Department of Dermatology and Venereology, I.M. Sechenov First Moscow State Medical University, 4 Bolshaya Pirogovskaya Street, Building 1, 119991, Moscow, Russian Federation.
Moscow Municipal Clinical Hospital Named After S. P. Botkin, 125284, Moscow, Russian Federation.
Int J Hematol. 2020 Oct;112(4):459-465. doi: 10.1007/s12185-020-02978-8. Epub 2020 Sep 5.
Chronic lymphocytic leukemia (CLL) is a malignant lymphoproliferative disease characterized by the accumulation of immature monoclonal B lymphocytes in blood cells, bone marrow, spleen and lymph nodes. This is the most common type of leukemia among the Caucasoid race. When CLL skin lesions occur in about 25% of patients, they are extremely diverse. These lesions can be divided into specific, including infiltration of the skin by leukemic cells and the skin form of Richter's syndrome, secondary skin tumors, nonspecific lesions and associated skin diseases.Leukemic infiltration of the skin in patients with leukemia is called specific skin lesions (SSL). Many authors associate the unfavorable prognosis with the transformation of CLL with specific infiltration of the skin into Richter syndrome, as well as the appearance of SSL before the diagnosis of CLL. The risk of developing various cancer pathologies in patients with CLL is three times higher than in healthy people identical in sex and age. It was found that the risk of skin cancer in these patients is eight times higher than in the healthy population. The most common secondary skin tumors in CLL are basal-cell carcinoma, squamous-cell carcinoma, melanoma, and Merkel tumor.Nonspecific skin changes are extremely diverse and occur in patients with CLL in 30-50% of cases. The most common secondary changes in the skin in CLL are those of infectious nature. There are also increased reactions to insect bites, generalized itching, exfoliative erythroderma, nodular erythema, paraneoplastic pemphigoid, bullous pemphigoid, drug eruption. Concomitant dermatoses in these patients are more severe and often torpid to the previously conducted therapy. There is no doubt that together with the clarification of the etiology and pathogenesis of CLL, particular issues related to the study of clinical and morphological changes in individual organs and systems, in particular the skin, formed at various stages of the development of this disease should be studied in detail. This can not only expand and clarify our understanding of this pathology, but also can help to clarify the essence of the disease.
慢性淋巴细胞白血病(CLL)是一种恶性淋巴增生性疾病,其特征是血液、骨髓、脾脏和淋巴结中不成熟的单克隆 B 淋巴细胞积累。这是白种人中最常见的白血病类型。当 CLL 皮肤病变发生在约 25%的患者中时,它们是极其多样化的。这些病变可分为特异性病变,包括白血病细胞浸润皮肤和 Richter 综合征的皮肤形式、继发性皮肤肿瘤、非特异性病变和相关皮肤病。白血病浸润皮肤的白血病患者称为特异性皮肤病变(SSL)。许多作者将不良预后与 CLL 向 Richter 综合征伴特异性浸润的转化以及 CLL 诊断前出现 SSL 联系起来。CLL 患者发生各种癌症病理的风险比性别和年龄相同的健康人高 3 倍。研究发现,这些患者患皮肤癌的风险比健康人群高 8 倍。CLL 中最常见的继发性皮肤肿瘤是基底细胞癌、鳞状细胞癌、黑色素瘤和 Merkel 细胞瘤。非特异性皮肤变化极其多样化,在 30-50%的 CLL 患者中发生。CLL 中最常见的皮肤继发性变化是具有感染性质的变化。还有昆虫叮咬、全身性瘙痒、剥脱性红皮病、结节性红斑、副肿瘤性天疱疮、大疱性类天疱疮、药物疹的反应增加。这些患者的伴随皮肤病更严重,并且经常对之前进行的治疗迟钝。毫无疑问,随着对 CLL 的病因和发病机制的阐明,应详细研究与研究该病发展的各个阶段中个别器官和系统,特别是皮肤的临床和形态变化相关的特定问题。这不仅可以扩展和阐明我们对这种病理学的理解,而且还可以帮助阐明疾病的本质。