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���� ������������ 2010���2019 ����. ������������������ �������������������������������������� ������������������������ ������������������ �������� �� 561 ���������������� �� ���������������������� ���� ���������������������������������������� ���������������������� ��������, �������������������������� �������������� ������������������ ������������ �������� �� 105 ��������������, ���� ������ ���������������������� ���������� �������������� �� 86 ��������������. �������������������������� ���������������������������� ���������� ���������������������������� ���������������������� �������������������������������������� �� ������������������������������������������ ������������������������ ������������������ �������� �������������� ���� ���������������� ������������������������������ ����������������, �� ������ ���������� �� ���������������������� ���� ������������������ �������������� ��������, ������������������������ ������������ ���������������������� ����������������������., In 2010���2019, pathomorphological investigations of the skin biopsies were performed in 561 patients with suspected lymphoproliferative skin manifestations. The diagnosis of primary skin lymphomas was verified in 105 patients, of which Mycosis Fungoides was detected in 86 patients. Involvement of additional options for objectifying the results of pathomorphological and immunohistochemical studies of skin biopsies of patients with ��complex diagnostic search��, including those with suspected primary skin lymphomas, contributes to early diagnostics of the diseases., �������������� ��������, ������������ 5 2020

Die Gruppe der primär kutanen Lymphome bietet in der klinischen Präsentation als auch im Erkrankungsverlauf eine außerordentliche Vielfalt. Das Wissen über Wirksamkeit und Nebenwirkungen einer Therapie ist Voraussetzung, um dem Patienten für das jeweilige Krankheitsbild eine geeignete Therapie mit ausgewogenem Nutzen-Risiko-Verhältnis empfehlen zu können. Durch die Auswertungen der Behandlungsergebnisse von subkutanem Interferon-α und intravenös appliziertem Rituximab konnten wir die Wirksamkeit dieser Therapieformen für indolente primär kutane B-Zell-Lymphome belegen bzw. bestätigen. Beide Therapieansätze hatten eine hohe Rezidivrate und sind somit nicht kurativ wirksam. Sowohl Interferon-α als auch Rituximab wären für eine Erhaltungstherapie geeignet, welche aufgrund der insgesamt guten Prognose der indolenten primär kutanen B-Zell-Lymphome im Regelfall jedoch nicht indiziert erscheint. Auch das Radioimmuntherapeutikum 90Yttrium-Ibritumomab-Tiuxetan erzielte bei primär kutanen B-Zell-Lymphomen eine hohe Ansprechrate, ohne jedoch Remissionsdauer oder Rezidivrate im Vergleich zum weniger knochenmarkstoxischen Rituximab zu verbessern. Bei den primär kutanen T-Zell- Lymphomen besteht insbesondere für fortgeschrittene Stadien der Mycosis fungoides ein Bedarf an therapeutischen Weiterentwicklungen. Da selbst wirksame Einzelsubstanzen meist nur zeitlich begrenzte Remissionen erzielen, haben wir die Wirksamkeit von Kombinationstherapien untersucht. Die Kombination von Bexaroten und Vorinostat zeigte dabei in vitro, nicht aber in der klinischen Studie eine gegenseitige Wirkungsverstärkung. Auch die systematische Analyse der Literatur konnte keine einer Monotherapie eindeutig überlegene Kombinationstherapie identifizieren. Dennoch legen die Daten nahe, dass eine Remission bei einem Teil der zuvor refraktären Patienten durch die Kombinationen bestimmter Therapieverfahren erreicht werden kann. Zur Nachsorge von Patienten mit indolenten primär kutanen Lymphomen haben wir Vorschläge für die Methodik und Intervalle der Nachsorgeuntersuchungen erarbeitet. Hierbei stellt die klinische Untersuchung mit sorgfältiger Hautinspektion die wesentliche Maßnahme zur Feststellung von Krankheitsrückfällen dar. Bildgebende Untersuchungen sind nur für Patienten indiziert, die zuvor eine extrakutane Manifestation aufwiesen oder ein hohes Risiko für eine solche extrakutane Progression aufweisen., The primary cutaneous lymphomas as a group offer an extraordinary variety with regard to clinical presentation as well as course of disease. Detailled knowledge on efficacy and adverse events of a treatment is a prerequisite to be able to recommend an appropriate therapy with a balanced risk-benefit-ratio to a patient suffering from a cutaneous lymphoma. We were able to demonstrate the efficacy of subcutaneously applied interferon-α and confirm succesful use of intravenously applied rituximab for the treatment of indolent primary cutaneous B-cell-lymphomas by analysing results of patients treated with these substances in our department. However, both treatments had a high rate of recurrences and are therefore not curative. Both interferon-α and rituximab could be used for maintenace therapy, which is - due to the good prognosis of indolent subtypes of primary cutaneous B-cell-lymphomas – in general not indicated. Treatment with the radioimmunotherapeutic 90Yttrium-ibritumomab- tiuxetan also obtained a high rate of remissions in primary cutaneous B-cell- lymphomas but resulted neither in an improved duration of response nor a reduction in the recurrence rate in comparison to the less toxic monoclonal antibody rituximab. Within the group of primary cutaneous T-cell-lymphomas there is a high need of therapeutic innovations for advanced stages of mycosis fungoides. As approved drugs used for monotherapy achieve mostly short-lived remissions we analysed the efficacy of combination therapies. The combination of bexarotene and vorinostat showed in vitro, but not in a clinical trial an additive effect. A systematic literature review did also not identify a combination of drugs which was superior to the respective monotherapies. However, data of few trials indicated that some drug combinations but not other are able to achieve remissions in patients refractory to monotherapy. Finally, we formulated recommendations for the follow-up of patients with indolent primary cutaneous lymphomas with regard to examinations and time intervals. The main focus should be on the thorough clinical examination including an inspection of the entire skin as this is by far the most important investigation to identify disease recurrences. Imaging studies are indicated only to monitor patients with prior extracutanepus manifestations or who have risk factors for progression of disease to extracutaneous sites.

Primary cutaneous lymphomas are currently classified by the European Organization for Research and Treatment of Cancer (EORTC) classification or the World Health Organization (WHO) classification, but both systems have shortcomings. In particular, differences in the classification of cutaneous T-cell lymphomas other than mycosis fungoides, Sezary syndrome, and the group of primary cutaneous CD30+ lymphoproliferative disorders and the classification and terminology of different types of cutaneous B-cell lymphomas have resulted in considerable debate and confusion. During recent consensus meetings representatives of both systems reached agreement on a new classification, which is now called the WHO-EORTC classification. In this paper we describe the characteristic features of the different primary cutaneous lymphomas and other hematologic neoplasms frequently presenting in the skin, and discuss differences with the previous classification schemes. In addition, the relative frequency and survival data of 1905 patients with primary cutaneous lymphomas derived from Dutch and Austrian registries for primary cutaneous lymphomas are presented to illustrate the clinical significance of this new classification.

Background: Nonmycosis fungoides/Sezary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. Aims: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts. Materials and Methods: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications. Results: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein–Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety. Conclusions: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.