Sézary Syndrome

A 55-year-old woman with no significant medical history reported that diffuse erythematous, patchy, purpuric skin lesions over most of her body had been present for the past year. Applications of an over-the-counter corticosteroid cream helped control the pruritus but did not clear the skin lesions.

Other physical examination findings were unremarkable. The results of the initial serum chemistry were normal. Examination of a peripheral blood smear revealed abnormal-appearing lymphocytes with hyperconvoluted, cribriform nuclei. A biopsy of a skin specimen showed lymphocytes with hyperchromic, irregularly shaped nuclei in the epidermis, suggesting cutaneous T-cell lymphoma, or mycosis fungoides. Szary syndrome-a subtype of mycosis fungoides-was diagnosed.

Drs Hesham Taha, Japinder Singh, Gamil Kostandy, Osama Ayad, Maged Ghaly, and David Dosik of New York Methodist Hospital in Brooklyn comment that Szary syndrome is a relatively rare neoplasm that accounts for only about 5% of cutaneous T-cell lymphoma cases. The precise cause of the disease is not clear; various theories implicate infectious agents, oncogenes, cytokines, and occupational exposures, but case-controlled studies have not proved a link.

The disease is characterized by laboratory findings of peripheral blood mononuclear cell leukocytosis with at least 1000/µL atypical T cells that have hyperconvoluted cribriform nuclei (Szary cells). Most likely, these cells come from the affected skin, because they are seldom found in large numbers in bone marrow. Clinical features often present in Szary syndrome are edema, diffuse adenopathy, hepatomegaly, alopecia, and palmar-plantar keratoderma.²

Cutaneous T-cell lymphoma and Szary syndrome progress from patch to plaque to tumor stages. At the earliest stage of disease, the skin is slightly scaly and there are erythematous, flat patches. As the patches become more infiltrated, they evolve into palpable plaques, which generally are erythematous and scaly with well-marginated borders. Both the patches and the plaques have an asymmetric distribution, are broad and frequently pruritic, and may involve the palms and/ or soles. The tumor phase signals neoplastic infiltrate extending below the upper dermis.³

Localized patches and plaques usually are treated with ultraviolet irradiation. Patients who have patches or plaques covering more than 10% of skin and those in the tumor phase are treated with total skin electron beam radiotherapy. Systemic chemotherapy is used when there is visceral involvement. Patients in the patch or plaque stage without lymph node, blood, or visceral involvement have a median survival of more than 12 years. Those with skin tumors and no affected internal organs may survive 5 years. The median survival time drops to 2 to 5 years if the viscera are involved.⁴

REFERENCES:1. Barcos M. Mycosis fungoides: diagnosis and pathogenesis. Am J Clin Pathol. 1993;99:452-458.
2. Lorincz AL. Cutaneous T-cell lymphoma (mycosis fungoides). Lancet. 1996;347:871-876.
3. Hoppe RT, Wood GS, Abel EA. Mycosis fungoides and the Szary syndrome: pathology, staging, and treatment. Curr Probl Cancer. 1990;14:293-371.
4. Sausville EA, Eddy JL, Makuch RW, et al. Histopathologic staging at the initial diagnosis of mycosis fungoides and Szary syndrome. Definition of three distinctive prognostic groups. Ann Intern Med. 1988;109:372-382.