(Last Updated On: October 30, 2018)

Authors: Marie Claudine Francesca B. Perlas, MD, Johannes F. Dayrit, MD, FPDS, Maria Teresita G. Gabriel, MD, FPDS, Cara Lynn Marie N. Chia, MD

Abstract

Background: Sezary syndrome is an extremely rare leukemic cutaneous T cell lymphoma, with annual incidence of 0.3 to 1 per 100,000, characterized by triad of erythroderma, lymphadenopathy, and circulating Sézary cells in the peripheral blood.

Case Presentation: We present a case of a 64 year-old Filipino female with a 4- year history of recurrent generalized erythematous scaly patches and plaques, diagnosed as mycosis fungoides. Work-up revealed elevated LDH and peripheral blood smear showing atypical mononuclear cells with blast-like features. Blood flow cytometry revealed markedly increased total lymphocyte population and markedly increased Thelper population. CD4/CD8 ratio was elevated at 41. Skin punch biopsy revealed epidermotropism of medium-sized to large lymphocytes, with convoluted nuclei, perinuclear halo and a dermal lichenoid inflammatory infiltrate of atypical lymphocytes which were positive for the immunohistochemical marker CD3. Bone marrow flow cytometry showed bright expression of CD2, CD3, CD4, CD5, CD45 consistent with a mature T-cell neoplasm. Bone marrow biopsy revealed increased number of small to medium lymphocytes with convoluted, irregularly-shaped nuclei, conspicuous nucleoli and condensed chromatin pattern. A diagnosis of Sezary syndrome, stage IVA1 was given and she was co-managed with an oncologist for chemotherapy.

Conclusion: Sézary Syndrome is a rare and aggressive type of cutaneous T-cell lymphoma. This is the first documented case of Sezary syndrome at our institution from a total of 236 mycosis fungoides cases seen from 1998 to 2018. Prognosis is usually poor, with overall median survival of less than 3 years. Early diagnosis and appropriate treatment may be life saving.

 

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Keywords

Sezary syndrome, mycosis fungoides, prognosis, blood flow cytometry

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