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Iranian Journal of Pathology، جلد ۱۹، شماره ۴، صفحات ۴۶۰-۴۶۶
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عنوان انگلیسی Diagnostic Approach to Adult Erythroderma: A Rare Case of Sezary Syndrome
چکیده انگلیسی مقاله Background & Objective: Sezary Syndrome is an uncommon leukemic variant of Cutaneous T-cell Lymphoma (CTCL), comprising only 5% of all CTCL cases. The rarity of this syndrome emphasizes the critical need to comprehend its distinct clinical presentation, diagnosis, and treatment.  
Case Presentation:  A 51-year-old man was admitted with itchy, persistent, and extensive erythematous patches, ulcers, lumps, lymphadenopathy, alopecia, and nail dystrophy that had been present for eight months. Laboratory findings showed elevated LDH and 𝛽2-microglobulin. Peripheral blood smear analysis confirmed the presence of Sezary cells, while imaging revealed multiple lymph node enlargements. Skin biopsy and immunohistochemistry suggested cutaneous T-cell lymphoma (CTCL), while immunophenotyping verified a diagnosis of  Sezary syndrome . The patient underwent fluid therapy, systemic antibiotics, topical antibiotics, phototherapy, and chemotherapy. Tenofovir was given due to the hepatitis B co-infection. Despite the improvement when discharged from the hospital, the patient's health eventually deteriorated, which led to death at home.
Conclusion: This patient presented with Sezary Syndrome, exhibiting atypical dermatologic manifestations that must be differentiated from other causes of erythroderma. This case highlights the importance of a comprehensive diagnostic approach, including clinical evaluation, laboratory tests, imaging, and biopsies. Sezary Syndrome is an inherently aggressive malignancy, characterized by a poor response to treatment and a low 5-year survival rate. 
کلیدواژه‌های انگلیسی مقاله Biopsy,Cutaneous T-cell lymphoma,Immunohistochemistry
نویسندگان مقاله Quri Meihaerani Savitri |
Department of Internal Medicine, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Fitria Rettobyan |
Department of Internal Medicine, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Linda Astari |
Department of Dermatology and Venereology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Amira Suryani Rahmatika |
Department of Dermatology and Venereology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Muhammad Noor Diansyah |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Putu Niken Ayu Amrita |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Pradana Zaky Romadhon |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Sipirianus Ugroseno Yudho Bintoro |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Ami Ashariati |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Merlyna Savitri |
Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

نشانی اینترنتی https://ijp.iranpath.org/article_720323_7b46ddeada370b3026171a574e125dfc.pdf
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