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Sezary Syndrome : Prognosis

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HemePath_SezarySyndrome3.jpg

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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.

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