Fatal anaplastic lymphoma kinase positive anaplastic large cell lymphoma presenting with cutaneous lesions: A case report [TURKDERM]
TURKDERM. 2017; 51(2): 52-55 | DOI: 10.4274/turkderm.76148  

Fatal anaplastic lymphoma kinase positive anaplastic large cell lymphoma presenting with cutaneous lesions: A case report

Funda Bapur Erduran1, Esra Adışen1, Nalan Akyürek2, Mehmet Ali Gürer1
1Department of Dermatology, Gazi University Faculty of Medicine, Department of Dermatology Ankara, Turkey
2Department of Pathology, Gazi University Faculty of Medicine, Department of Dermatology, Ankara, Turkey

Anaplastic large-cell lymphoma (ALCL) is a CD30-positive non-Hodgkin lymphoma of T-cell origin. It comprises approximately 3% of all non- Hodgkin lymphomas. The skin may be the primary involvement site (primary cutaneous) or systemic ALCL may affect the skin as cutaneous metastasis. In systemic ALCL, 80-85% of cases exhibit anaplastic lymphoma kinase-1 (ALK). However, primary cutaneous ALCL is typically ALK-negative. The most important prognostic marker in systemic ALCL is the expression of ALK. Positive ALK is associated with a favourable prognosis in systemic ALCL. Here, we report a case of ALK-positive ALCL with a very aggressive clinical course. Our case was evaluated for brown-violaceous nodules appearing on the trunk, groin and arm for about 3-4 weeks. In a few days after the diagnosis of ALCL, the patient died because of sepsis which was thought to be associated with ALCL.

Keywords: Anaplastic, large cell lymphoma, CD30, anaplastic lymphoma kinase, primary cutaneous


Deri lezyonları ile prezente olan fatal anaplastik lenfoma kinaz pozitif anaplastik büyük hücreli lenfoma: Bir olgu sunumu

Funda Bapur Erduran1, Esra Adışen1, Nalan Akyürek2, Mehmet Ali Gürer1
1Gazi Üniversitesi Dermatoloji Ana Bilim Dalı, Ankara
2Gazi Üniversitesi Patoloji Ana Bilim Dalı, Ankara

Anaplastik büyük hücreli lenfoma (ABHL), T hücre kökenli CD30 pozitif bir non-Hodgkin lenfomadır. Non-Hodgkin lenfomaların yaklaşık %3’ünü oluşturur. ABHL’de deri primer tutulum yeri olabileceği gibi (primer kutanöz ABHL), sistemik ABHL’nin deri metastazı şeklinde de deri tutulumu görülebilir. Sistemik ABHL’de olguların %80-85’i anaplastik lenfoma kinaz (ALK) pozitifliği göstermektedir. Primer kutanöz ABHL ise tipik olarak ALK negatiftir. Sistemik ABHL’nin en önemli prognostik belirteci ALK pozitifliğidir. ALK pozitifliği sistemik ABHL’de iyi prognozu gösterir. Burada gövde, inguinal bölge ve kolda 3-4 haftadır gelişen kahverengi-viyolase nodüler lezyonlar nedeniyle kliniğimizde değerlendirilerek ABHL tanısı koyulan ve günler içerisinde ABHL’ye bağlı geliştiği düşünülen sepsis nedeniyle kaybedilen çok agresif klinik seyirli ALK pozitif bir ABHL olgusu sunulmaktadır.

Anahtar Kelimeler: Anaplastik, büyük hücreli lenfoma, CD30, anaplastik lenfoma kinaz, primer kutanöz


Funda Bapur Erduran, Esra Adışen, Nalan Akyürek, Mehmet Ali Gürer. Fatal anaplastic lymphoma kinase positive anaplastic large cell lymphoma presenting with cutaneous lesions: A case report. TURKDERM. 2017; 51(2): 52-55

Corresponding Author: Funda Bapur Erduran, Türkiye


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