Spałkowska Magdalena, Brzewski Pawel, Walter Scott, Jaworek Andrzej K, Sułowicz Joanna, Andres Michal, Wojas-Pelc Anna
Przegl Lek. 2016;73(7):452-9.
Primary cutaneous lymphomas are lymphoproliferative skin infiltrates of T-, B- or NK-cells, classified according to the World Health Organization - European Organization of the Research and Treatment of Cancer (WHO-EORTC) criteria. They are the second most common group of extranodal non-Hodgkin lymphomas, that present in the skin with no evidence of systemic involvement at the time of diagnosis.
The aim of the study was the analysis of clinical profile of cutaneous lymphomas in the tertiary referral center in Poland.
We analyzed case records of 63 patients (26 women, 37 men aged 19 - 86) referred to the Department of Dermatology, University Hospital in Cracow for the diagnosis and treatment of cutaneous lymphoma.
After analysis of clinical and histological data, the final diagnoses were: mycosis fungoides (42 patients), primary cutaneous CD30+ lymphoproliferative disorder (7), Sezary syndrome (3), parapsoriasis (3), primary cutaneous B-cell lymphoma (1), acute myeloid leukemia (1), Hodgkin lymphoma coexistent with mycosis fungoides (1), generalized allergic contact dermatitis (2) and erythema elevatum diutinum (1). We excluded 2 patients due to incomplete data. The most common location of skin lesions was the lower limb (52.46%) and most common clinical presentation was raised erythematous lesion (26.23%). Pruritus was present in 45.9% of the patients and 39.3% had extracutaneous symptoms, with lymphadenopathy as the most common symptom. 37.7% of patients presented with mild eosinophilia and another 37.7% with mild monocytosis. Prior to referral to our center, general practitioners misdiagnosed the lymphomas commonly as: atopic and contact dermatitis, borreliosis, drug-induced exanthema.
The diagnosis of cutaneous lymphoma is often delayed due to their indolent, often recurring course, non-specific symptoms and uncommon appearance. The cooperation of a clinician and pathologist is essential in the diagnostic process.
原发性皮肤淋巴瘤是T细胞、B细胞或NK细胞的淋巴增殖性皮肤浸润,根据世界卫生组织-欧洲癌症研究与治疗组织(WHO-EORTC)标准进行分类。它们是第二常见的结外非霍奇金淋巴瘤,在诊断时表现为皮肤受累且无全身受累证据。
本研究的目的是分析波兰三级转诊中心皮肤淋巴瘤的临床特征。
我们分析了63例患者(26例女性,37例男性,年龄19 - 86岁)的病例记录,这些患者因皮肤淋巴瘤的诊断和治疗转诊至克拉科夫大学医院皮肤科。
经临床和组织学数据分析,最终诊断为:蕈样肉芽肿(42例患者)、原发性皮肤CD30 +淋巴增殖性疾病(7例)、塞扎里综合征(3例)、副银屑病(3例)、原发性皮肤B细胞淋巴瘤(1例)、急性髓系白血病(1例)、与蕈样肉芽肿共存的霍奇金淋巴瘤(1例)、泛发性过敏性接触性皮炎(2例)和持久性隆起性红斑(1例)。因数据不完整排除2例患者。皮肤病变最常见的部位是下肢(52.46%),最常见的临床表现是隆起性红斑病变(26.23%)。45.9%的患者有瘙痒,39.3%有皮肤外症状,最常见的症状是淋巴结病。37.7%的患者有轻度嗜酸性粒细胞增多,另有37.7%有轻度单核细胞增多。在转诊至我们中心之前,全科医生常将淋巴瘤误诊为:特应性皮炎和接触性皮炎、莱姆病、药物性皮疹。
皮肤淋巴瘤的诊断常常延迟,原因是其病程缓慢、常复发、症状不特异且外观不常见。临床医生和病理学家的合作在诊断过程中至关重要。