查看“边缘区淋巴瘤”的源代码

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            <strong>[[边缘区淋巴瘤]]([[Marginal Zone Lymphoma]]/[[MZL]])</strong>是一组起源于淋巴滤泡边缘区的慢性成熟<strong>[[B细胞]]</strong>外周淋巴瘤，约占所有非霍奇金淋巴瘤（[[NHL]]）的7%至8%。[[MZL]]在生物学上表现为高度异质性，通常分为三个主要亚型：<strong>[[粘膜相关淋巴组织淋巴瘤]]([[MALT淋巴瘤]])</strong>、<strong>[[脾边缘区淋巴瘤]]([[SMZL]])</strong>及<strong>[[节点边缘区淋巴瘤]]([[NMZL]])</strong>。其发病常与慢性抗原刺激（如[[幽门螺杆菌]]感染或[[自身免疫性疾病]]）密切相关。临床病程多呈惰性，但具有复发倾向，现代诊疗强调基于亚型的分层管理及精准靶向干预。
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            <div style="font-size: 1.2em; font-weight: bold; letter-spacing: 1.2px;">边缘区淋巴瘤 (MZL)</div>
            <div style="font-size: 0.7em; opacity: 0.85; margin-top: 4px; white-space: nowrap;">Marginal Zone Lymphoma·点击展开</div>
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                <div style="display: inline-block; background: #ffffff; border: 1px solid #e2e8f0; border-radius: 12px; padding: 15px; box-shadow: 0 4px 10px rgba(0,0,0,0.04);">
                     <div style="width: 120px; height: 80px; background-color: #f1f5f9; display: flex; align-items: center; justify-content: center; color: #94a3b8; font-size: 0.75em; padding: 10px; text-align: center;">H&E Staining: Small lymphoid cells in the marginal zone</div>
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                <div style="font-size: 0.8em; color: #64748b; margin-top: 10px; font-weight: 600;">核心表型：CD20+ / CD5- / CD10-</div>
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                    <th style="text-align: left; padding: 6px 10px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0; width: 40%;">[[Entrez]]ID</th>
                    <td style="padding: 6px 10px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">10892([[MALT1]])</td>
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                    <td style="padding: 6px 10px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">Q9UDY8</td>
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                    <th style="text-align: left; padding: 6px 10px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">关键染色体</th>
                    <td style="padding: 6px 10px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">t(11;18)/t(1;14)</td>
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                    <th style="text-align: left; padding: 6px 10px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">ICD-11</th>
                    <td style="padding: 6px 10px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">2A83</td>
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                    <th style="text-align: left; padding: 6px 10px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">核心靶点</th>
                    <td style="padding: 6px 10px; border-bottom: 1px solid #e2e8f0; color: #1e40af;">[[CD20]]/[[BTK]]</td>
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                    <th style="text-align: left; padding: 6px 10px; background-color: #f1f5f9; color: #475569;">主要驱动</th>
                    <td style="padding: 10px; color: #0f172a;">[[NF-kappaB]]通路激活</td>
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    <h2 style="background: #f1f5f9; color: #0f172a; padding: 10px 18px; border-radius: 0 6px 6px 0; font-size: 1.25em; margin-top: 40px; border-left: 6px solid #0f172a; font-weight: bold;">分子机制：慢性抗原驱动与通路重塑</h2>
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        [[MZL]]的发病机制体现了从“慢性炎症”向“恶性肿瘤”演变的典型模式：
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        <li style="margin-bottom: 12px;"><strong>慢性抗原刺激：</strong> 在胃 [[MALT淋巴瘤]] 中，[[幽门螺杆菌]]的持续刺激导致 B 细胞过度增殖。长期刺激下，细胞发生遗传学改变（如 [[t(11;18)]] 易位），产生 <strong>[[API2-MALT1]]</strong> 融合蛋白。</li>
        <li style="margin-bottom: 12px;"><strong>NF-kappaB 异常激活：</strong> 易位导致的 [[MALT1]] 或 [[BCL10]] 异常表达，会持续激活下游的 <strong>[[NF-kappaB]]</strong> 信号通路，赋予肿瘤细胞极强的抗凋亡能力。</li>
        <li style="margin-bottom: 12px;"><strong>Notch 通路突变：</strong> 在 [[SMZL]] 亚型中，约 20% 至 30% 的患者存在 <strong>[[NOTCH2]]</strong> 突变，这增强了边缘区 B 细胞的定向发育和生存信号。</li>
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    <h2 style="background: #f1f5f9; color: #0f172a; padding: 10px 18px; border-radius: 0 6px 6px 0; font-size: 1.25em; margin-top: 40px; border-left: 6px solid #0f172a; font-weight: bold;">亚型特征与临床对照矩阵</h2>
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                    <th style="padding: 10px; border: 1px solid #e2e8f0; text-align: left;">亚型</th>
                    <th style="padding: 10px; border: 1px solid #e2e8f0; text-align: left;">最常见部位</th>
                    <th style="padding: 10px; border: 1px solid #e2e8f0; text-align: left;">关联因素</th>
                    <th style="padding: 10px; border: 1px solid #e2e8f0; text-align: left;">分子标记</th>
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                    <td style="padding: 10px; border: 1px solid #e2e8f0; font-weight: 600;">[[MALT淋巴瘤]]</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">胃、肺、眼眶、涎腺</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">幽门螺杆菌/干燥综合征</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">t(11;18)(q21;q21)</td>
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                    <td style="padding: 10px; border: 1px solid #e2e8f0; font-weight: 600;">[[SMZL]]</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">脾脏、骨髓、外周血</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">丙型肝炎(部分)</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">7q31-32缺失/NOTCH2</td>
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                    <td style="padding: 10px; border: 1px solid #e2e8f0; font-weight: 600;">[[NMZL]]</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">淋巴结</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">原因不明</td>
                    <td style="padding: 10px; border: 1px solid #e2e8f0;">无特征性重排</td>
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    <h2 style="background: #f1f5f9; color: #0f172a; padding: 10px 18px; border-radius: 0 6px 6px 0; font-size: 1.25em; margin-top: 40px; border-left: 6px solid #0f172a; font-weight: bold;">诊疗策略：从感染清除到精准靶向</h2>
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        [[MZL]]的管理强调“**[[权衡利弊、分期干预]]**”：
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        <li style="margin-bottom: 12px;"><strong>病原体清除：</strong> 对于早期胃 [[MALT淋巴瘤]]，一线治疗首选抗[[幽门螺杆菌]]治疗。约 70% 至 80% 的患者在感染清除后可获得长期临床缓解。</li>
        <li style="margin-bottom: 12px;"><strong>免疫靶向治疗：</strong> 针对复发或全身性 [[MZL]]，<strong>[[利妥昔单抗]]</strong>（单药或联合化疗）是标准方案。对于二线及以上患者，新一代 <strong>[[BTK抑制剂]]</strong>（如[[泽布替尼]]、[[伊布替尼]]）已展现出卓越的客观缓解率。</li>
        <li style="margin-bottom: 12px;"><strong>PI3K路径拦截：</strong> 对于多线经治的复发患者，针对 [[PI3K-delta]] 或 [[PI3K-alpha]] 亚型的抑制剂（如[[库潘尼西]]）提供了重要的补救选择。</li>
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    <h2 style="background: #f1f5f9; color: #0f172a; padding: 10px 18px; border-radius: 0 6px 6px 0; font-size: 1.25em; margin-top: 40px; border-left: 6px solid #0f172a; font-weight: bold;">关键相关概念</h2>
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            <li style="margin-bottom: 8px;"><strong>[[API2-MALT1]]：</strong> 预测抗幽门螺杆菌治疗无效的关键遗传学标志物。</li>
            <li style="margin-bottom: 8px;"><strong>[[脾切除术]]：</strong> 曾是 [[SMZL]] 的主要诊断与治疗手段，目前逐渐被利妥昔单抗取代。</li>
            <li style="margin-bottom: 8px;"><strong>[[惰性非霍奇金淋巴瘤]]：</strong> [[MZL]] 所属的大类，强调其缓慢进展的临床特性。</li>
            <li style="margin-bottom: 8px;"><strong>[[大细胞转化]]：</strong> [[MZL]] 演变为侵袭性淋巴瘤（如 [[DLBCL]]）的过程，需严密监测。</li>
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        <span style="color: #0f172a; font-weight: bold; font-size: 1.05em; display: inline-block; margin-bottom: 10px;">学术参考文献与权威评审</span>
        <p style="margin: 8px 0; border-bottom: 1px solid #e2e8f0; padding-bottom: 8px;">
            [1] <strong>Zucca E, et al. (2020).</strong> <em>ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up of marginal zone lymphomas.</em> <strong>[[Annals of Oncology]]</strong>.<br>
            <span style="color: #475569;">[点评]：该指南详尽规范了三大亚型在不同分期下的标准化诊疗路径。</span>
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            [2] <strong>Bertoni F, et al. (2022).</strong> <em>Molecular pathogenesis of marginal zone B-cell lymphoma.</em> <strong>[[Hematological Oncology]]</strong>.[Academic Review]<br>
            <span style="color: #475569;">[点评]：系统解析了 NF-kappaB 和 Notch 通路在 MZL 发生发展中的驱动作用。</span>
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            边缘区淋巴瘤 (MZL) 诊疗生态 · 知识图谱
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                <td style="width: 80px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px; text-align: right;">关联靶点</td>
                <td style="padding: 10px 15px;">[[CD20]]•[[BTK]]•[[MALT1]]•[[PI3K-delta]]•[[Notch2]]•[[BCL10]]</td>
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                <td style="width: 80px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px; text-align: right;">核心药物</td>
                <td style="padding: 10px 15px;">[[利妥昔单抗]]•[[泽布替尼]]•[[伊布替尼]]•[[奥妥珠单抗]]•[[来那度胺]]</td>
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                <td style="width: 80px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px; text-align: right;">前沿方向</td>
                <td style="padding: 10px 15px;">[[双特异性抗体]]•[[CAR-NK序贯方案]]•[[MALT1小分子抑制剂]]•[[无化疗联合策略]]</td>
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