[Sézary-Baccaredda syndrome].

Abstract

One case of Sezary Syndrome is presented and studied from the clinical, histopathological and hematological point of view. Clinically the patient presented besides keratodermia on the palms and a severe pruritus, an erythrodermia of very ostensive redness, like the so-called Hallopeau's red man. On the skin, by histological examination, it was observed an infiltration with some round cells, whose nucleus were hyperchromatic and irregular, as it is to be seen in the Sezary's cells. A lymph node taken from the cervical region did not show any specific infiltrate but only a dermopathic adenopathy. Lymphoid cells of folded nucleus were revealed by the hemogram, quite suggestive of Sezary's lymphoma. In the marrow, the normal cells kept on their regular proportion, but some abnormal and invasive ones stand out with their convoluted nucleus, quite suggestive of Sezary's cells. These typical Sezary's cells have been found in the skin lesions, in the blood and in the marrow. The possible origin--in the skin or in the lymph nodes, but not in the marrow--of such cells is put to a summarized discussion. Finally, the Sezary's Syndrome may be an erythrodermic or leukemic variant of "morbus fungoide". Treatment on the basis of MOPP and CHOP methods with Bleomycin association has been attempted but, so far, the results have not been satisfactory in spite of an 18 month treatment.