Classical Case of Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Abstract

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) usually occurs in elderly patients and in most cases affects lower legs. A 76-year old man presented with red-brown coloured patches and plaques with irregular borders and multiple firm nodes on the anterior surface of the right lower leg. The excision biopsy of a node and histological examination has confirmed the diagnosis of PCDLBCL-LT. Clinical, laboratory and radiological examination showed no evidence of systemic involvement. The treatment was started with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone) regimen. The patient died within 10 months due to cardiac complications since the initial diagnosis.

Zusammenfassung

Das primäre kutane diffuse großzellige B-Zell-Lymphom, Unterschenkeltyp (PCDLBCL-LT) tritt bei älteren Patienten auf und fast immer an den Unterschenkeln. Ein 76-jähriger älterer Herr stellte sich mit rot-braunen Makeln und Plaques mit unregelmäßiger Begrenzung und mehreren indurierten Knoten am Schienbein des rechten Unterschenkels vor. Die Exzisionsbiopsie eines Knotens und die histologische Untersuchung bestätigten die Diagnose eines PCDLBCL-LT. Klinische, laborchemische und radiologische Untersuchungen zeigten keine Evidenz für eine systemische Beteiligung. Die Behandlung startete mit einem R-CHOP-Schema (Rituximab-Cyclophosphamid, Doxorubicin, Vincristin und Prednisolon). Der Patient verstarb aufgrund von kardialen Komplikationen 10 Monate nach Diagnosestellung.

• References

• 1 Senff NJ, Noordijk EM et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008; 112: 1600-1609
• 2 Thomas V, Dobson R et al. Primary cutaneous large B-cell lymphoma, leg type. Proc (Bayl Univ Med Cent ) 2011; 24: 350-353
• 3 Patsatsi A, Kyriakou A et al. Primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple local relapses: case presentation and brief review of literature. Hippokratia 2013; 17: 174-176
• 4 Kempf W, Denisjuk N et al. Primary cutaneous B-cell lymphomas. J Dtsch Dermatol Ges 2012; 10: 12-22
• 5 Bessell EM, Humber CE et al. Primary cutaneous B cell lymphoma in Nottinghamshire U.K.: prognosis of sub-types defined in the WHO-EORTC classification. Br J Dermatol 2012; 167: 1118-1123
• 6 Willemze R, Jaffe ES et al. WHOEORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768-3785
• 7 Mondal SK, Mandal PK et al. Primary cutaneous large B-cell lymphoma, leg type: Report of two cases and review of literature. Indian Journal of Medical and Paediatric Oncology: Official Journal of Indian Society of Medical & Paediatric Oncology 2012; 33: 54-57
• 8 Kodoma K, Massone C et al. Primary cutaneous large B-cell lymphomas: Clinicopathologic features, classification and prognostic factors in a large series of patients. Blood 2005; 106: 2491-2497
• 9 Slater DN. The new World Health Organization – European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants. British Journal of Dermatology 2005; 153: 874-880
• 10 Sokol L, Naghashpour M. Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management. Cancer Control 2012; 19: 236-244
• 11 Grange F, Beylot-Barry M et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol 2007; 143: 1144-1150
• 12 Antic D, Petrovic N et al. “Invisible” primary cutaneous diffuse large B-cell lymphoma, leg type, as a cause of fever of unknown origin. J Clin Oncol 2013; 31: e276-e279
• 13 Wobser M, Kneitz H et al. Primary cutaneous diffuse large B-cell lymphoma, leg-type, treated with a modified R-CHOP immunochemotherapy – diagnostic and therapeutic challenges. JDDG 2011; 9: 204-210
• 14 Hayakazu S, Sugaya M et al. Frequent Relapse and Irradiation Strategy in Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg-type. Acta Dermato-Venereologica 2013; 97-98
• 15 Stadler R, Assaf C et al. Brief S2k guidelines – Cutaneous lymphomas. JDDG 2013; 11: 19-28