Sézary syndrome: 62-year-old patient. 1 year ago first skin changes with uncharacteristic moderately itchy erythema on the trunk and extremities. Findings: Erythroderma with extensive edematous swelling of the skin; massive pruritus; taut lower legs; massive lymph node packages of the groin.
Differential diagnosis "Sezary syndrome" : non "leukemic" erythrodermic form of mycosis fungoides.
Sézary Syndrome: universal redness with small-focus recesses. small spotted scaling. massive itching, pain at times. here detailed picture of the right arm
Sézary syndrome. immunophenotyping of the peripheral blood of a patient with clinical evidence of leukemic CTCL. anti-CD3, anti-CD4 and anti-CD8 antibodies were used. CD3 was used for gating to the T cells. the subtyping shows a dramatic shift in the CD4/CD8 ratio.
Sézary syndrome. further analysis of the CD4 cells of the same patient. anti-CD3, anti-CD4 and anti-CD7/anti-CD26 antibodies were used. In this patient, mainly helper cells are found which have the marker recognition CD4+ CD7- and CD4+ CD26- respectively. These populations most likely correspond to the leukemic fraction. These cells also show a decreased CD4 expression (population shifted to the left).
Sézary Syndrome. subanalysis of CD4 cells with CD7/CD26 markers of a control proband. predominantly helper cells are found which have the marker recognition CD4+ CD7+ or CD4+ CD26+. normal ranges for CD7/CD26 positive helper cells are 80?100% and 70?100%, respectively. CD7 and CD26 are called pan-T-cell markers.
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