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style=\"font-size: 15px;\">间变性大细胞淋巴瘤 (anaplastic large cell lymphoma，ALC）属于一种罕见的、可快速增长的、侵袭性T细胞非霍奇金淋巴瘤 (NHL) ，ALK阳性间变大细胞淋巴瘤（ALK-positive anaplastic large cell lymphoma）是ALCL最常见的一种亚型，由T 淋巴细胞（T细胞）发展而来。\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cbr\u002F>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">ALK阳性间变大细胞淋巴瘤通常影响儿童和年轻人，男性患者是女性的2~3倍；ALK阳性ALCL往往对化疗反应良好，患者通常会获得长期缓解。\u003C\u002Fspan>\u003C\u002Fp>","⚕️","2026-08-03",{"key":90,"title":91,"html":92,"icon":93,"updatedAt":88},"symptom","症状表现","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\"> ALK阳性间变大细胞淋巴瘤不仅会累及淋巴结，还会侵犯淋巴结外的器官，这使得它的临床表现多种多样，常见的症状包括：\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·一般症状，很多患者出现B症状，如盗汗、发烧和不明原因的体重减轻。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·颈部、腋窝或腹股沟的无痛淋巴结肿大。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·结外侵犯的症状与受累的器官有关，容易受影响的身体部位包括骨骼、皮肤、骨髓、肺和肝脏。其中，皮肤和软组织是最常出现结外侵犯的部位，主要表现为皮下结节、溃疡、丘疹样皮损等；骨骼受侵犯可表现为溶骨性损害；肺部病变可表现为结节；骨髓（制造血细胞的骨骼中心的海绵组织）受到影响后，可能会导致贫血和血小板减少症。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·50% 的全身性ALCL患者中，血液中的乳酸脱氢酶 (LDH）水平会升高。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>","📋",{"key":95,"title":96,"html":97,"icon":98,"updatedAt":88},"exam","检查方式","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">为确诊ALK阳性间变大细胞淋巴瘤，医生可能会选择的诊断性检查包括体格检查、组织活检、实验室检查、影像学检查等。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">1.体格检查\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">主要检查患者的一般状况，包括全身皮肤检查，触诊全身浅表淋巴结，是否出现韦氏环（咽淋巴环），以及触诊肝脏、脾脏。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">2.血液检查\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">血常规可观察不同类型血细胞的数量和形态，ALCL患者白细胞增高、血小板增高、CRP增高的情况都比较常见；部分患者的淋巴瘤细胞可侵犯骨髓和外周血，出现白血病的表现。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">3.病理活检\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">病理活检主要是取多个组织样本在镜下观察，看是否出现ALK阳性间变大细胞淋巴瘤特异性的病理学特征，以及需要对组织样本做免疫分型。ALCL患者淋巴瘤细胞的表面会特异性地表达一种叫CD30的抗原。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">4.骨髓活检\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">由于ALCL患者受到骨髓侵犯的比例不高，骨髓活检和免疫组化染色等检查可获得比骨髓穿刺更高的阳性率。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">5.流式细胞分析\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">流式细胞分析可根据细胞的大小、颗粒、抗原表达特征，将细胞分为不同群体，明确淋巴瘤的免疫分型，帮助医生选择最适合患者的治疗方案。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">6.影像学检查\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·CT检查：可用于观察身体各部位的横截面图像，也是患者需接受的常规检查。患者可能需要接受颈部、胸部、腹部、盆腔等部位的扫描，检查结果可作为确诊和分期的依据。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·PET\u002FCT检查：可对全身的肿瘤病灶进行显像，并能显示出CT无法充分显像的病变，PET\u002FCT也是疾病分期和评估疗效的重要工具。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·怀疑患者的骨骼受到侵犯，可以做骨扫描检查。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·如果患者出现了中枢神经系统受累的症状，还需要做头颅 MRI\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">或 CT 扫描。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">7.细胞遗传学和分子遗传学检查\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">细胞遗传学方法主要检测常见染色体数量或结构异常，分子遗传学方法，主要检测患者常见的基因突变；分子诊断不仅是诊断的重要标志，同时也是以后治疗的靶点。\u003C\u002Fspan>\u003C\u002Fdiv>","🩺",{"key":100,"title":101,"html":102,"icon":103,"updatedAt":88},"treatment","治疗方式","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\"> ALK阳性间变性大细胞淋巴瘤，主要的治疗方法包括化疗、放疗以及造血干细胞移植、分子靶向药治疗。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·化疗\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">化疗是一种通过口服或注射给药来杀灭肿瘤细胞的药物治疗，一般2~3周做一次治疗，通常要持续多个周期。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">对于ALK阳性间变性大细胞淋巴瘤，化疗是最主要的治疗方法，常用的化疗方案为CHEOP或CHOP 方案 （环磷酰胺、多柔比星、依托泊苷、长春新碱和泼尼松龙的联合化疗），ALK阳性的ALCL比其他类型的外周T细胞淋巴瘤预后都要好。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·放疗\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">放疗利用高能射线来破坏肿瘤细胞、治疗肿瘤，可单独使用，也可与其他癌症治疗方法联用。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·造血干细胞移植\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">对于预后不良的ALK阳性间变性大细胞淋巴瘤患者，可以早期做造血干细胞移植治疗。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">·分子靶向药\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">靶向治疗是针对肿瘤的特定基因、蛋白质或有助于癌症生长和存活的组织环境的治疗，靶向治疗精准度高，不仅能阻止肿瘤细胞的生长和扩散，还能避免对正常细胞的损害。适用于ALK阳性间变性大细胞淋巴瘤的药物有CD30抗体、ALK 抑制剂（克唑替尼）等。\u003C\u002Fspan>\u003C\u002Fdiv>","💊",{"author":105,"reviewedBy":105,"lastReviewed":105,"datePublished":105,"citations":106,"disclaimer":105},"",[107,109],{"title":108},"中国临床肿瘤学会（CSCO）淋巴瘤诊疗指南2020",{"title":110},"儿童ALK阳性间变大细胞淋巴瘤诊疗规范",false,1789384106554]