Sezary Syndrome

Question 1

Sezary Syndrome

Answer 1

late stage of mycoses fungoides. Defined as erythroderma, lymphadenopathy and the presence of neoplastic T cells (Sezary Cells)

Question 2

Where are Sezary cells found

Answer 2

skin, lymph nodes, peripheral blood.

Question 3

Pathogenesis

Answer 3

unknown, occurs only in adults. lacking chromosomes.

Question 4

Clinical features

Answer 4

exfoliation, edema, lichenifcation, intense pruritus. Lymphadenopathy, alopecia and onychodystrophy may occur, hyperkeratosis palms and plantar surfaces is common

Question 5

Pathology

Answer 5

dense sezary cells in involved lymph nodes, peripheral blood smear with sezary cells is diagnostic

Question 6

Tx systemic

Answer 6

Extracorporeal Photopheresis, is a cell-based immunomodulatory therapy that involves collecting leukocytes from peripheral blood. These cells are exposed to a photosensitizing agent, 8-methoxypsoralen, and are then treated with ultraviolet (uv) radiation, after which they are re-infused. 30-80% response rate

Question 7

Reduce erythema and pruritus

Answer 7

PUVA and potent topical steroids