Sezary syndrome initially presenting as psoriasis vulgaris, partial response after 6 cycles of gemcitabine and radiotherapy: A rare case report

Aprizal Satria; Erty Sundarita

doi:10.22146/inajbcs.v57i3.Supplement.24265

Aprizal Satria Trainee of Haematologic Oncologic Internal Medicine, Department of Internal Medicine, Faculty of Medicine, Sriwijaya University/Mohammad Hoesin General Hospital, Palembang, South Sumatera, Indonesia
Erty Sundarita Division of Hematology and Oncology, Department of Internal Medicine, Faculty of Medicine, Sriwijaya University/Mohammad Hoesin General Hospital, Palembang, South Sumatera, Indonesia

DOI: https://doi.org/10.22146/inajbcs.v57i3.Supplement.24265

Keywords: Sezary syndrome, Sezary cell, limfoprolferatif disease, gemcitabine, radiotherapy

Abstract

Sezary syndrome (SS) is a variant of Cutaneous T-Cell Lymphoma, a rare type of lymphoma in adults. The incidence of SS is estimated epidemiologically at around 2-3% of all primary cutaneous lymphomas. SS has not been reported in Mohammad Hoesin General Hospital Palembang. Here, we report one such case. A 45-year-old female presented with generalized pruritus for 2 years. She had a history of psoriasis, previously treated with secukinumab, methotrexate, and methylprednisolone for 1 year. Physical examination showed a painful lymphadenopathy and hyperpigmentation macules on the skin. Skin biopsy revealed lymphoproliferative lesions. PET-Scan showed multiple lymphadenopathies. Immunohistochemistry (IHC) examination revealed CD3, CD4 positive diffuse, with K167 positive in 20% of tumor cells, supporting a T-cell lymphoproliferative lesion. Examination of peripheral blood images found large atypical lymphocytes (3/100 cells) and indented nuclei with basophilia, suggestive of lymphoproliferative disease suspicious for SS. Leukemia phenotyping showed large atypical lymphocytes with indented nuclei suggestive of Sezary cells, a CD4+: CD8+ ratio: 25: 1, and CD4+/CD7 ratio of 70.41% of total T lymphocytes, consistent with SS markers. SS can mimic several mild dermatoses such as psoriasis vulgaris, or initially present as psoriasis vulgaris and later progress to erythrodermic skin lesions due to plaque extension. The triad of SS consists of pruritic erythroderma, lymphadenopathy, and Sezary cells in the blood circulation. This patient fulfilled all three criteria of SS, with severe skin manifestations and multiple systemic symptoms, lymphadenopathy, and large atypical lymphocytes in the peripheral blood smear (3/100 cells), CD4+: CD8+ ratio: 25: 1 and CD4+/CD7 ratio of 70.41% consistent with SS markers. The therapy administered was gemcitabine chemotherapy 1000 mg/mm2 (days 1, 8, 15) and radiotherapy. The patient achieved a partial response after 6 cycles of gemcitabine and radiotherapy.