PMID- 28815289 OWN - NLM STAT- MEDLINE DCOM- 20190102 LR - 20191210 IS - 1432-1173 (Electronic) IS - 0017-8470 (Linking) VI - 68 IP - 9 DP - 2017 Sep TI - [Aggressive primary cutaneous B-cell lymphomas and novel EBV+ entities]. PG - 727-739 LID - 10.1007/s00105-017-4018-0 [doi] AB - Primary cutaneous large B‑cell lymphomas (PCBLT), EBV-positive large B‑cell lymphomas, not otherwise specified (EBV+ DLBCL, NOS), and primary cutaneous intravascular large B‑cell lymphomas (PCIVLBL) are recognized as cutaneous lymphomas with intermediate to poor prognosis. Differentiation from indolent B‑cell lymphomas or other pathologies of the skin can be complex, both clinically and histologically, but vital for the outcome of the patient. The combination of immunotherapy and polychemotherapy regimens, such as R‑CHOP, has led to significant improvements in prognosis, especially in diffuse large B‑cell lymphomas. Therapeutic decisions need to be individually made for each patient, ideally within an interdisciplinary tumor conference. Immunosenescence may be an important factor in the pathogenesis of EBV+ DLBCL, NOS in elderly individuals. Their prognosis is less favorable than that of patients with EBV-negative PCBLT, whereby this has been observed particularly in elderly patients. One third of patients with PCIVLBL progress to systemic disease. The occurrence of nodal manifestation is rarely observed. Symptoms may vary depending on the organ system involved. Currently there are no evidence-based therapy recommendations due to the rarity of the disease. EBV-positive mucocutaneous ulcer is a new provisional category in the current WHO classification for lymphoid neoplasms. It has been segregated from EBV+ DLBCL, NOS due to its self-limiting course and good response to conservative therapy. FAU - Lamos, C AU - Lamos C AD - Hautklinik Ludwigshafen, Klinikum der Stadt Ludwigshafen am Rhein, Bremserstr. 79, 67063, Ludwigshafen, Deutschland. lamosc@klilu.de. FAU - Dippel, E AU - Dippel E AD - Hautklinik Ludwigshafen, Klinikum der Stadt Ludwigshafen am Rhein, Bremserstr. 79, 67063, Ludwigshafen, Deutschland. LA - ger PT - Journal Article PT - Review TT - Aggressive primar kutane B-Zell-Lymphome und neue EBV-positive Entitaten. PL - Germany TA - Hautarzt JT - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JID - 0372755 RN - 0 (Antibodies, Monoclonal, Murine-Derived) RN - 0 (R-CHOP protocol) RN - 4F4X42SYQ6 (Rituximab) RN - 5J49Q6B70F (Vincristine) RN - 80168379AG (Doxorubicin) RN - 8N3DW7272P (Cyclophosphamide) RN - VB0R961HZT (Prednisone) SB - IM MH - Adult MH - Aged MH - Antibodies, Monoclonal, Murine-Derived/therapeutic use MH - Antineoplastic Combined Chemotherapy Protocols/therapeutic use MH - Biopsy MH - Combined Modality Therapy MH - Cyclophosphamide/therapeutic use MH - Doxorubicin/therapeutic use MH - Epstein-Barr Virus Infections/classification/*diagnosis/pathology/therapy MH - Female MH - Guideline Adherence MH - Humans MH - Lymphoma, B-Cell/classification/*diagnosis/pathology/therapy MH - Lymphoma, Large B-Cell, Diffuse/classification/diagnosis/pathology/therapy MH - Male MH - Middle Aged MH - Neoplasm Staging MH - Prednisone/therapeutic use MH - Prognosis MH - Rituximab MH - Skin/pathology MH - Skin Neoplasms/classification/*diagnosis/pathology/therapy MH - Vincristine/therapeutic use OTO - NOTNLM OT - Epstein-Barr virus OT - Immunosenescence OT - Immunotherapy OT - Polychemotherapy OT - Prognosis EDAT- 2017/08/18 06:00 MHDA- 2019/01/03 06:00 CRDT- 2017/08/18 06:00 PHST- 2017/08/18 06:00 [pubmed] PHST- 2019/01/03 06:00 [medline] PHST- 2017/08/18 06:00 [entrez] AID - 10.1007/s00105-017-4018-0 [pii] AID - 10.1007/s00105-017-4018-0 [doi] PST - ppublish SO - Hautarzt. 2017 Sep;68(9):727-739. doi: 10.1007/s00105-017-4018-0.