关键词:
Primary cutaneous B-cell lymphomas
a retrospective study
diagnostics
treatment outcomes
comorbidities
摘要:
Background: Primary cutaneous B-cell lymphomas (PCBCLs) are a group of commonly indolent diseases that comprises primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT). PCBCLs affect mainly adults but also children. Treatment is watchful waiting, excision, radiotherapy, rituximab, and systemic chemotherapy in case of extracutaneous spread disease. The disease is rare and there is a huge need of studies on clinical features and treatment outcomes. Aim: The aim of the study was to present demographics, clinical features and treatment outcomes of patients with PCBCLs in West Sweden. Methods: This is a descriptive, register-based, single center retrospective study of patients diagnosed with PCBCLs at Department of Dermatology and Venerology at Sahlgrenska University Hospital between 2002-2021. Data regarding demographics, clinical characteristics, pathological- and medical examination findings, comorbidities, staging according to TNM-system and treatment response were collected from patients’ medical records and the hospital-based skin lymphoma register. Results: Nine patients with PCBCLs were identified and included in the study. Mean age at diagnosis was 53.8±14.5 years for patients with PCFCL (n=5) and 49.2±28.1 for PCMZL (n=4), respectively. Skin presentations of PCFCL were in form of papules, plaques and tumors (40%) preferably distributed on the head (60%) and trunk (40%). Papules (75%) on upper extremity (50%) were the most common manifestation in PCMZL. In most cases the histopathological diagnosis was set after the first pathological anatomical diagnosis but for PCFCL it required two or three biopsies before the diagnosis was confirmed. Monoclonality was identified in 20–25% in PCFCL and PCMZL. Lactate dehydrogenase was increased at any time in PCFCL 40% and PCMZL 25%. All the patients with PCFCL were tested for borreliosis and all were nega