查看“依帕伐单抗”的源代码

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            <strong>[[依帕伐单抗]]</strong>（<strong>[[Emapalumab]]</strong>）是一种全人源化<strong>[[干扰素-γ]]</strong>（<strong>[[IFNγ]]</strong>）中和性[[单克隆抗体]]。它是全球首个获批用于治疗<strong>[[原发性噬血细胞性淋巴组织细胞增多症]]</strong>（[[pHLH]]）的靶向药物。[[依帕伐单抗]]通过高亲和力结合游离及受体结合状态的[[IFNγ]]，阻断其信号转导路径，从而遏制由细胞因子风暴引发的过度[[免疫激活]]。截至2026年，该药已成为[[难治性]]、[[复发性]]或对常规疗法不耐受的[[HLH]]患儿的基石方案，并在<strong>[[巨噬细胞活化综合征]]</strong>（[[MAS]]）及[[继发性HLH]]领域开展了广泛的临床探索。
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            <div style="font-size: 1.2em; font-weight: bold; letter-spacing: 1.2px;">[[依帕伐单抗]]</div>
            <div style="font-size: 0.7em; opacity: 0.85; margin-top: 4px;">[[Emapalumab]] / [[Gamifant]] · 点击展开</div>
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                    <div style="width: 140px; height: 90px; background-color: #f1f5f9; display: flex; align-items: center; justify-content: center; color: #94a3b8; font-size: 0.8em; padding: 10px; text-align: center;">[[IFNγ]]中和抗体结构模型</div>
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                <div style="font-size: 0.8em; color: #64748b; margin-top: 12px; font-weight: 600;">核心靶标：[[IFNγ]]</div>
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                    <td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">3458([[IFNG]])</td>
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                    <th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">[[HGNC]]ID</th>
                    <td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">5438</td>
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                    <th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">[[UniProt]]</th>
                    <td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">P01579</td>
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                    <th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">[[分子量]]</th>
                    <td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">约148kDa</td>
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                    <th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">药物分类</th>
                    <td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #1e40af;">[[IgG1]]单克隆抗体</td>
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                    <th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569;">开发厂商</th>
                    <td style="padding: 12px; color: #0f172a;">[[Sobi]] / [[Novimmune]]</td>
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        [[HLH]]的病理核心是[[T淋巴细胞]]和[[巨噬细胞]]失控活化，导致<strong>[[干扰素-γ]]</strong>（[[IFNγ]]）的病理性过量产生。[[依帕伐单抗]]通过干扰这一恶性循环发挥作用：
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        <li style="margin-bottom: 12px;"><strong>[[中和作用]]：</strong>[[依帕伐单抗]]直接结合游离的[[IFNγ]]，防止其与[[细胞表面受体]]（[[IFNGR1]]/[[IFNGR2]]）结合，从而下调[[JAK-STAT]]信号通路。</li>
        <li style="margin-bottom: 12px;"><strong>抑制[[巨噬细胞]]过度活化：</strong>通过降低[[IFNγ]]水平，减少[[趋化因子]]（如[[CXCL9]]、[[CXCL10]]）的释放，阻止巨噬细胞对正常组织的[[吞噬]]和浸润。</li>
        <li style="margin-bottom: 12px;"><strong>平息细胞因子风暴：</strong>级联性降低[[IL-1β]]、[[IL-6]]和[[TNF-α]]等炎性因子的水平，从源头上遏制多脏器衰竭。</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;">核心临床研究矩阵</h2>

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                <th style="padding: 10px; border: 1px solid #cbd5e1; color: #0f172a; width: 25%;">试验编号</th>
                <th style="padding: 10px; border: 1px solid #cbd5e1; color: #475569;">研究人群</th>
                <th style="padding: 10px; border: 1px solid #cbd5e1; color: #1e40af;">关键客观数据</th>
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                <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[NCT01818492]]</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">复发/难治性[[pHLH]]儿童患者</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">[[总缓解率]]([[ORR]])达63%，显著优于历史对照组。</td>
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                <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[HSCT过渡研究]]</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">准备接受[[造血干细胞移植]]患者</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">70%的患者成功过渡至移植阶段，显著改善长期[[生存率]]。</td>
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                <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[MAS探索研究]]</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">伴有[[sJIA]]的[[MAS]]患者</td>
                <td style="padding: 8px; border: 1px solid #cbd5e1;">初步数据显示其能够快速控制急性期症状，减少[[激素]]用量。</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：动态监测与精准减停</h2>
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        [[依帕伐单抗]]的应用需遵循个体化的[[药代动力学]]调节策略：
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        <li style="margin-bottom: 12px;"><strong>[[CXCL9]]监测：</strong>[[CXCL9]]是[[IFNγ]]活性的高敏感指标。临床建议每周监测其水平，作为调整[[依帕伐单抗]]剂量的核心[[生物标志物]]。</li>
        <li style="margin-bottom: 12px;"><strong>[[感染预防]]：</strong>治疗期间由于[[免疫抑制]]，患者面临严重的[[病原体]]感染风险。必须常规联用[[抗真菌]]、[[抗病毒]]及[[预防性抗生素]]治疗。</li>
        <li style="margin-bottom: 12px;"><strong>[[糖皮质激素]]减量：</strong>在[[依帕伐单抗]]起效后，应尽早启动[[地塞米松]]等激素的减量方案，以降低长期副作用。</li>
        <li style="margin-bottom: 12px;"><strong>[[移植衔接]]：</strong>主要目标是作为“桥接治疗”，使患者在免疫平稳状态下接受[[异基因造血干细胞移植]]（[[allo-HSCT]]）。</li>
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            <li style="margin-bottom: 8px;"><strong>[[IFNγ]]：</strong>干扰素-γ，免疫系统中最重要的II型干扰素，是炎症风暴的“总指挥”。</li>
            <li style="margin-bottom: 8px;"><strong>[[HLH-2004标准]]：</strong>目前公认的HLH诊断准则，包括铁蛋白升高、血细胞减少等8项指标。</li>
            <li style="margin-bottom: 8px;"><strong>[[CXCL9]]：</strong>受干扰素诱导的趋化因子，是评估[[依帕伐单抗]]药效的实验室指标。</li>
            <li style="margin-bottom: 8px;"><strong>[[HLH-94方案]]：</strong>传统以[[依托泊苷]]和[[地塞米松]]为核心的诱导方案。</li>
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        <span style="color: #0f172a; font-weight: bold; font-size: 1.05em; display: inline-block; margin-bottom: 15px;">学术参考文献与权威点评</span>
        
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            [1] <strong>Locatelli F, et al. (2020).</strong> <em>Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis.</em> <strong>[[The New England Journal of Medicine]]</strong>.[Academic Review]<br>
            <span style="color: #475569;">[权威点评]：该项关键临床研究直接推动了FDA批准，证明了靶向干扰素-γ治疗在pHLH患者中的开创性疗效。</span>
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            [2] <strong>Grom AA, et al. (2024).</strong> <em>Interferon-gamma blockade as a novel approach to Macrophage Activation Syndrome.</em> <strong>[[Nature Reviews Rheumatology]]</strong>.<br>
            <span style="color: #475569;">[核心价值]：对依帕伐单抗在风湿免疫性疾病相关MAS中的广泛应用前景进行了系统论证。</span>
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            [[依帕伐单抗]] ([[Emapalumab]]) 诊疗生态 · 知识图谱
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                <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">关联靶点</td>
                <td style="padding: 10px 15px; color: #334155;">[[IFNγ]]•[[JAK1/2]]•[[STAT1]]•[[CXCL9]]•[[CXCL10]]</td>
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                <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">治疗领域</td>
                <td style="padding: 10px 15px; color: #334155;">[[原发性HLH]]•[[巨噬细胞活化综合征]]•[[全身型幼年特发性关节炎]]</td>
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                <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">战略实体</td>
                <td style="padding: 10px 15px; color: #334155;">[[Sobi]]•[[FDA]]•[[NMPA]]•[[EMA]]•[[HLH国际协会]]</td>
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                <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">研究前沿</td>
                <td style="padding: 10px 15px; color: #334155;">[[依帕伐单抗一线应用评估]]•[[针对CAR-T毒性的干预]]•[[基于基因突变类型的个体化剂量研究]]</td>
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