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ABL-class - 版本历史
2026-04-18T23:21:14Z
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2026年1月23日 (五) 16:21 77921020
2026-01-23T16:21:53Z
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<td colspan="2" style="background-color: #fff; color: #202122; text-align: center;">←上一版本</td>
<td colspan="2" style="background-color: #fff; color: #202122; text-align: center;">2026年1月23日 (五) 16:21的版本</td>
</tr><tr><td colspan="2" class="diff-lineno" id="mw-diff-left-l74" >第74行：</td>
<td colspan="2" class="diff-lineno">第74行：</td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[ABL1]]</strong></td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[ABL1]]</strong></td></div></td></tr>
<tr><td class='diff-marker'>−</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[ETV6]]</strong>, <strong>[[NUP214]]</strong>, RANBP2, FOXP1, SNX2</td></div></td><td class='diff-marker'>+</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[ETV6]]</strong>, <strong>[[NUP214]]</strong>, <ins class="diffchange diffchange-inline">[[</ins>RANBP2<ins class="diffchange diffchange-inline">]]</ins>, <ins class="diffchange diffchange-inline">[[</ins>FOXP1<ins class="diffchange diffchange-inline">]]</ins>, <ins class="diffchange diffchange-inline">[[</ins>SNX2<ins class="diffchange diffchange-inline">]]</ins></td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">与 [[BCR-ABL1]] 机制最接近。[[ETV6-ABL1]] 常隐匿，需 FISH/RNA-seq 检测。</td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">与 [[BCR-ABL1]] 机制最接近。[[ETV6-ABL1]] 常隐匿，需 FISH/RNA-seq 检测。</td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[ABL2]]</strong></td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[ABL2]]</strong></td></div></td></tr>
<tr><td class='diff-marker'>−</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[RCSD1]]</strong>, PAG1, ZC3HAV1</td></div></td><td class='diff-marker'>+</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[RCSD1]]</strong>, <ins class="diffchange diffchange-inline">[[</ins>PAG1<ins class="diffchange diffchange-inline">]]</ins>, <ins class="diffchange diffchange-inline">[[</ins>ZC3HAV1<ins class="diffchange diffchange-inline">]]</ins></td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">较少见。ABL2 (ARG) 与 ABL1 高度同源，对 TKI 同样敏感。</td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">较少见。ABL2 (ARG) 与 ABL1 高度同源，对 TKI 同样敏感。</td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[PDGFRB]]</strong></td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[PDGFRB]]</strong></td></div></td></tr>
<tr><td class='diff-marker'>−</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[EBF1]]</strong>, TNIP1, ZEB2</td></div></td><td class='diff-marker'>+</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[EBF1]]</strong>, <ins class="diffchange diffchange-inline">[[</ins>TNIP1<ins class="diffchange diffchange-inline">]]</ins>, <ins class="diffchange diffchange-inline">[[</ins>ZEB2<ins class="diffchange diffchange-inline">]]</ins></td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">[[EBF1-PDGFRB]] 是 ABL 类中<strong>最常见</strong>的融合之一（约占 Ph-like 的 3-5%）。对 [[伊马替尼]] 极其敏感。</td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">[[EBF1-PDGFRB]] 是 ABL 类中<strong>最常见</strong>的融合之一（约占 Ph-like 的 3-5%）。对 [[伊马替尼]] 极其敏感。</td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[CSF1R]]</strong></td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;"><strong>[[CSF1R]]</strong></td></div></td></tr>
<tr><td class='diff-marker'>−</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">SSBP2, MEF2D</td></div></td><td class='diff-marker'>+</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;"><ins class="diffchange diffchange-inline">[[</ins>SSBP2<ins class="diffchange diffchange-inline">]]</ins>, <ins class="diffchange diffchange-inline">[[</ins>MEF2D<ins class="diffchange diffchange-inline">]]</ins></td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">罕见。CSF1R 是 PDGFR 家族成员，也受 ABL TKI 抑制。</td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 8px; border: 1px solid #cbd5e1;">罕见。CSF1R 是 PDGFR 家族成员，也受 ABL TKI 抑制。</td></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> </tr></div></td></tr>
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<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr style="border-bottom: 1px solid #f1f5f9;"></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <tr style="border-bottom: 1px solid #f1f5f9;"></div></td></tr>
<tr><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">所属疾病</td></div></td><td class='diff-marker'> </td><td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div> <td style="width: 85px; background-color: #f8fafc; color: #334155; font-weight: 600; padding: 10px 12px; text-align: right; vertical-align: middle; white-space: nowrap;">所属疾病</td></div></td></tr>
<tr><td class='diff-marker'>−</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 10px 15px; color: #334155;"><strong>[[Ph-like ALL]]</strong> (B-ALL) • 偶见 <strong>[[T-ALL]]</strong> (如 NUP214-ABL1)</td></div></td><td class='diff-marker'>+</td><td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div> <td style="padding: 10px 15px; color: #334155;"><strong>[[Ph-like ALL]]</strong> (B-ALL) • 偶见 <strong>[[T-ALL]]</strong> (如 <ins class="diffchange diffchange-inline">[[</ins>NUP214-ABL1<ins class="diffchange diffchange-inline">]]</ins>)</td></div></td></tr>
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2026年1月23日 (五) 16:03 77921020
2026-01-23T16:03:31Z
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<p>建立内容为“<div style="padding: 0 4%; line-height: 1.8; color: #1e293b; font-family: 'Helvetica Neue', Helvetica, 'PingFang SC', Arial, sans-serif; background-color: #ffffff…”的新页面</p>
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<strong>ABL类融合</strong>（ABL-class fusions）是<strong>[[Ph样急性淋巴细胞白血病]]</strong>（Ph-like ALL / BCR-ABL1-like ALL）中最重要的一个分子亚群，约占 Ph-like ALL 病例的 15%-20%。这组疾病的共同特征是：虽然缺乏经典的 [[BCR-ABL1]] 融合基因，但携带涉及 <strong>[[ABL1]]</strong>、<strong>[[ABL2]]</strong>、<strong>[[CSF1R]]</strong> 或 <strong>[[PDGFRB]]</strong> 等激酶基因的变异，导致激酶信号通路的持续激活。其基因表达谱（Gene Expression Profile）与 Ph+ ALL 高度相似。临床上，ABL 类融合患者通常对常规化疗反应不佳，预后较差，但对<strong>[[酪氨酸激酶抑制剂]]</strong>（TKI，如[[伊马替尼]]、[[达沙替尼]]）高度敏感。及时识别该亚群并联合 TKI 治疗是改善患者生存的关键。<br />
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<div style="font-size: 1.2em; font-weight: bold; letter-spacing: 1.2px;">ABL-class Fusions</div><br />
<div style="font-size: 0.7em; opacity: 0.85; margin-top: 4px; white-space: nowrap;">Kinase-Activating Alterations (点击展开)</div><br />
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[[Image:Ph_like_ALL_kinase_fusions_pie_chart.png|100px|Ph-like ALL 中 ABL 类融合的占比]]<br />
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<div style="font-size: 0.8em; color: #64748b; margin-top: 12px; font-weight: 600;">Ph-like ALL 核心亚群</div><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0; width: 40%;">疾病归属</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">[[Ph-like ALL]] (B-ALL)</td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">核心激酶</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #1e40af;"><strong>ABL1, ABL2, PDGFRB, CSF1R</strong></td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">常见融合</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">[[ETV6-ABL1]], [[EBF1-PDGFRB]]</td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">发生率</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;">儿童 ALL: ~3%<br>成人 ALL: ~2-4%</td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">检测金标准</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #0f172a;"><strong>[[RNA-seq]]</strong> (转录组测序)</td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569; border-bottom: 1px solid #e2e8f0;">敏感药物</th><br />
<td style="padding: 6px 12px; border-bottom: 1px solid #e2e8f0; color: #b91c1c;">[[伊马替尼]], [[达沙替尼]]</td><br />
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<th style="text-align: left; padding: 6px 12px; background-color: #f1f5f9; color: #475569;">预后特征</th><br />
<td style="padding: 6px 12px; color: #0f172a;">化疗反应差，MRD持续阳性</td><br />
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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;">定义与基因景观：四大激酶家族</h2><br />
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ABL 类融合是指在 Ph-like ALL 中，由 ABL1 原癌基因或其功能类似的激酶基因（ABL2, PDGFRB, CSF1R）发生的 3' 端融合事件。这些融合蛋白均保留了完整的酪氨酸激酶结构域，并通过 5' 端融合伙伴（如 ETV6, EBF1）的寡聚化结构域实现<strong>组成性激活</strong>。<br />
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<th style="padding: 10px; border: 1px solid #cbd5e1; color: #0f172a; width: 15%;">激酶基因</th><br />
<th style="padding: 10px; border: 1px solid #cbd5e1; color: #475569; width: 35%;">常见融合伙伴 (5' Partners)</th><br />
<th style="padding: 10px; border: 1px solid #cbd5e1; color: #1e40af;">特征与频率</th><br />
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<td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[ABL1]]</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[ETV6]]</strong>, NUP214, RANBP2, FOXP1, SNX2</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;">与 BCR-ABL1 机制最接近。ETV6-ABL1 常隐匿，需 FISH/RNA-seq 检测。</td><br />
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<td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[ABL2]]</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>RCSD1</strong>, PAG1, ZC3HAV1</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;">较少见。ABL2 (ARG) 与 ABL1 高度同源，对 TKI 同样敏感。</td><br />
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<td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[PDGFRB]]</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;"><strong>[[EBF1]]</strong>, TNIP1, ZEB2</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;">EBF1-PDGFRB 是 ABL 类中<strong>最常见</strong>的融合之一（约占 Ph-like 的 3-5%）。对伊马替尼极其敏感。</td><br />
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<td style="padding: 8px; border: 1px solid #cbd5e1; font-weight: 600;">[[CSF1R]]</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;">SSBP2, MEF2D</td><br />
<td style="padding: 8px; border: 1px solid #cbd5e1;">罕见。CSF1R 是 PDGFR 家族成员，也受 ABL TKI 抑制。</td><br />
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[[Image:ABL_class_fusion_proteins_mechanism_and_partners.png|100px|ABL 类融合蛋白的结构域与激活机制]]<br />
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<h2 style="background: #fff1f2; color: #9f1239; padding: 10px 18px; border-radius: 0 6px 6px 0; font-size: 1.25em; margin-top: 40px; border-left: 6px solid #9f1239; font-weight: bold;">临床意义：诊断与精准治疗</h2><br />
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<h3 style="margin-top: 0; color: #be123c; font-size: 1.1em;">必须进行筛查的亚群</h3><br />
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NCCN 和 WHO 指南强烈建议对所有 BCR-ABL1 阴性的 B-ALL 患者（特别是化疗诱导失败或 MRD 持续阳性的患者）进行 Ph-like 相关异常的筛查。<br />
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<li style="margin-bottom: 12px;"><strong>诊断挑战：</strong><br />
<br>ABL 类融合涉及的基因多、断点多、伙伴多（Promiscuous），且许多是<strong>隐匿性重排</strong>（常规核型分析正常）。<br />
<br><em>策略：</em> <br />
<br>1. <strong>初筛：</strong> 流式细胞术检测 CRLF2（虽然 ABL 类通常 CRLF2 低表达，但可排除另一大类）。<br />
<br>2. <strong>确诊：</strong> <strong>[[RNA测序]] (RNA-seq)</strong> 是金标准，可一次性检出所有融合。若不可用，可使用多色 FISH 探针组（Break-apart probes for ABL1, ABL2, PDGFRB, CSF1R）。</li><br />
<li style="margin-bottom: 12px;"><strong>治疗革命：TKI + 化疗</strong><br />
<br>多项回顾性和前瞻性研究（如 COG AALL1131）表明，在化疗基础上加用 <strong>[[达沙替尼]]</strong> (Dasatinib) 或 <strong>[[伊马替尼]]</strong> (Imatinib)，可显著提高 ABL 类 ALL 患者的无病生存率 (DFS) 和总生存率 (OS)。<br />
<br><em>推荐：</em> 一旦确诊 ABL 类融合，应立即在治疗方案中加入 TKI，并持续至维持治疗结束或移植后。</li><br />
<li style="margin-bottom: 12px;"><strong>预后监测：</strong><br />
<br>微小残留病 (<strong>[[MRD]]</strong>) 是评估 TKI 疗效的关键。对于联合 TKI 后 MRD 仍不转阴的高危患者，应考虑异基因造血干细胞移植。</li><br />
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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><br />
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[1] <strong>Roberts KG, Li Y, Payne-Turner D, et al. (2014).</strong> <em>Targetable kinase-activating lesions in Ph-like acute lymphoblastic leukemia.</em> <strong>[[New England Journal of Medicine]]</strong>. 2014;371(11):1005-1015.<br><br />
<span style="color: #475569;">[奠基之作]：该研究首次系统定义了 Ph-like ALL 的基因组图谱，明确了 ABL-class 融合是其中对 TKI 高度敏感的一个独特亚群，为精准治疗奠定了基础。</span><br />
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[2] <strong>Reshmi SC, Harvey RC, Roberts KG, et al. (2017).</strong> <em>Targetable kinase gene fusions in high-risk B-ALL: a study from the Children's Oncology Group.</em> <strong>[[Blood]]</strong>. 2017;129(25):3352-3361.<br><br />
<span style="color: #475569;">[诊断策略]：通过大规模 COG 队列分析，确定了 ABL 类融合在 NCI 高危 B-ALL 中的发生率，并评估了不同检测平台（基因表达谱 vs 融合检测）的效能。</span><br />
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[3] <strong>Tasian SK, Loh ML, Hunger SP. (2017).</strong> <em>Philadelphia chromosome-like acute lymphoblastic leukemia.</em> <strong>[[Blood]]</strong>. 2017;130(19):2064-2072.<br><br />
<span style="color: #475569;">[综述]：全面总结了 Ph-like ALL 的生物学特征和治疗进展，特别是强调了 ABL 类患者从 TKI 治疗中获得的显著生存获益。</span><br />
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ABL 类融合 · 知识图谱<br />
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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;">相关基因</td><br />
<td style="padding: 10px 15px; color: #334155;">[[ABL1]] • [[ABL2]] • [[PDGFRB]] • [[CSF1R]]</td><br />
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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;">所属疾病</td><br />
<td style="padding: 10px 15px; color: #334155;">[[Ph-like ALL]] (B-ALL) • 偶见 T-ALL (如 NUP214-ABL1)</td><br />
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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;">特效药物</td><br />
<td style="padding: 10px 15px; color: #334155;">[[达沙替尼]] (优选) • [[伊马替尼]] • [[氟马替尼]]</td><br />
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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;">另一亚群</td><br />
<td style="padding: 10px 15px; color: #334155;">[[JAK-STAT]] 类融合 (CRLF2, JAK2) → 需用 JAK 抑制剂 (鲁索替尼)</td><br />
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