D Nashan1, C M Friedrich2, E Geissler3, A Schmitt-Graeff4,5, F Klein6, F Meiss7,5. 1. Hautklinik, Klinikum Dortmund gGmbH, Beurhausstr. 40, 44137, Dortmund, Deutschland. dorothee.nashan@klinikumdo.de. 2. Fachhochschule Dortmund - Fachbereich Informatik und Institut für Medizinische Informatik, Biometrie und Epidemiologie (IMIBE), Universitätsklinikum Essen, Essen, Deutschland. 3. Hautklinik, Klinikum Ludwigshafen, Ludwighafen, Deutschland. 4. Institut für Klinische Pathologie, Universitätsklinikum Freiburg, Freiburg, Deutschland. 5. Medizinische Fakultät, Albert-Ludwigs-Universität, Freiburg, Deutschland. 6. Wissenschaftliche Fachkommunikation, München, Deutschland. 7. Klinik für Dermatologie und Venerologie, Universitätsklinikum Freiburg, Freiburg, Deutschland.
Abstract
BACKGROUND: In addition to a broad and clinically diverse spectrum of known primary cutaneous lymphomas, for which an incidence of 1-3:100,000 is postulated, each year further entities are specified and defined. The goal is the presentation of a case series from daily clinical routine. METHODS: Over a period of 6 years and 2 months, patients consulting the Department of Dermatology, Medical Center University of Freiburg, were registered. Subsequently, collectives of mycosis fungoides (MF), Sezary syndrome (SS), CD30+ lymphoproliferative diseases, single cases with rare primary cutaneous lymphomas, and subcollectives of B‑cell lymphomas were examined. The high number of MF cases allowed the additional quantitative analyses of the types of therapies used in this group. RESULTS: Yearly 16-25 new diagnoses of primary cutaneous lymphoma are made. The evaluation of 163 primary cutaneous lymphoma revealed 111 cases with MF (68.1%), including 9 particular variants, 15 primary cutaneous CD30+ lymphoproliferative diseases (9.2%) dominated by 10 lymphomatoid papulosis (LyP), in addition to 5 primary cutaneous anaplastic large cell lymphoma (PCALCL), 6 SS (3.68%), and 24 cutaneous B‑cell lymphomas (14-72%). Three cases with rare primary cutaneous T/NK cell lymphomas are addressed in detail. In all, 82% of MF cases were stage IA and IB. The descending use of therapies for stage I-III included steroids and diverse UV therapies followed by bexarotene, interferon-α, methotrexate, and extracorporal photophoresis. CONCLUSIONS: Diagnoses of cutaneous lymphomas belong to a vast spectrum of differential diagnoses. This registry describes frequent findings and shows rare variants. You can only diagnose what you know; accordingly, a collection of case reports, which we wish to encourage, can help in processing and specification of entities.
BACKGROUND: In addition to a broad and clinically diverse spectrum of known primary cutaneous lymphomas, for which an incidence of 1-3:100,000 is postulated, each year further entities are specified and defined. The goal is the presentation of a case series from daily clinical routine. METHODS: Over a period of 6 years and 2 months, patients consulting the Department of Dermatology, Medical Center University of Freiburg, were registered. Subsequently, collectives of mycosis fungoides (MF), Sezary syndrome (SS), CD30+ lymphoproliferative diseases, single cases with rare primary cutaneous lymphomas, and subcollectives of B‑cell lymphomas were examined. The high number of MF cases allowed the additional quantitative analyses of the types of therapies used in this group. RESULTS: Yearly 16-25 new diagnoses of primary cutaneous lymphoma are made. The evaluation of 163 primary cutaneous lymphoma revealed 111 cases with MF (68.1%), including 9 particular variants, 15 primary cutaneous CD30+ lymphoproliferative diseases (9.2%) dominated by 10 lymphomatoid papulosis (LyP), in addition to 5 primary cutaneous anaplastic large cell lymphoma (PCALCL), 6 SS (3.68%), and 24 cutaneous B‑cell lymphomas (14-72%). Three cases with rare primary cutaneous T/NK cell lymphomas are addressed in detail. In all, 82% of MF cases were stage IA and IB. The descending use of therapies for stage I-III included steroids and diverse UV therapies followed by bexarotene, interferon-α, methotrexate, and extracorporal photophoresis. CONCLUSIONS: Diagnoses of cutaneous lymphomas belong to a vast spectrum of differential diagnoses. This registry describes frequent findings and shows rare variants. You can only diagnose what you know; accordingly, a collection of case reports, which we wish to encourage, can help in processing and specification of entities.
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Authors: Franz Trautinger; Johanna Eder; Chalid Assaf; Martine Bagot; Antonio Cozzio; Reinhard Dummer; Robert Gniadecki; Claus-Detlev Klemke; Pablo L Ortiz-Romero; Evangelia Papadavid; Nicola Pimpinelli; Pietro Quaglino; Annamari Ranki; Julia Scarisbrick; Rudolf Stadler; Liisa Väkevä; Maarten H Vermeer; Sean Whittaker; Rein Willemze; Robert Knobler Journal: Eur J Cancer Date: 2017-03-31 Impact factor: 9.162