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Home :: Sezary's Syndrome
Sezary's Syndrome
Sezary's syndrome is a rare special variant of cutaneous T cell lymphoma (CTCL) (mycosis fungoides) characterized by universal erythroderma, peripheral lymphadenopathy, and cellular infiltrates of atypical lymphocytes (Sezary cells) in the skin and in the blood. The disease may arise de novo or, less commonly, result from extension of a preexisting circumscribed CTCL. It usually occurs in patients older than 60 years and more commonly in males than in females.
Patients appear sick, shivering, and scared and there is generalized scaling erythroderma with considerable thickening of the skin. Because of the bright red color, the syndrome has been called the "red man syndrome". There is diffuse hyperkeratosis of palms and soles, diffuse hair loss which can lead to baldness, and generalized lymphadenopathy.
Causes of Sezary's Syndrome
The tumor cell is a small lymphocyte with cerebriform nucleus, clumped chromatin and inconspicuous nucleoli. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabsecsses is the hallmark of the disease.
Symptoms of Sezary's Syndrome
The disease usually shows cutaneous patches, plaques, tumors or generalized erythroderma (Mycosis fungoides). Pruritus is a common symptom. Extracutaneous manifestations are more frequent in the presence of locally advanced disease (cutaneous tumors). The presence of erythroderma with circulating malignant cells (Sezary's cells) in the peripheral blood (PB) and in the bone marrow is consistent with Sezary's syndrome, which is usually associated with lymphadenopathy
Diagnosis
The three features are erythroderma, generalized lymphadenopathy, and presence of increased numbers of atypical lymphocytes in the
buffy coat.
Treatment
As in CTCL, plus appropriate supportive measures required for erythroderma.
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