Turkiye Klinikleri Journal of Dermatology

.: REVIEW
Primer Kutanöz Lenfomalarda Tanı ve Evreleme
Diagnosis and Staging in Primary Cutaneous Lymphomas
Esra PANCAR YÜKSELa
aOndokuz Mayıs Üniversitesi Tıp Fakültesi, Deri ve Zührevi Hastalıkları ABD, Samsun, TÜRKİYE
Turkiye Klinikleri J Dermatol. 2020;30(2):56-62
doi: 10.5336/dermato.2019-72771
Article Language: TR
Full Text
ÖZET
 Primer kutanöz lenfomalar, kutanöz T hücreli ve B hücreli lenfomaların heterojen bir grubudur. Tanı aldıklarında çoğunlukla deriye lokalizedirler. Yaklaşık %80'ini kutanöz T hücreli lenfomalar, %20'sini kutanöz B hücreli lenfomalar oluşturmaktadır. Tanı için klinik, histopatoloji, immünfenotiplendirme ve gen rearranjman analizleri birlikte değerlendirilmelidir. En sık görülen kutanöz T hücreli lenfoma mikozis fungoidestir. Klinik olarak yama, plak ve tümöral lezyonlar görülür. Histopatolojik incelemede atipik lenfositler, epidermotropizm, Pautrier mikroapseleri tespit edilir. Fakat histopatolojik bulgular ve immünfenotiplendirme karakteristik olmadığında, erken evre mikozis fungoideste tanı zorlukları yaşanmaktadır. Eritrodermi ile karakterize Sezary sendromunda, Sezary hücreleri deride, lenf nodunda ve periferik kanda görülür. Primer kutanöz CD30+ lenfoproliferatif hastalıklar ise kutanöz T hücreli lenfomaların 2. büyük grubunu oluşturur. Bunlar dışında nadir görülen diğer primer kutanöz T hücreli lenfomalar için klinik, histopatolojik, immünfenotiplendirme özellikleri ile ayırıcı tanıya gidilir. Primer kutanöz marjinal zon lenfoma, primer kutanöz folikül merkez hücreli lenfoma, primer kutanöz diffüz büyük B hücreli lenfoma, bacak tipi ise başlıca 3 primer kutanöz B hücreli lenfomadır. Primer kutanöz lenfoma hastalarında evreleme yapılması hastaların takibi, prognozu ve tedavisi için önemlidir. Mikozis fungoides ve Sezary sendromu için TNMB sınıflandırması kullanılır. Derinin değerlendirilmesi için lezyonların klinik özellikleri ve kapladıkları yüzey alan önemlidir. Anormal lenf nodlarının incelenmesi için eksizyonel biyopsi önerilir. Lenf nodlarında, prognoz nodal yapının silinmesi ile ilişkilidir. Diğer primer kutanöz lenfomaları evrelendirmek için TNM sınıflandırması kullanılır ve santral lenf nodları değerlendirilir.

Anahtar Kelimeler: Kutanöz B hücreli lenfoma; kutanöz T hücreli lenfoma; primer kutanöz lenfoma
ABSTRACT
 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.

Keywords: Cutaneous B-cell lymphoma; cutaneous T-cell lymphoma; primary cutaneous lymphoma
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-Information with wrong, incomplete, deceiving or immoral expressions is recorded to the website,

-Proclamation, advertisement, announcement, libelous expressions are used against natural person or legal identity,

-During various attacks to the website,

-Disruption of the website because of a virus.

Written, visual and audible materials of the website, including the code and the software are under protection by legal legislation.

Without the written consent of Ortadoğu Advertisement Presentation Publishing Tourism Education Architecture Industry and Trade Inc. the information on the website cannot be downloaded, changed, reproduced, copied, republished, posted or distributed.

All rights of the software and the design of the website belong to Ortadoğu Advertisement Presentation Publishing Tourism Education Architecture Industry and Trade Inc.

Ortadoğu Advertisement Presentation Publishing Tourism Education Architecture Industry and Trade Inc. will be pleased to hear your comments about our terms of use. Please share the subjects you think may enrich our website or if there is any problem regarding our website.

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