Abstract
We report a case of Sézary syndrome demonstrating cytologic transformation from small convoluted cells to anaplastic large cells. A 43-year-old woman was admitted to our hospital because of erythroderma and a marked generalized lymphoadenopathy. The white blood cell count increased to as high as 133700/mm3. Most of the white blood cells possesed convoluted cerebriform nuclei characteristic of Sézary cells, and were CD4 positive. After she was treated with low dose methotrexate and prednisolone for one year, the white blood cell count decreased to normal values and the lymphoadenopathy almost disappeared. However, soon after the treatment was discontinued due to pancytopenia, an enlargement of the lymph nodes, hepatosplenomegaly and skin tumors all appeared. She died of cardiac insufficiency associated with pleural effusion 6 years after the onset of the disease. Large anaplastic cells in the skin tumor developed in the late stages of disease which were also CD4 positive, and the similar cells in the pleural effusion obtained at the time of death were positive for CD30 as well.
