Vojislav Arnovljević described “Sézary syndrome” ten years before Sézary and Bouvrain

Abstract

In 1938 Sézary and Bouvrain reported on a patient with a set of symptoms which later began to carry an eponymous designation “Sézary syndrome.” Ten years previously, Vojislav Arnovljević had described a patient with exactly the same set of symptoms, as well as physical, laboratory, autopsy, and histopathology findings. Unfortunately, his contribution remained unnoticed, not only by the international but Serbian audience as well. In 1928, in the Serbian Archives of Medicine, in Serbian, Vojislav Arnovljević published an article titled “The chronic lymphoid leukaemia with skin lymphomatosis,” in which he described a 43-year-old man with a two-year history of progressive development of a diffuse erythroderma with itching and hair loss over the entire torso, leukemia of 240,000/mm³, 91% of which lymphocytes and 5% eosinophils, who soon after admission developed a bronchopulmonary infection and died. The autopsy showed a pronounced lymphadenopathy in axillae, chest, and abdomen, enlarged liver and spleen with multiple infiltrates and thick skin. The histology confirmed a profound lymphocyte infiltration of axillar, mediastinal and abdominal lymph nodes, as well as liver, spleen and skin, while “the reaction of the other parts of the lymph and blood systems was relatively weak.” There is more than enough clinical, laboratory, autopsy and histological evidence to support that the patient Arnovljević described in 1928 had a syndrome that ten years later was described by Sézary and Bouvrain, which now bears the eponymous designation of Sézary syndrome.

Keywords: erythroderma; itching; lymphadenopathy; lymphoid leukemia; eosinophilia; hepatosplenomegaly