Primary cutaneous diffuse large B-cell lymphoma, leg type – case report and literature review

Abstract

The primary cutaneous diffuse large B-cell lymphoma leg type (PCDLBCL-LT) is a rare form of cutaneous lymphoma with aggressive and unpredictable evolution. The patients are often aged and with comorbid conditions so that immunochemotherapy can be poorly tolerated. Despite its complexity, a differential diagnosis is possible in the primary care setting and it is of great importance in guiding further investigation and referral. We present the case of a 70-year-old man with reddish nodular lesions appeared on the anterior region of the right tibia. Anatomopathological subtype identified a PCDLBCL-LT, T2aN1M0 stage. The first line of R-CHOP regimen (rituximab – cyclophosphamide, doxorubicin, vincristine, prednisone) associating intrathecal prophylaxis with methotrexate was prescribed with a good response. An early relapse after first line chemotherapy was noticed. Salvage R-DHAP regimen (rituximab-dexamethasone, high dose cytarabine, cisplatin) with modest response and progression of the disease under chemotherapy was instituted. During targeted therapy, the patient experienced an acute myocardial infarction, heart failure, and acute kidney injury. Thus, comorbid conditions have dramatically reduced systemic therapeutic options. The multidisciplinary approach with the implication of general physician, hematologist, cardiologist and other specialists was necessary for the management of this case.

Keywords

• primary cutaneous diffuse large b-cell lymphoma
• leg type
• diagnosis
• treatment
• relapse
• multidisciplinary approach