Lymph node histopathology in Sézary syndrome

Twenty-six lymph node biopsy specimens from twenty-three patients with Sézary syndrome were microscopically evaluated and compared with nodal specimens from six patients with pre-Sézary syndrome and four control patients with dermatopathic lymphadenopathy. Three phases of nodal involvement by cerebriform lymphocytes were perceived. A trend toward greater effacement of lymph node tissue (phase II or phase III) could be related to longer duration of disease. Unchanged stable histologic features were seen in repeated specimens at intervals as long as 6 years. Cases of pre-Sézary syndrome were of phase 0 or phase I, with nodal histology overlapping that of many of the cases of typical Sézary syndrome. In contrast to mycosis fungoides, Sézary syndrome, with its hematogenous circulation of cerebriform cells, features early recognizable nodal infiltration. The degree of nodal involvement does not consistently correlate with the prognosis.