ORBİTAL MUKORMİKOZİS: İKİ OLGU SUNUMU

Mukormikozis, ölüm oranı yüksek fırsatçı bir mantar enfeksiyonudur. Orbita lokalizasyonunda, hızla kalıcı görme kaybına ve potansiyel olarak ölümcül serebral yayılıma yol açabilir. Burada diyabetik hastalara ait iki orbital mukormikozis olgusu sunulmaktadır.İlk olgu 70 yaş erkek hastanın göze yabancı cisim gelmesi şikayetiyle acil servise başvurması ile başlamaktadır. Hasta muayene ve opere edilmiş olup daha sonrasında gözde şişme şikayetiyle tekrar hastaneye başvurmuştur. Bunun üzerine hasta tekrar opere edilmiş ve tarafımıza orbita, yabancı cisim ve göz kapağı kayıtlı biyopsi örnekleri gönderilmiştir. Mikroskopik incelemede orbital yumuşak dokular içerisinde kurdela benzeri görünüme sahip, düzensiz, geniş açılı dallanmalar yapan, septasız hif yapıları izlenmiştir. PAS histokimyasında hif yapıları daha belirgin olarak gözlenmiştir (Resim 1). Göz kapağı olarak gönderilen materyalde hif yapılarına eşlik eden süpüratif enflamasyon izlenmiştir. Bu morfolojik görünüm ile olguya ‘’Mukormikozis’’ tanısı verilmiştir. Hastaya daha sonra klinik gereklilik nedeniyle orbital ekzenterasyon yapılmış ve bu materyal de tarafımızca incelenmiş olup ‘’Mukormikozis’’ tanısını almıştır.İkinici olgu; 70 yaşında diabetes mellitus tanılı erkek hasta sağ gözde şişlik ve kaşıntı şikayetleriyle hastanemize başvurmuştur. Fizik muayenesinde sağ gözde tüm hareketlerde kayıp, proptosis, direkt ve indirekt ışık refleksi kaybı saptanmış olup ‘’orbital sellülit’’ klinik tanısıyla, sağ orbital ekzentrasyon uygulanmıştır. Histopatolojik incelemede; içerisinde mikroabse odakları içeren değişik şekil ve boyutlarda süpüratif granülom yapıları (Resim 2) ile özellikle abse odakları içerisinde hif yapıları izlenmiştir. Bu morfolojik bulgular eşliğinde olgu ’’Mukormikozis ’’ tanısı almıştır.Mukormikozis, saprofitik mantarların neden olduğu akut, genellikle ölümcül bir enfeksiyondur.Enfeksiyon bizim olgularımızda olduğu gibi sıklıkla kontrolsüz diyabet, lenfoma, kortikosteroid tedavisi ve radyoterapi nedeniyle immünsüpressif hastalarda ortaya çıkar. Ayrıca eşlik eden komorbiditeler ve immünsüpresyon nedeniyle COVID-19 ilişkili olarak bildirilen mukormikozis enfeksiyonları da mevcuttur. Bu nedenle immünsüpresyon yanı sıra COVID-19 enfeksiyonlu hastalarda, özellikle komorbiditesi olanlarda mukormikozis gibi mantar enfeksiyonlarının gelişme olasılığı akılda tutulmalıdır.</p

Clinicopathologic characteristics of BRAF V600K mutant malignant melanoma

Background &amp; objectives: BRAF V600K mutation, the second mostcommon mutation in malignant melanoma with a rate of 10-30%, isrelated to worse response to treatment and adverse prognosis. However,data for comparing V600K/V600E groups for the histopathologic andprognostic features are limited.Methods: A total of 23 malignant melanoma cases with BRAFV600E or BRAF V600K mutations detected by pyrosequencingin our department were retrospectively analysed. The associationsbetween the type of BRAF mutations and histopathologic, clinicaland prognostic characteristics were statistically investigated.Results: Of the 23 cases, 7 (30.4%) had V600K and 16 (69.6%)had V600E mutation. Although there was no statistical significancebetween two groups, most of the cases with V600K mutation weremale (85.7%). In BRAF V600K mutant cases, histologic type wasmostly superficial spreading melanoma (85.7%), tumour localizationwas mostly head and neck (57.1%); ulceration and regressionwere slightly higher. In BRAF V600E mutant group, the numberof mitosis (&gt;10/HPF) was higher (81.3%). V600E mutant groupwas generally more advanced (pT4) at the time of diagnosis (75%).In survival analysis, the estimated survival time was shorter inpatients with V600K mutation than those with V600E mutation(17.9 vs 33.2 months).Conclusion: Although it’s a preliminary study and no statisticalsignificance was detected due to the low number of cases, ourresults emphasize that overall survival time is almost half asshort in V600K mutant cases than those with V600E mutation.Considering the prognostic differences, since the double nucleotidechange seen in the V600K mutation(GTG to AAG) includes thesingle nucleotide change seen in the V600E mutation(GTG toGAG), it’s important to be careful in the evaluation to distinguishthese two mutations.</p

Clinicopathologic characteristics of BRAF V600K mutant malignant melanoma in comparison with V600E mutant cases: a preliminary study

Background &amp; objectives: BRAF V600K mutation, the second most common mutation in malignant melanoma with a rate of 10-30%, is related to worse response to treatment and adverse prognosis. However, data for comparing V600K/V600E groups for the histopathologic and prognostic features are limited. Methods: A total of 23 malignant melanoma cases with BRAF V600E or BRAF V600K mutations detected by pyrosequencing in our department were retrospectively analysed. The associations between the type of BRAF mutations and histopathologic, clinical and prognostic characteristics were statistically investigated. Results: Of the 23 cases, 7 (30.4%) had V600K and 16 (69.6%) had V600E mutation. Although there was no statistical significance between two groups, most of the cases with V600K mutation were male (85.7%). In BRAF V600K mutant cases, histologic type was mostly superficial spreading melanoma (85.7%), tumour localization was mostly head and neck (57.1%); ulceration and regression were slightly higher. In BRAF V600E mutant group, the number of mitosis (&gt;10/HPF) was higher (81.3%). V600E mutant group was generally more advanced (pT4) at the time of diagnosis (75%). In survival analysis, the estimated survival time was shorter in patients with V600K mutation than those with V600E mutation (17.9 vs 33.2 months). Conclusion: Although it’s a preliminary study and no statistical significance was detected due to the low number of cases, our results emphasize that overall survival time is almost half as short in V600K mutant cases than those with V600E mutation. Considering the prognostic differences, since the double nucleotide change seen in the V600K mutation(GTG to AAG) includes the single nucleotide change seen in the V600E mutation(GTG to GAG), it’s important to be careful in the evaluation to distinguish these two mutations.</p

Exploring the immunological basis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: immunohistochemical staining features of palatine tonsils

Objectives: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome during early childhood period with regular febrile attacks of sterile upper airway inflammation. The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. The aim of this study is to explore the immunological basis of PFAPA by evaluating the cellular properties of tonsils, and microbial exposition such as Helicobacter pylori on tonsillectomy materials. Methods: The paraffinized tonsil samples of 26 PFAPA and 29 control patients with obstructive upper airway disorder were compared in terms of immunohistochemical staining features including CD4, CD8, CD123, CD1a, CD20, and H. pylori. Results: The median number of CD8+ cells was 1485 (1218-1287) in PFAPA while it was 1003 (852–1261.5) in control group and the difference was statistically significant (p=0.001). Similarly, CD4+ cell counts were statistically higher in PFAPA group than control (833.5 vs 622). The ratio of CD4/CD8 did not differ between two groups; also, there was no statistically difference in terms of the other immunohistochemical staining results, such as CD20, CD1a, CD123 and H. pylori. Conclusion: This is the largest number of pediatric tonsillar tissue study of PFAPA patients in current literature and we emphasized the triggering effects of CD8+ and CD4+ T-cells on PFAPA tonsils. Key Points:• The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily.• In current study, 92.3% of our patients did not experience any attacks following operation similarly with literature.• We observed the increased number of CD4+ and CD8+ T cell counts on PFAPA tonsils compared to control group and emphasized the active role of both CD4+ and CD8+ cells localized on PFAPA tonsils in immune dysregulation.• Some other cell types evaluated in this study such as CD19+ (B cells), CD1a (dendritic cells), and CD123 (IL-3 receptors, for pluripotent stem cells) and H. pylori did not differ in PFAPA patients compared to the control group