Akademik Veri Yönetim Sistemi
Turkiye Klinikleri Dermatoloji, cilt.30, sa.2, ss.56-62, 2020 (Scopus)
Primary cutaneous lymphomas are a heterogeneous group of cutaneous T and B cell lymphomas. They present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Cutaneous T cell lymphomas represent approximately 80% and cutaneous B cell lymphomas represent approximately 20% of all primary cutaneous lymphomas. Clinical properties, histopathology, immunophenotyping and gene rearrangement analyzes are evaluated for the diagnosis. Mycosis fungoides represents the most common type of cutaneous T cell lymphomas and presents in the skin as patches, plaques and tumors. Histopathological examination reveals atypical lymphocytes, epidermotropism and Pautrier microabscesses. However, when histopathological findings and immunophenotyping are not characteristic, diagnosis of early mycosis fungoides could be difficult. Sezary syndrome is characterized by erythroderma and Sezary cells are present in the skin, lymph nodes and peripheral blood. Primary cutaneous CD30+ lymphoproliferative disorders represent the second most common group of cutaneous T cell lymphomas. For other rare primary cutaneous T cell lymphomas, differential diagnosis is made with clinical, histopathological and immunophenotyping features. Primary cutaneous marginal zone lymphoma, primary cutaneous follicle center lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg type are 3 types of primary cutaneous B-cell lymphomas. Staging is important for the follow up, prognosis and treatment of the patients with primary cutaneous lymphomas. Staging of mycosis fungoides and Sezary syndrome is based on TNMB classification. Clinical features and surface area of the lesions are important for the evaluation of the skin. Excisional biopsy is recommended for examination of abnormal lymph nodes. Prognosis regarding lymph nodes is related to effaced nodal architecture. TNM classification is used for staging other primary cutaneous lymphomas and central lymph nodes are also evaluated.