Sèzary Syndrome-A Rarely Seen Variant of Non-Hodgkins Lymphoma
Premila De Sousa Rocha,
Department of Pathology, 3rd Floor, Dean’s Office Bldg, Goa Medical College, Bambolim, Goa, India.
|Cutaneous T-Cell Lymphomas (CTCL) is a group of T-Cell Non-Hodgkin’s Lymphomas, originating from T-cells of skin associated lymphoid tissue. Mycosis fungoides is a specific type of CTCL derived from CD4+T-cells. Sèzary Syndrome (SS) is the leukemic counterpart of Mycosis fungoides and is defined by the triad of erythroderma, generalised lymphadenopathy and presence of clonally related neoplastic T-cells (Sèzary cells) in skin, lymph nodes and peripheral blood. In addition one or more of the following criteria are required: an absolute Sèzary cell count of at least 1000/cu.mm, an expanded CD4+T-Cell population resulting in a CD4/CD8 ratio of more than 10 and/or loss of one or more T-Cell antigens. Here, the authors report a case of 63-year-old male patient who fulfilled the criteria for diagnosis of SS. Clinically, the presentation of this lesion can be mistreated and misdiagnosed as chronic dermatitis or any psoariasiform lesion. If the patient fails to respond to treatment, then it prompts an extensive search for more serious disease along with thorough investigatory approach.|
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