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Primer Kutanöz Difüz Büyük B Hücreli Lenfoma Olgusu: Mevcut Sınıflamalar Eşliğinde Primer Kutanöz B Hücreli Lenfomaların Gözden Geçirilmesi
Primary Cutaneous Diffuse Large B Cell Lymphoma: A Case Presentation and the Review of Primary Cutaneous B-Cell Lymphoma Classifications
Turkiye Klinikleri J Dermatol. 2019;29(3):131-7
doi: 10.5336/dermato.2019-64812
Makale Dili: TR
Tam Metin
ÖZET
B hücreli lenfomalar, lenf nodlarında ortaya çıkan non-Hodgkin lenfomaların büyük çoğunluğunu oluşturmalarına karşın, primer kutanöz lenfomaların az bir kısmını oluşturur. Primer kutanöz lenfomalar farklı klinik görünüm, histopatoloji, immünfenotip özellikler gösteren heterojen bir grup hastalıktır. Primer kutanöz B hücreli lenfomalar, tüm kutanöz lenfomaların %20-25'ini oluşturmaktadır. 'Primer kutanöz difüz büyük B hücreli lenfoma, diğer tip' oldukça nadir görülen bir kutanöz lenfoma olup, literatürde sınırlı sayıda olgu bildirilmiştir. Tanı kriterlerinin olmaması ve sınıflandırma kriterlerinin karışık/belirsiz olması nedeniyle hastalar yanlış veya geç tanı alabilmektedirler. Bu olgu sunumunda, primer kutanöz B hücreli lenfomaların oldukça nadir görülen bu tipini ayırıcı tanıda akla gelmesini vurgulamak amacıyla sunmak istedik.
Anahtar Kelimeler: B hücreli lenfoma; difüz büyük B hücreli lenfoma; deri neoplazmları
B hücreli lenfomalar, lenf nodlarında ortaya çıkan non-Hodgkin lenfomaların büyük çoğunluğunu oluşturmalarına karşın, primer kutanöz lenfomaların az bir kısmını oluşturur. Primer kutanöz lenfomalar farklı klinik görünüm, histopatoloji, immünfenotip özellikler gösteren heterojen bir grup hastalıktır. Primer kutanöz B hücreli lenfomalar, tüm kutanöz lenfomaların %20-25'ini oluşturmaktadır. 'Primer kutanöz difüz büyük B hücreli lenfoma, diğer tip' oldukça nadir görülen bir kutanöz lenfoma olup, literatürde sınırlı sayıda olgu bildirilmiştir. Tanı kriterlerinin olmaması ve sınıflandırma kriterlerinin karışık/belirsiz olması nedeniyle hastalar yanlış veya geç tanı alabilmektedirler. Bu olgu sunumunda, primer kutanöz B hücreli lenfomaların oldukça nadir görülen bu tipini ayırıcı tanıda akla gelmesini vurgulamak amacıyla sunmak istedik.
Anahtar Kelimeler: B hücreli lenfoma; difüz büyük B hücreli lenfoma; deri neoplazmları
ABSTRACT
B-cell lymphoma is the most common non-Hodgkin's lymphoma which arise in lymph nodes, but on the other hand, it comprises only a small fraction of primary cutaneous lymphomas. Primary cutaneous lymphomas are a heterogeneous group of diseases with different clinical features, histopathology and immunophenotypes. Primary cutaneous lymphomas represent 20-25% of all cutaneous lymhomas. "Primary cutaneous diffuse large B cell lymphoma, other type" is a very rare primary cutaneous lymphoma and so far, only a limited number of cases have been reported in the literature. Patients might be misdiagnosed or diagnosed late because of the vague classification criteria of the disease and/or the absence of diagnostic criteria. We would like to present this case with this rare subtype of primary cutaneous B-cell lymphoma in order to emphasize that this disease should be considered in the differential diagnosis.
Keywords: B cell lymphoma; diffuse large B cell lymphoma; skin neoplasms
B-cell lymphoma is the most common non-Hodgkin's lymphoma which arise in lymph nodes, but on the other hand, it comprises only a small fraction of primary cutaneous lymphomas. Primary cutaneous lymphomas are a heterogeneous group of diseases with different clinical features, histopathology and immunophenotypes. Primary cutaneous lymphomas represent 20-25% of all cutaneous lymhomas. "Primary cutaneous diffuse large B cell lymphoma, other type" is a very rare primary cutaneous lymphoma and so far, only a limited number of cases have been reported in the literature. Patients might be misdiagnosed or diagnosed late because of the vague classification criteria of the disease and/or the absence of diagnostic criteria. We would like to present this case with this rare subtype of primary cutaneous B-cell lymphoma in order to emphasize that this disease should be considered in the differential diagnosis.
Keywords: B cell lymphoma; diffuse large B cell lymphoma; skin neoplasms
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