Sezary Syndrome : Prognosis
Sezary syndrome (SS) is an aggressive neoplasm with poor prognosis. Median survival is less than 3 years and overall 5-yr survival rate is 10-30% depending on stage. Involvement of lymph nodes and internal viscera further worsens the prognosis. Overwhelming opportunistic infections are the terminal event in most cases. Important prognostic indicators include: lymph node status, absolute Sezary cell count, clinical progress of the disease, large cell transformation, and serum LDH and beta-2-microglobulin levels. This skin biopsy from a patient with SS shows small, medium-sized, and a few large lymphoid cells with convoluted nuclei.