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Journals / Akademik Gastroenteroloji Dergisi / 2023 / Cilt: 22 Sayı: 3

Perianal Langerhans cell histiocytosis

Perianal Langerhans hücreli histiyositoz

Vedat Göral(Istanbul Medipol Üniversitesi Gastroenteroloji Bölümü. Istanbul.)0000-0002-2197-0466
Ece Altun(Istanbul Medipol Üniversitesi Dermatoloji Bölümü, Istanbul)0000-0002-8118-3405
Ferhat Özden(Istanbul Medipol Üniversitesi Patoloji Bölümü, İstanbul)0000-0002-2901-4671
Ömür Gökmen Sevindik(Istanbul Medipol Üniversitesi Hematoloji Bölümü, Istanbul)0000-0001-9636-4113
Kerem Mert Göral(Koç Üniversitesi Tıp Fakültesi, İstanbul)0009-0009-8672-1610
Pages
168–173
Publication Date
2023-12-22
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Here, we will present a case diagnosed with Langerhans cell histiocytosis after many years of treatment.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Langerhans cell histiocytosis is a rare disease with an unknown pathogenesis.
📊
Key Findings & Data:These include mutations of the BRAF, MAP2K1, RAS, and ARAF genes.
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Conclusion & Impact:Here, we will present a case diagnosed with Langerhans cell histiocytosis after many years of treatment.
🔬Design:Araştırma Makalesi
📍Scope:Comprehensive Sample
⚙️Variables:Perianal lezyon · Langerhans hücreli histiyositoz · Diabetes insipidus

Abstract

Langerhans cell histiocytosis is a rare disease with an unknown pathogenesis. It is thought that environmental factors, infections, immunological and genetic factors play a role in its etiology. Langerhans cell histiocytosis is a disorder of antigen presenting cells. It is the most common disease of the monocytic phagocytic system. Sometimes mutations (changes) develop in genes that control how dendritic cells function. These include mutations of the BRAF, MAP2K1, RAS, and ARAF genes. In the histopathology; Inflammatory cells such as T-cells, eosinophils, macrophages as well as Langerhans cells, multinucleated giant cells attract attention. The disease has a wide clinical spectrum. The clinical course varies according to the tissues and organs involved. Sometimes spontaneous recovery may occur in the clinic, as well as the disease may be progressive and fatal. Here, we will present a case diagnosed with Langerhans cell histiocytosis after many years of treatment.

Özet

Langerhans hücreli histiyositoz, nadir görülen ve patogenezi henüz tam olarak bilinmeyen bir hastalıktır. Etiyolojisinde çevresel etmenler, enfeksiyonlar, immünolojik ve genetik etkenlerin rol oynadığı düşünülmektedir. Langerhans hücreli histiyositoz, antijen sunan hücrelerin bir bozukluğudur. Monositer fagositer sistemin en sık görülen hastalığıdır. Bazen dendritik hücrelerin nasıl çalıştığını kontrol eden genlerde mutasyonlar (değişiklikler) gelişir. Bunlar BRAF, MAP2K1, RAS ve ARAF genlerinin mutasyonlarını içerir. Histopatolojisinde; Langerhans hücreleri kadar T-hücreleri, eozinofil, makrofajlar gibi inflamasyon hücreleri yanında, çok çekirdekli dev hücreler dikkati çeker. Hastalığın klinik olarak geniş bir yelpazesi vardır. Tutulan doku ve organlara göre klinik seyir çeşitlilik gösterir. Klinikte bazen kendiliğinden iyileşme olabildiği gibi, hastalık ilerleyici ve ölümcül de olabilir. Burada uzun yıllar tedavi sonunda Langerhans hücreli histiyositoz tanısı alan bir vakayı sunacağız.

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Cite This Article

Göral, V., Altun, E., Özden, F., Sevindik, Ö. G., Göral, K. M. (2023). Perianal Langerhans cell histiocytosis. Akademik Gastroenteroloji Dergisi, Cilt: 22 Sayı: 3, 168–173. https://doi.org/10.17941/agd.1408035

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