Laboratory Medicine ›› 2023, Vol. 38 ›› Issue (10): 971-976.DOI: 10.3969/j.issn.1673-8640.2023.10.012
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DING Jing1, LIU Weiling1, XIA Xinxin2, LIN Lihui1()
Received:
2022-03-20
Revised:
2023-02-15
Online:
2023-10-30
Published:
2023-12-18
CLC Number:
DING Jing, LIU Weiling, XIA Xinxin, LIN Lihui. Analysis of chronic mast cell leukemia secondary to hematopoietic stem cell transplantation in myelodysplastic syndrome[J]. Laboratory Medicine, 2023, 38(10): 971-976.
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项目 | 初诊 | 移植前 | 供者 | 移植后 | 形态 | 处理 | 预后 |
---|---|---|---|---|---|---|---|
本病例 | MDS-EB-1 | MDS-EB-1 | 全相合同胞供者 | +1.5年诊断为肥大细胞白血病 | 肥大细胞约占96.5%。细胞中等偏大,多数规则,8%为梭形;核中等,圆形,居中或稍偏位,染色质偏成熟;胞质偏多,布满紫黑色粗大颗粒。21%胞体偏大,核畸形呈哑铃样或双核,染色质偏细,可有少数细胞颗粒缺乏 | 甲磺酸伊马替尼联合小剂量醋酸泼尼松片 | 移植后7年内早期完全嵌合状态,后期处于混合嵌合状态,存在肥大细胞、不存在髓系原始细胞 |
病例1[ | 真性红细胞增多症进展为急性髓细胞白血病 | 真性红细胞增多症存在,急性髓细胞白血病缓解 | 全相合无关供者 | +6年嵌合率降低、JAK2V617F变异等位基因频率上升,诊断为肥大细胞白血病伴骨髓增殖性肿瘤 | 肥大细胞占有核细胞的36%。细胞稍大,具有单个圆形或椭圆形细胞核。颗粒良好,核/质比低。形态介于非典型Ⅰ型、未成熟和成熟肥大细胞之间。纺锤形的肥大细胞<5%。异染原始细胞占有核细胞的1% | 供体淋巴细胞输注 | 移植后6年内持续缓解,无肥大细胞、JAK2V617F阴性、嵌合率100%;移植后4个月因新发颅内出血死亡 |
病例2[ | 急性髓细胞白血病伴t(8;21)(q22;q22) | 系统性肥大细胞增多症-急性髓细胞白血病 | 半相合无关供者 | +29 d骨髓涂片中肥大细胞占有核细胞的 62.4%;+49 d肥大细胞69.2%。诊断为肥大细胞白血病 | +29 d骨髓中异染性母细胞约62.4%,高核/质比、染色质细、核仁不明显、胞浆颗粒减少。+49 d非典型肥大细胞(Ⅱ型)占骨髓有核细胞的69.2%,核双叶或多叶,染色质较致密,无核仁,胞质中有丰富的异染颗粒 |
项目 | 初诊 | 移植前 | 供者 | 移植后 | 形态 | 处理 | 预后 |
---|---|---|---|---|---|---|---|
本病例 | MDS-EB-1 | MDS-EB-1 | 全相合同胞供者 | +1.5年诊断为肥大细胞白血病 | 肥大细胞约占96.5%。细胞中等偏大,多数规则,8%为梭形;核中等,圆形,居中或稍偏位,染色质偏成熟;胞质偏多,布满紫黑色粗大颗粒。21%胞体偏大,核畸形呈哑铃样或双核,染色质偏细,可有少数细胞颗粒缺乏 | 甲磺酸伊马替尼联合小剂量醋酸泼尼松片 | 移植后7年内早期完全嵌合状态,后期处于混合嵌合状态,存在肥大细胞、不存在髓系原始细胞 |
病例1[ | 真性红细胞增多症进展为急性髓细胞白血病 | 真性红细胞增多症存在,急性髓细胞白血病缓解 | 全相合无关供者 | +6年嵌合率降低、JAK2V617F变异等位基因频率上升,诊断为肥大细胞白血病伴骨髓增殖性肿瘤 | 肥大细胞占有核细胞的36%。细胞稍大,具有单个圆形或椭圆形细胞核。颗粒良好,核/质比低。形态介于非典型Ⅰ型、未成熟和成熟肥大细胞之间。纺锤形的肥大细胞<5%。异染原始细胞占有核细胞的1% | 供体淋巴细胞输注 | 移植后6年内持续缓解,无肥大细胞、JAK2V617F阴性、嵌合率100%;移植后4个月因新发颅内出血死亡 |
病例2[ | 急性髓细胞白血病伴t(8;21)(q22;q22) | 系统性肥大细胞增多症-急性髓细胞白血病 | 半相合无关供者 | +29 d骨髓涂片中肥大细胞占有核细胞的 62.4%;+49 d肥大细胞69.2%。诊断为肥大细胞白血病 | +29 d骨髓中异染性母细胞约62.4%,高核/质比、染色质细、核仁不明显、胞浆颗粒减少。+49 d非典型肥大细胞(Ⅱ型)占骨髓有核细胞的69.2%,核双叶或多叶,染色质较致密,无核仁,胞质中有丰富的异染颗粒 |
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