罕见的非霍奇金淋巴瘤:原发性骨淋巴瘤的治疗经验

I. Lysenko, A. A. Barashev, T. Lapteva, N. Nikolaeva, E. Kapuza, O. Shatokhina, T. F. Pushkareva
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摘要

原发性骨淋巴瘤是一种罕见的非霍奇金淋巴瘤。它最多占成人所有非霍奇金淋巴瘤的1-2%。原发性骨淋巴瘤在一个或多个骨骼的局灶性病变中诊断;软组织和局部淋巴结也可能受累。排除标准仅为骨髓损伤和远处淋巴结受累。最初的症状包括顽固性骨痛,常伴有局部水肿,在患处形成肿块;B症状偶有加入。骨干和干骺端长管骨的局部病变更为常见(80%),而多灶性病变较少(20%)。原发性和继发性骨组织病变的诊断是基于使用所有可用的研究方法(放射照相;计算机,磁共振和正电子发射断层扫描)。鉴别诊断需要免疫组织化学研究,确定总白细胞抗原,b细胞和t细胞标志物的表达,免疫球蛋白轻链之一的克隆性κ或λ, bcl 2和bcl 6, ALK, Ki-67的增殖活性。对原发性骨淋巴瘤的各种治疗方法的有效性的评估由于少量的观察和缺乏统一的治疗策略而变得复杂。chop样化疗周期常被用作一线治疗。个体化治疗包括免疫化疗、放射治疗和手术治疗——内源性修复术。
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Rare forms of non-Hodgkin lymphomas: experience in treatment for primary bone lymphomas
Primary bone lymphoma is a rare presentation of non-Hodgkin lymphoma. It accounts for a maximum of 1–2% of all non-Hodgkin lymphomas in adults. Primary bone lymphoma is diagnosed in focal lesions of one or more bones; soft tissue and regional lymph nodes may be involved too. The exclusion criteria are only bone marrow damage and involvement of distant lymph nodes. The first symptoms include intractable bone pain often accompanied by local edema, the formation of a tumor mass in the affected area; B symptoms occasionally join. Local lesions of long tubular bones in the diaphysis and metadiaphysis regions are more common (80%), while multifocal lesions are less frequent (20%). Diagnosis of lesions of the bone tissue in its primary and secondary involvement is based on the use of all available research methods (radiography; computed, magnetic resonance and positron emission tomography). Differential diagnosis requires an immunohistochemical study with determination of the expression of total leukocyte antigen, B-cell and T-cell markers, and clonality in one of immunoglobulin light chains κ or λ, bcl 2 and bcl 6, ALK, proliferative activity of Ki-67. Evaluation of the effectiveness of various treatments for primary bone lymphoma is complicated by a small number of observations and the absence of a uniform treatment strategy. CHOP-like chemotherapy cycles are often used as first-line therapy. Personalized therapy involves immunochemotherapy, radiation therapy and surgical treatment — endoprosthetics.
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