[Sjögren's syndrome, mixed cryoglobulinemia and Waldenström's purpura (author's transl)]

Abstract

A 66-year-old patient was hospitalized after the appearance of a deep ulcer in the posterior face of the right calf. She had purpuric spots on the lower limbs only, as well as parotiditis, especially on the left side, and was affected with dryness of the mouth but not of the eyes. The patient also presented, in contact with the cold, Raynaud's phenomenon. Physical examination revealed as pathologic signs: purpura of the lower limbs, a deep ulceration on the right calf, parotiditis, and a reduction in the pallaesthesia of the lower limbs. Biologically an increased sedimentation rate, elevation of the total proteins (particularly the gammaglobulins), positive antinuclear factor, reduction of the total complement and of the C3 fraction, circulating immunocomplexes, positive latex test, positive Sia test, and the presence of mixed cryoglobulin IgG-IgM type kappa, stood out. The patient was diagnosed as having Sjögren's syndrome, mixed cryoglobulinemia and Waldenström's purpura. Probably the two latter conditions characterized by the presence or circulating immunocomplexes are part of the same process. According to recently studies described in the literature the relationships between these three entities are discussed, as well as the possibility of developing lymphoproliferative syndromes under certain immune stimuli.