Sezary syndrome – A case report

ABSTRACT

Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of lymphoproliferative disorders characterised by monoclonal expansion of malignant T cells, primarily helper T (CD4) cells. Mycosis fungoides (MF) and its erythrodermic and leukemic variant, the Sezary syndrome (SS) are the most common clinical types of CTCL. A 48 year old female presented to medical outpatient department with complaints of increasing breathlessness and cough with expectoration of six months duration. She was a known treated case of pulmonary tuberculosis. On examination she had multiple papules and rashes all over the face neck and trunk and generalized lymphadenopathy involving bilateral axillary, cervical and inguinal nodes. Due to the presence of the characteristic triad of erythroderma, lymphadenopathy and circulating atypical lymphoid cells (Sezary cells) and immunophenotypic positivity for T helper subtype, a diagnosis of Sezary syndrome was done.