Dermatol. praxi. 2016;10(3):152-154 | DOI: 10.36290/der.2016.035
Sézary syndrome and primary cutaneous CD30+ lymphoproliferative disorders
- FN u sv. Anny a LF MU v Brně
Sézary syndrome (SS) is a rare, but agressive variant of T-cell lymphoma. It is characterised by: erytrodermy, generalised lymfadenopathy, presence of Sézary cells in periferal blood (more than 1 000/mm3). Medical care of patient with SS is always multidisciplenary, including hematooncologists. Despite SS primary cutaneous CD 30+ lymfoproliferative deseases are indolent and are the second most common type of cutaneous T-cell lymhomas (CTCL). This group of CTCL includes primary cutaneous anaplastic large cell CD30+ T-cell lymphoma (C- ALCL) a lymphomatoid papulosis (LyP). Despite its benign course all the patients should be follwed-up lifelong, becaues of the risk of systemic progression or development of other cutaneous T-cell lymhoma.
Keywords: Sézary syndrome, primary cutaneous anaplastic large cell CD30+ T-cell lymphoma, lymphomatoid papulosis
Published: October 1, 2016 Show citation
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