Primer kutanöz lenfoma: Eortic sınıflamasına göre retrospektif ve prospektif değerlendirme

Yakın tarihlere kadar kutanöz lenfo¬malar primer veya sistemik lenfomala¬rın deri tutulumu olmaları gözönüne a-lınmaksızın nodal non-Hodgkin lenfo¬malar için kullanılan sınıflamalarla de¬ğerlendirilirlerdi. Zaman içinde primer kutanöz lenfomaların (PKL) biolojik davranışlarının farklı olduğu anlaşıldı ve her bir lenfomayı klinik, histolojik, immunohistokimyasal ve genotipik özel¬liklere dayanarak birer antite olarak kabul eden EORTC (European Organi¬sation for Research and Treatment of Cancer) sınıflaması ortaya atıldı. Proje¬de Anabilim dalımızda tanı almış 25 mikozis fungoides (MF)/Sezary send-romu (SS) dışı PKTL, 13 PKBL, 35 MF ve 8 psödolenfoma olgusu çalışma kap¬samına alınarak multidisipliner bir yak¬laşımla değerlendirildi. Anabilim Dalı¬mızda incelenen PKL'larm %82.2 si T hücreli, %17.8 i B hücreli idi. MF tüm T hücreli lenfomaların % 58.3 ünü, tüm PKL lerin. ise %47.9 unu oluşturmaktaydi. MF dışı T hücreli lenfomalarm oranı da tüm PKL'ler içinde % 34.2 idi. Histo¬lojik olarak, MF in özellikle erken dö¬nem lezyonlarmda benign likenoid ve spongiotik dermatitlerle ayırıcı tanı zor¬lukları yaşandı. Çalışma grubunda yer alan 9 lenfomatoid papüloz (LP) olgusu¬nun 6 sı tip A, 3 ü tip C olarak değerlen¬dirildi. PZR ile yo TCR genlerinde klonal yeniden düzenlenme oranı tip A da % 20, tip C de ise % 50 olarak bulun¬du. Yedi CD 30(+) PKTL nın prognozları gözönüne alındığında MF zemininde ge¬lişen 3 olgu agresif seyir gösterdi. Olgu¬lardan birinde t(2:5) saptandı. CD 30(-) PKTL tanısı almış 6 olgunun biri page-toid retiküloz zemininde gelişti. Bir di¬ğer olgunun sistemik lenfomaya dönüş¬mesi, diğerlerinde de nükslerin görül¬mesi bu lenfomalarm EORTC de belir¬tildiği gibi agresif seyirli lenfomalar ol¬duğunu düşündürdü. PKBL larm 4 ü folikül merkez hücreli lenfoma (FMHL), 8 i marginal zon lenfoma (MZL), biri ise T hücrelerinden zengin B hücreli len¬foma idi. FMHL larm hepsi difüz ve bü¬yük hücreli idi. Bu hastaların sadece bi¬rinde sistemik lenfomaya dönüşüm sap¬tandı. Sekiz MZL olgusunun kısa süreli takiplerinde nüks veya sistemik tutulu¬ma rastlanmadı. Çalışmamızda T ve B hücreli psödolenfomalarda RZR ile Ig ağır zincir genlerinde klonal yeniden dü¬zenlenme oranı % 50 olarak tespit edil¬di. Bu olguların 3-15 aylık takiplerinde ise lenfomaya dönüşüm saptanmadı. Bugün için özellikle B hücreli lenfo-malarda aksıyan yönleri olmakla birlik¬te EORTC sınıflamasının primer kuta-nöz lenfomalar için en uygun sınıflama olduğu inancındayız.

Primary cutaneous lymphoma: A retrospective and prospective evaluation based on EORTC classification

Background.- Until recently, cutaneous lymphomas were evaluated according to the classification shemas used for nodal non-Hodgkin lymphomas without considering whether they are primary or cutaneous involvement of systemic lymphomas. In time, it is realized that primary cutaneous lymphomas displayed distinct biological behavior. As a result, EORTC (the European Organization for Research and Treatment of Cancer) classification, considering each lymphoma as an entity with their clinical, histological, immunohistochemical and genotypic features altogether, was offered. Design.- In our project, 25 non-mycosis fungoides (MF)/Sezary syndrome (SS) primary cutaneous T cell lymphomas (PCTL), 13 primary cutaneous B cell lymphomas (PCBL), 35 MF and 8 pseudolymphomas whose diagnosis was established in our department, were taken into our study group and they were evaluated in a multidisciplinary approach. Results.- Among the PCL diagnosed in our department 82.2 % showed T cell phenotype while 17.8 % were B cell phenotype. MF constituted 58.3 % of all PCTL and 47.9 % of all PCL. Besides, non-MF PCTL were 34.2 % of all PCLs. Histologically, especially the early lesions of MF caused difficulties in the differential diagnosis of benign lichenoid and spongiotic dermatitis. Out of 9 cases of lymphomatoid papulosis (LP) in our study group, 6 were LP type A while 3 were type C. Using PCR, 20 % of LP type A and 50 % of type C displayed clonal rearrangement in TCR genes. Out of 7 CD 30(+) PCTL, 3 cases arised in a background of MF, and showed an aggressive course. Only in one case, translocation(2:5) was detected. Out of 6 CD 30(-) PCTL, one developed from a preexisting pagetoid reticulosis. Progression to a systemic lymphoma in another case, as well as frequent recurrences in the other cases, pointed out the aggressive course in this type of lymphomas, as suggested by EORTC. Among the PCBL, 4 were follicle center cell lymphoma (FCCL), 8 were marginal zone lymphoma (MZL) and one was T cell rich B cell lymphoma. All FCCLs had a diffuse pattern and composed of mainly large cells. Only one case out of 4, developed systemic lymphoma. Neither of the 8 cases of MZL, had recurrence or progression to systemic lymphoma in a rather short period of follow-up. Both T and B cell pseudolymphomas in our study group, showed 50 % of clonal rearrangement in Ig heavy chain genes using PCR. During the follow-up period of 3-15 months, none of them turned out to be a frank lymphoma. Conclusion.- Although there were some controversies in PBCL category, EORTC classification is yet found to be the best classification for primary cutaneous lymphomas

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