations and complications seen in 18 cases of Sjögren's syndrome are described: a slight anemia of the inflammatory type, a leucopenia, and/or an eosinophilia occur in about half of the patients. One case of auto-immune hemolytic anemia, one of pernicious anemia, 3 of hypoplastic anemia--one of them drug-induced--, and one of thrombocytopenic purpura have also occurred. The clinical and biological manifestations related to the disturbed immunologic status are also frequent: hyperglobulinemic purpura. Raynaud's syndrome due to cryoglobulinemia, rheumatoid factor, anti-nuclear factor. In 4 patients, the disease presented with or evolved into lymphadenopathy and/or splenomegaly; the initial "pseudo-lymphoma" diagnosis corresponded to different aspects or courses: in one cases, malignancy appeared soon, another has evolved in a chronic lympho-proliferative disease, a third one had the histologic features of angio-immunoblastic lymphadenopathy. These various manifestations, which may reveal the Sjögren's syndrome, are discussed in the light of the litterature data.