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【生物学特征】（一）克隆特征CML是一个起源于骨髓单株多能干细胞的获得性疾病，由于多能干细胞有向多个细胞系发育的潜能，CML急变时具不均一性，如急淋变、急粒变、急单变。
|
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（二）细胞生长特性CML从单个病变细胞增殖，直至骨髓、外周血、脾脏中堆积大量的CML细胞，多种机制参与了这一过程（表11-11）。
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"entity": "脾脏",
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表11-9CML细胞异常增生机制（三）细胞动力学CML慢性期外周血粒细胞数数十倍甚至数百倍于正常粒细胞数，这些细胞可自由循环于骨髓、外周血、脾脏间；CML细胞的半衰期比正常人粒细胞长5～10倍，这些细胞形态学上为未完全成熟的粒细胞，同时形态学上完全成熟的粒细胞半衰期也比正常人长2～4倍。
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"entity": "粒细胞数",
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"entity": "脾脏",
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"entity": "CML细胞",
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"entity": "粒细胞",
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"entity": "粒细胞",
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CML细胞的增殖速度比正常人慢，因此细胞寿命延长是CML外周血中粒细胞明显增高的主要原因，而不是它的增殖速度。
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"entity": "CML外周血中粒细胞",
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CML慢性期时髓系定向干细胞明显增多，因此在干细胞培养中粒-单细胞集落形成单位（GM-CFU）数明显增多，可高于正常人10～20倍。
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（四）疾病转化基础CML起病时多能造血干细胞发生c-abl基因重排，形成肿瘤前期血细胞克隆，这一转化过程中的克隆、出现细胞遗传学变化，即形成Ph1</sup>染色体使bcr/abl融合，融合基因下调酪氨酸激酶活力，使细胞凋亡紊乱。
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"entity": "bcr/abl融合",
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"entity": "酪氨酸激酶",
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这些细胞中基因的不稳定性增加和DNA复制过程中的自发性错误使病变细胞向更异常的方向发展，直至出现另一个新的细胞遗传学变化。
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随着这些变化，细胞的增殖与分化失去正常关系，异常克隆抑制正常克隆生长，异常的未成熟克隆呈优势，最终进入急性白血病期。
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【临床表现】CML分为三个阶段，即慢性期、加速期和急变期。
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各阶段代表疾病的性质由高增生性、基本正常的相对成熟血细胞成分为主进展为分化停滞、未成熟细胞为优势的过程。
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（一）慢性期主要由造血细胞池扩张造成的一系列变化，此期粒细胞数明显增高，主要浸润于骨髓、外周血、脾脏和肝脏，血黏稠度增高。
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"entity": "外周血",
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"entity": "脾脏",
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"id": 5,
"entity": "肝脏",
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病人有非特异性症状如发热、盗汗、乏力、左上腹饱满或疼痛、骨痛白细胞数，称为白血危象，造成血管阻塞并出现相应的临床情况如中枢神经系统症状、呼吸窘迫综合征、视力障碍等。
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"entity": "视力障碍",
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（二）加速期出现进展性加重的全身症状，如发热、盗汗、乏力、消瘦和出血倾向，肝、脾增大，化疗难于控制。
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{
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"entity": "肝、脾增大",
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{
"id": 2,
"entity": "化疗",
"start_offset": 43,
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"label": "pro"
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急变期临床表现与急性白血病相似，贫血、出血、发热，肝肿大并可伴有其他髓外浸润灶。
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【实验室检查】（一）外周血象1.慢性期轻度贫血，粒细胞数明显增高伴左移，计数在（8.0～80）×109</sup>/L之间，平均为25×109</sup>/L，大于50×109</sup>/L的病例较成人多见，涂片分类各期细胞均可见，但未完全成熟粒细胞小于15%，嗜酸及嗜碱粒细胞绝对值增高，并可见嗜酸嗜碱双染细胞。
|
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血小板数常增高，接近500×109</sup>/L。
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2.加速期粒细胞数下降，但原始及幼稚细胞比例明显增高。
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血小板数下降。
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此期病人50%发展为急性白血病，另约45%逐渐发展为骨髓增生异常综合征样状态。
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3.急变期血小板、血色素进一步下降，原始加幼稚细胞比例进一步增高与急性白血病相似。
|
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（二）骨髓象1.慢性期骨髓高度增生，以粒系为主，见各阶段细胞，以中晚幼粒细胞及杆状核粒细胞为主，原始加幼稚细胞比例小于5%。
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易见嗜碱和嗜酸细胞。
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骨髓纤维化不明显。
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偶见类似高雪细胞和海蓝细胞的有脂质沉积的组织细胞组织化学染色示白细胞碱性磷酸酶（AKP）活力明显减低原始加幼稚细胞比例大于30%是急变的主要依据，加速期原始加幼稚细胞比例在5%～30%之间。
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{
"id": 2,
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{
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"entity": "幼稚细胞比例在5%～30%之间",
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60%～70%病人向髓系急变，但此时过氧化物酶（POX）通常阴性，用单抗作表面抗原检测可发现幼稚细胞中也包含有少量巨核系、红系和单核系细胞。
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{
"id": 4,
"entity": "巨核系、红系和单核系细胞",
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"end_offset": 69,
"label": "bod"
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（三）细胞遗传学大部分病人Ph1</sup>染色体阳性，同时可检测到bcr/abl融合基因（P210）。
|
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急变期常有新的染色体变化，如Ph1</sup>复制，8-三体，19-三体，17q异构等。
|
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{
"id": 3,
"entity": "19-三体",
"start_offset": 31,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "17q异构",
"start_offset": 37,
"end_offset": 42,
"label": "sym"
}
] |
【预后】目前常用的治疗手段可使CML的中位生存期达5～5.5年，35%～40%的病人可生存7～8年。
|
[
{
"id": 0,
"entity": "CML",
"start_offset": 15,
"end_offset": 18,
"label": "dis"
}
] |
影响慢性期长短的不利因素包括脾左肋下大于15cm，肝右肋下大于6cm，血小板低于150×109</sup>/L或大于500×109</sup>/L，外周血幼稚细胞大于1%或未完全成熟（核左移）细胞大于20%。
|
[
{
"id": 0,
"entity": "脾",
"start_offset": 14,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "肝",
"start_offset": 25,
"end_offset": 26,
"label": "bod"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
},
{
"id": 3,
"entity": "外周血幼稚细胞",
"start_offset": 74,
"end_offset": 81,
"label": "bod"
},
{
"id": 4,
"entity": "未完全成熟（核左移）细胞",
"start_offset": 86,
"end_offset": 98,
"label": "bod"
}
] |
【治疗】常规化疗很难达到细胞遗传学水平缓解，即彻底清除Ph1</sup>染色体阳性细胞。
|
[
{
"id": 0,
"entity": "化疗",
"start_offset": 6,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "Ph1</sup>染色体阳性细胞",
"start_offset": 27,
"end_offset": 43,
"label": "bod"
}
] |
目前异基因骨髓移植仍是CML获得长期无病生存的唯一治疗手段。
|
[
{
"id": 0,
"entity": "基因骨髓移植",
"start_offset": 3,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "CML",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
}
] |
针对bcr/abl融合基因的靶向治疗在CML治疗中取得了举世瞩目的成绩，可获得细胞/分子遗传学水平缓解，但是否能达到根治，目前尚无结论。
|
[
{
"id": 0,
"entity": "bcr/abl融合基因",
"start_offset": 2,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "靶向治疗",
"start_offset": 14,
"end_offset": 18,
"label": "pro"
},
{
"id": 2,
"entity": "CML",
"start_offset": 19,
"end_offset": 22,
"label": "dis"
}
] |
（一）非特异性处理高尿酸血症，水、电解质紊乱，白血危象，高血小板血症需紧急对症处理（同急性白血病），有脑膜浸润时按脑膜白血病处理。
|
[
{
"id": 0,
"entity": "高尿酸血症",
"start_offset": 9,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "水、电解质紊乱",
"start_offset": 15,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "白血危象",
"start_offset": 23,
"end_offset": 27,
"label": "dis"
},
{
"id": 3,
"entity": "高血小板血症",
"start_offset": 28,
"end_offset": 34,
"label": "dis"
},
{
"id": 4,
"entity": "急性白血病",
"start_offset": 43,
"end_offset": 48,
"label": "dis"
},
{
"id": 5,
"entity": "脑膜浸润",
"start_offset": 51,
"end_offset": 55,
"label": "sym"
},
{
"id": 6,
"entity": "脑膜白血病",
"start_offset": 57,
"end_offset": 62,
"label": "dis"
}
] |
（二）慢性期治疗1.单药化疗标准药物为马利兰或羟基脲，可有效地控制临床症状、体征和血液学变化，但不会使急变期延迟。
|
[
{
"id": 0,
"entity": "化疗",
"start_offset": 12,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "马利兰",
"start_offset": 19,
"end_offset": 22,
"label": "dru"
},
{
"id": 2,
"entity": "羟基脲",
"start_offset": 23,
"end_offset": 26,
"label": "dru"
}
] |
羟基脲是一种核苷酸还原酶抑制剂，抑制核苷酸转化为脱氧核苷酸，从而影响DNA合成，推荐剂量为10～20mg/（kg•d），根据临床情况作调整，它与马利兰疗效相仿，它的作用时间较短，因此相对安全，全身性的毒副作用也相对较小。
|
[
{
"id": 0,
"entity": "羟基脲",
"start_offset": 0,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "核苷酸还原酶",
"start_offset": 6,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "核苷酸",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "脱氧核苷酸",
"start_offset": 24,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "马利兰",
"start_offset": 72,
"end_offset": 75,
"label": "dru"
}
] |
马利兰是一种烷化剂，为非细胞周期特异性药物，常用剂量为0.06～0.1mg/（kg•d），用药10～14天后血细胞开始明显下跌、肝、脾缩小晚于血象变化，约3个月肝脾完全恢复正常。
|
[
{
"id": 0,
"entity": "马利兰",
"start_offset": 0,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "血细胞",
"start_offset": 54,
"end_offset": 57,
"label": "bod"
},
{
"id": 2,
"entity": "肝",
"start_offset": 64,
"end_offset": 65,
"label": "bod"
},
{
"id": 3,
"entity": "脾",
"start_offset": 66,
"end_offset": 67,
"label": "bod"
},
{
"id": 4,
"entity": "肝",
"start_offset": 80,
"end_offset": 81,
"label": "bod"
},
{
"id": 5,
"entity": "脾",
"start_offset": 81,
"end_offset": 82,
"label": "bod"
}
] |
马利兰特点为作用反应出现较晚但持续时间长，因此在白细胞计数跌至（30～40）×109</sup>/L时药物剂量应减半，至20×109</sup>/L时应停药。
|
[
{
"id": 0,
"entity": "马利兰",
"start_offset": 0,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "白细胞",
"start_offset": 24,
"end_offset": 27,
"label": "ite"
}
] |
停药后2～3周内白细胞计数仍可继续下跌。
|
[
{
"id": 0,
"entity": "白细胞",
"start_offset": 8,
"end_offset": 11,
"label": "ite"
}
] |
除骨髓抑制外，马利兰尚有肺纤维化、色素沉着、消瘦和低血压等不良反应。
|
[
{
"id": 0,
"entity": "骨髓抑制",
"start_offset": 1,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "马利兰",
"start_offset": 7,
"end_offset": 10,
"label": "dru"
},
{
"id": 2,
"entity": "肺纤维化",
"start_offset": 12,
"end_offset": 16,
"label": "sym"
},
{
"id": 3,
"entity": "色素沉着",
"start_offset": 17,
"end_offset": 21,
"label": "sym"
},
{
"id": 4,
"entity": "消瘦",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
},
{
"id": 5,
"entity": "低血压",
"start_offset": 25,
"end_offset": 28,
"label": "sym"
}
] |
2.α-干扰素其作用机制未明确。
|
[
{
"id": 0,
"entity": "α-干扰素",
"start_offset": 2,
"end_offset": 7,
"label": "dru"
}
] |
70%病人对干扰素治疗有效，达到血液学水平缓解。
|
[
{
"id": 0,
"entity": "干扰素",
"start_offset": 6,
"end_offset": 9,
"label": "dru"
}
] |
α-干扰素和马利兰合用时骨髓抑制严重，缓解率不高于单用α-干扰素。
|
[
{
"id": 0,
"entity": "α-干扰素",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "马利兰",
"start_offset": 6,
"end_offset": 9,
"label": "dru"
},
{
"id": 2,
"entity": "骨髓",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "α-干扰素",
"start_offset": 27,
"end_offset": 32,
"label": "dru"
}
] |
与羟基脲合用时达缓解时间缩短，毒副反应可以耐受，但并不提高细胞遗传学水平的缓解率。
|
[
{
"id": 0,
"entity": "羟基脲",
"start_offset": 1,
"end_offset": 4,
"label": "dru"
}
] |
与小剂量阿糖胞苷合用可提高细胞遗传学水平的缓解率和慢性期后期缓解率。
|
[
{
"id": 0,
"entity": "阿糖胞苷",
"start_offset": 4,
"end_offset": 8,
"label": "dru"
}
] |
α-干扰素与羟基脲合用时推荐用法如下：先用羟基脲，在白细胞数降至（10～20）×109</sup>/L时减停，同时加用α-干扰素，α-干扰素从小剂量开始，3～7天后增加剂量，在2周内达全量。
|
[
{
"id": 0,
"entity": "α-干扰素",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "羟基脲",
"start_offset": 6,
"end_offset": 9,
"label": "dru"
},
{
"id": 2,
"entity": "羟基脲",
"start_offset": 21,
"end_offset": 24,
"label": "dru"
},
{
"id": 3,
"entity": "白细胞",
"start_offset": 26,
"end_offset": 29,
"label": "ite"
},
{
"id": 4,
"entity": "α-干扰素",
"start_offset": 59,
"end_offset": 64,
"label": "dru"
},
{
"id": 5,
"entity": "α-干扰素",
"start_offset": 65,
"end_offset": 70,
"label": "dru"
}
] |
α-干扰素最好在睡前应用，并同时加用退热剂，以避免发热反应。
|
[
{
"id": 0,
"entity": "α-干扰素",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "退热剂",
"start_offset": 18,
"end_offset": 21,
"label": "dru"
},
{
"id": 2,
"entity": "发热反应",
"start_offset": 25,
"end_offset": 29,
"label": "sym"
}
] |
其他的不良反应尚有疲劳、抑郁、失眠等。
|
[
{
"id": 0,
"entity": "疲劳",
"start_offset": 9,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "抑郁",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "失眠",
"start_offset": 15,
"end_offset": 17,
"label": "sym"
}
] |
当白细胞降至2×109</sup>/L或血小板低于50×109</sup>/L时减量。
|
[
{
"id": 0,
"entity": "白细胞",
"start_offset": 1,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 20,
"end_offset": 23,
"label": "ite"
}
] |
α-干扰素应长期应用直至加速期或急变期或细胞遗传学水平缓解3年以上。
|
[
{
"id": 0,
"entity": "α-干扰素",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
}
] |
3.异基因骨髓移植目前仍是唯一已证实的可治愈CML的治疗方法（见干细胞移植章节）。
|
[
{
"id": 0,
"entity": "异基因骨髓移植",
"start_offset": 2,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "CML",
"start_offset": 22,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "干细胞移植",
"start_offset": 32,
"end_offset": 37,
"label": "pro"
}
] |
CML慢性期非亲缘供体骨髓移植结果（表11-11）。
|
[
{
"id": 0,
"entity": "非亲缘供体骨髓移植",
"start_offset": 6,
"end_offset": 15,
"label": "pro"
}
] |
表11-10CML慢性期非亲缘供体骨髓移植结果4.靶向治疗甲磺酸伊马替尼（imatiribmesylate，Gleevec，Glivec）通过特异性抑制肿瘤细胞酪氨酸激酶活性而抑制恶性肿瘤克隆，因具有高度特异性，故与其他化疗药物相比其毒副作用相对低，对CML非常有效。
|
[
{
"id": 0,
"entity": "CML",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "骨髓移植",
"start_offset": 17,
"end_offset": 21,
"label": "pro"
},
{
"id": 2,
"entity": "靶向治疗",
"start_offset": 25,
"end_offset": 29,
"label": "pro"
},
{
"id": 3,
"entity": "甲磺酸伊马替尼",
"start_offset": 29,
"end_offset": 36,
"label": "dru"
},
{
"id": 4,
"entity": "imatiribmesylate",
"start_offset": 37,
"end_offset": 53,
"label": "dru"
},
{
"id": 5,
"entity": "Gleevec",
"start_offset": 54,
"end_offset": 61,
"label": "dru"
},
{
"id": 6,
"entity": "Glivec",
"start_offset": 62,
"end_offset": 68,
"label": "dru"
},
{
"id": 7,
"entity": "肿瘤细胞酪氨酸激酶",
"start_offset": 76,
"end_offset": 85,
"label": "bod"
},
{
"id": 8,
"entity": "肿瘤",
"start_offset": 92,
"end_offset": 94,
"label": "dis"
},
{
"id": 9,
"entity": "化疗",
"start_offset": 110,
"end_offset": 112,
"label": "pro"
},
{
"id": 10,
"entity": "CML",
"start_offset": 126,
"end_offset": 129,
"label": "dis"
}
] |
经过大型临床研究（IRIS），伊马替尼每天400mg与IFN-α+Ara-C随机对照比较，伊马替尼有明显优势，包括血液缓解，细胞遗传学缓解及分子生物学缓解，同时亦延长存活及非进展存活，其后多个临床研究亦有相类结果。
|
[
{
"id": 0,
"entity": "伊马替尼",
"start_offset": 15,
"end_offset": 19,
"label": "dru"
},
{
"id": 1,
"entity": "IFN-α",
"start_offset": 27,
"end_offset": 32,
"label": "dru"
},
{
"id": 2,
"entity": "Ara-C",
"start_offset": 33,
"end_offset": 38,
"label": "dru"
},
{
"id": 3,
"entity": "伊马替尼",
"start_offset": 45,
"end_offset": 49,
"label": "dru"
},
{
"id": 4,
"entity": "血液",
"start_offset": 57,
"end_offset": 59,
"label": "bod"
}
] |
伊马替尼现在已是很多临床指示的第一线治疗CML药物，但目前尚不明确治疗时间和是否能完全替代异基因造血干细胞移植治愈CML。
|
[
{
"id": 0,
"entity": "伊马替尼",
"start_offset": 0,
"end_offset": 4,
"label": "dru"
},
{
"id": 1,
"entity": "CML",
"start_offset": 20,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "CML",
"start_offset": 57,
"end_offset": 60,
"label": "dis"
}
] |
（三）急变后治疗CML急变后预后不良，对治疗常耐药，急变后平均生存期为3个月，急淋变生存期稍长，急变后可按急性白血病相应类型治疗。
|
[
{
"id": 0,
"entity": "CML",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "白血病",
"start_offset": 55,
"end_offset": 58,
"label": "dis"
}
] |
第七节肺通气异常性疾病一、肺气肿肺气肿（emphysema）是指终末支气管远端部分，包括呼吸性细支气管，肺泡管、肺泡囊及肺泡的膨胀及过度充气，导致肺组织弹力减退和容积增大。
|
[
{
"id": 0,
"entity": "肺通气异常性疾病",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "肺气肿",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "肺气肿",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "emphysema",
"start_offset": 20,
"end_offset": 29,
"label": "dis"
},
{
"id": 4,
"entity": "终末支气管",
"start_offset": 32,
"end_offset": 37,
"label": "bod"
},
{
"id": 5,
"entity": "呼吸性细支气管",
"start_offset": 44,
"end_offset": 51,
"label": "bod"
},
{
"id": 6,
"entity": "肺泡管",
"start_offset": 52,
"end_offset": 55,
"label": "bod"
},
{
"id": 7,
"entity": "肺泡囊",
"start_offset": 56,
"end_offset": 59,
"label": "bod"
},
{
"id": 8,
"entity": "肺泡",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
}
] |
肺气肿可分三类（1）代偿性肺气肿：由于部分肺组织损坏，容积缩小，健康肺膨胀，填补空隙而形成代偿性肺气肿，多见于肺不张、脓胸、气胸等。
|
[
{
"id": 0,
"entity": "肺气肿",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "代偿性肺气肿",
"start_offset": 10,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "肺组织",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "肺",
"start_offset": 34,
"end_offset": 35,
"label": "bod"
},
{
"id": 4,
"entity": "代偿性肺气肿",
"start_offset": 45,
"end_offset": 51,
"label": "dis"
},
{
"id": 5,
"entity": "肺不张",
"start_offset": 55,
"end_offset": 58,
"label": "dis"
},
{
"id": 6,
"entity": "脓胸",
"start_offset": 59,
"end_offset": 61,
"label": "dis"
},
{
"id": 7,
"entity": "气胸",
"start_offset": 62,
"end_offset": 64,
"label": "dis"
}
] |
（2）梗阻性肺气肿：由于气管异物、支气管内膜结核、肺炎、支气管炎、百日咳、支气管哮喘等，导致支气管壁痉挛、狭窄及管腔内黏稠分泌物堵塞，形成活瓣，吸气时支气管腔扩大，吸入空气多，呼气时支气管管腔缩小，呼出空气少。
|
[
{
"id": 0,
"entity": "梗阻性肺气肿",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "气管异物",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "支气管内膜结核",
"start_offset": 17,
"end_offset": 24,
"label": "dis"
},
{
"id": 3,
"entity": "肺炎",
"start_offset": 25,
"end_offset": 27,
"label": "dis"
},
{
"id": 4,
"entity": "支气管炎",
"start_offset": 28,
"end_offset": 32,
"label": "dis"
},
{
"id": 5,
"entity": "百日咳",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
},
{
"id": 6,
"entity": "支气管哮喘",
"start_offset": 37,
"end_offset": 42,
"label": "dis"
},
{
"id": 7,
"entity": "支气管壁",
"start_offset": 46,
"end_offset": 50,
"label": "bod"
},
{
"id": 8,
"entity": "支气管管腔",
"start_offset": 91,
"end_offset": 96,
"label": "bod"
}
] |
或由于心脏扩大、肺动脉扩张、淋巴结肿大、纵隔肿瘤等压迫导致外因性支气管阻塞。
|
[
{
"id": 0,
"entity": "心脏",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肺动脉",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "淋巴结",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 3,
"entity": "纵隔肿瘤",
"start_offset": 20,
"end_offset": 24,
"label": "dis"
},
{
"id": 4,
"entity": "支气管",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
}
] |
（3）间质性肺气肿：剧烈咳嗽等情况下肺泡破裂，空气进入肺间质组织内而形成，空气可沿血管或淋巴管逆行至纵隔，形成纵隔气肿，亦可产生颈胸部皮下气肿和气胸。
|
[
{
"id": 0,
"entity": "间质性肺气肿",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "肺泡",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "肺间质组织",
"start_offset": 27,
"end_offset": 32,
"label": "bod"
},
{
"id": 3,
"entity": "血管",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 4,
"entity": "淋巴管",
"start_offset": 44,
"end_offset": 47,
"label": "bod"
},
{
"id": 5,
"entity": "纵隔气肿",
"start_offset": 55,
"end_offset": 59,
"label": "dis"
},
{
"id": 6,
"entity": "颈胸部皮下气肿和气胸",
"start_offset": 64,
"end_offset": 74,
"label": "dis"
}
] |
先天性肺叶气肿在本章第一节中讨论。
|
[
{
"id": 0,
"entity": "先天性肺叶气肿",
"start_offset": 0,
"end_offset": 7,
"label": "dis"
}
] |
症状随病因及受累范围和肺膨胀程度不同而异。
|
[
{
"id": 0,
"entity": "肺",
"start_offset": 11,
"end_offset": 12,
"label": "bod"
}
] |
一叶以上肺气肿常有严重呼吸困难、发绀等症状。
|
[
{
"id": 0,
"entity": "肺气肿",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "严重呼吸困难",
"start_offset": 9,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "发绀",
"start_offset": 16,
"end_offset": 18,
"label": "sym"
}
] |
听诊肺呼吸音减弱、遥远或消失，叩诊肺部有轻度或明显的鼓音，若一侧发生重度肺气肿，则纵隔移向对侧。
|
[
{
"id": 0,
"entity": "肺呼吸音减弱、遥远或消失",
"start_offset": 2,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "叩诊肺部有轻度或明显的鼓音",
"start_offset": 15,
"end_offset": 28,
"label": "sym"
},
{
"id": 2,
"entity": "纵隔移向对侧",
"start_offset": 41,
"end_offset": 47,
"label": "sym"
}
] |
X线透视起重要的诊断作用，表现为病侧肋间距较大，患区肺透亮度增强，膈肌运动受限、位置较低，心影移向健侧。
|
[
{
"id": 0,
"entity": "X线透视",
"start_offset": 0,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "病侧肋间距较大",
"start_offset": 16,
"end_offset": 23,
"label": "sym"
},
{
"id": 2,
"entity": "患区肺透亮度增强",
"start_offset": 24,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "膈肌运动受限",
"start_offset": 33,
"end_offset": 39,
"label": "sym"
},
{
"id": 4,
"entity": "位置较低",
"start_offset": 40,
"end_offset": 44,
"label": "sym"
},
{
"id": 5,
"entity": "心影移向健侧",
"start_offset": 45,
"end_offset": 51,
"label": "sym"
}
] |
两侧肺气肿者，心影较为狭小。
|
[
{
"id": 0,
"entity": "心影较为狭小",
"start_offset": 7,
"end_offset": 13,
"label": "sym"
}
] |
治疗包括去除病因和对症治疗。
|
[
{
"id": 0,
"entity": "去除病因",
"start_offset": 4,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "对症治疗",
"start_offset": 9,
"end_offset": 13,
"label": "pro"
}
] |
采用支气管解痉药及化痰药雾化吸入。
|
[
{
"id": 0,
"entity": "支气管解痉药",
"start_offset": 2,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "化痰药雾化吸入",
"start_offset": 9,
"end_offset": 16,
"label": "pro"
}
] |
第五章骨髓增生异常综合征骨髓增生异常综合征（myelodysplasticsyndrome，MDS）是一组临床表现为难治性贫血、感染和出血，外周血象表现为血细胞减少，骨髓为活跃或明显活跃增生，三系有病态造血，或原始细胞和早期细胞增多的综合征。
|
[
{
"id": 0,
"entity": "骨髓增生异常综合征",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "骨髓增生异常综合征",
"start_offset": 12,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "myelodysplasticsyndrome",
"start_offset": 22,
"end_offset": 45,
"label": "dis"
},
{
"id": 3,
"entity": "MDS",
"start_offset": 46,
"end_offset": 49,
"label": "dis"
},
{
"id": 4,
"entity": "难治性贫血",
"start_offset": 58,
"end_offset": 63,
"label": "sym"
},
{
"id": 5,
"entity": "感染",
"start_offset": 64,
"end_offset": 66,
"label": "sym"
},
{
"id": 6,
"entity": "出血",
"start_offset": 67,
"end_offset": 69,
"label": "sym"
},
{
"id": 7,
"entity": "血细胞",
"start_offset": 77,
"end_offset": 80,
"label": "bod"
},
{
"id": 8,
"entity": "细胞",
"start_offset": 107,
"end_offset": 109,
"label": "bod"
},
{
"id": 9,
"entity": "早期细胞增多的综合征",
"start_offset": 110,
"end_offset": 120,
"label": "dis"
}
] |
1953年Block等首先称之为白血病前期（preleukemia），简称“白前”。
|
[
{
"id": 0,
"entity": "白血病",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
}
] |
但并非所有的“白前”均转化为白血病，“白前”的诊断仅合适于已转化为白血病的回顾性诊断，因此1976年巴黎会议建议将这一组疾病称之为骨髓增生异常综合征，并渐被广泛接受。
|
[
{
"id": 0,
"entity": "白血病",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "白血病",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
},
{
"id": 2,
"entity": "骨髓增生异常综合征",
"start_offset": 65,
"end_offset": 74,
"label": "dis"
}
] |
【分类】2003年Hasle等参照成人MDS的WHO诊断分型标准提出了一个儿童MDS的WHO分型标准（表10-10），并提出了儿童MDS的最低诊断标准，认为至少符合以下四项中的任何两项方可诊断为MDS：1.持续性不能解释的血细胞减少（中性粒细胞减少、血小板减少或贫血）；2.至少二系有发育异常的形态学特征；3.造血细胞存在获得性克隆性细胞遗传学异常；4.原始细胞增高（≥5%）。
|
[
{
"id": 0,
"entity": "血细胞",
"start_offset": 111,
"end_offset": 114,
"label": "bod"
},
{
"id": 1,
"entity": "中性粒细胞",
"start_offset": 117,
"end_offset": 122,
"label": "bod"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 125,
"end_offset": 128,
"label": "bod"
},
{
"id": 3,
"entity": "贫血",
"start_offset": 131,
"end_offset": 133,
"label": "dis"
},
{
"id": 4,
"entity": "造血细胞",
"start_offset": 155,
"end_offset": 159,
"label": "bod"
},
{
"id": 5,
"entity": "原始细胞",
"start_offset": 177,
"end_offset": 181,
"label": "bod"
}
] |
按FAB标准诊断的儿童难治性贫血（RA）患儿与成人RA患者相比具有以下几点主要区别：①外周血贫血（Hb＜100g/L）所占比例较低（46%），主要表现为中性粒细胞绝对值（ANC）减少（其中ANC＜0.5×109</sup>/L比例为27%）和/或血小板数减低（＜150×109</sup>/L比例为75%）；②骨髓增生减低比例较高（43%）；③粒细胞系统和巨核细胞系统发育异常的细胞形态学改变与疾病演进和预后无相关性。
|
[
{
"id": 0,
"entity": "外周血贫血",
"start_offset": 43,
"end_offset": 48,
"label": "dis"
},
{
"id": 1,
"entity": "Hb",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
},
{
"id": 2,
"entity": "中性粒细胞",
"start_offset": 76,
"end_offset": 81,
"label": "bod"
},
{
"id": 3,
"entity": "ANC",
"start_offset": 85,
"end_offset": 88,
"label": "bod"
},
{
"id": 4,
"entity": "ANC",
"start_offset": 94,
"end_offset": 97,
"label": "bod"
},
{
"id": 5,
"entity": "血小板",
"start_offset": 123,
"end_offset": 126,
"label": "bod"
},
{
"id": 6,
"entity": "骨髓",
"start_offset": 155,
"end_offset": 157,
"label": "bod"
},
{
"id": 7,
"entity": "粒细胞",
"start_offset": 172,
"end_offset": 175,
"label": "bod"
},
{
"id": 8,
"entity": "巨核细胞",
"start_offset": 178,
"end_offset": 182,
"label": "bod"
},
{
"id": 9,
"entity": "细胞",
"start_offset": 189,
"end_offset": 191,
"label": "bod"
}
] |
表10-14儿童骨髓增生异常和骨髓增殖性疾病的诊断分类因此，采用难治性血细胞减少（RC）的定义而非RA。
|
[
{
"id": 0,
"entity": "骨髓",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "难治性血细胞减少",
"start_offset": 32,
"end_offset": 40,
"label": "sym"
},
{
"id": 3,
"entity": "RC",
"start_offset": 41,
"end_offset": 43,
"label": "sym"
},
{
"id": 4,
"entity": "RA",
"start_offset": 49,
"end_offset": 51,
"label": "sym"
}
] |
RC的确诊，特别是无克隆性染色体核型异常患儿，有时显得较困难。
|
[
{
"id": 0,
"entity": "RC",
"start_offset": 0,
"end_offset": 2,
"label": "sym"
}
] |
首先需能除外感染、代谢性疾病、营养缺乏症、药物。
|
[
{
"id": 0,
"entity": "感染",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "营养缺乏症",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
}
] |
【临床表现】（一）MDS的临床表现多样，通常起病隐匿，症状轻重取决于贫血、白细胞和血小板减少的程度和速度。
|
[
{
"id": 0,
"entity": "贫血",
"start_offset": 34,
"end_offset": 36,
"label": "dis"
},
{
"id": 1,
"entity": "白细胞",
"start_offset": 37,
"end_offset": 40,
"label": "bod"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 41,
"end_offset": 44,
"label": "bod"
}
] |
出血常表现为皮肤黏膜瘀点和瘀斑，重者反复鼻衄、牙龈渗血、血尿、消化道出血，甚至颅内出血，有出血表现者约占MDS患者的60%～80%。
|
[
{
"id": 0,
"entity": "皮肤黏膜瘀点",
"start_offset": 6,
"end_offset": 12,
"label": "sym"
},
{
"id": 1,
"entity": "瘀斑",
"start_offset": 13,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "反复鼻衄",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 3,
"entity": "牙龈渗血",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 4,
"entity": "血尿",
"start_offset": 28,
"end_offset": 30,
"label": "sym"
},
{
"id": 5,
"entity": "消化道出血",
"start_offset": 31,
"end_offset": 36,
"label": "sym"
},
{
"id": 6,
"entity": "颅内出血",
"start_offset": 39,
"end_offset": 43,
"label": "sym"
}
] |
肝、脾大者较多见，但淋巴结增大者不多，约5%～20%。
|
[
{
"id": 0,
"entity": "肝",
"start_offset": 0,
"end_offset": 1,
"label": "bod"
},
{
"id": 1,
"entity": "脾",
"start_offset": 2,
"end_offset": 3,
"label": "bod"
},
{
"id": 2,
"entity": "淋巴结",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
}
] |
（二）儿童MDSFAB亚型的特异表现儿童MDS与成人不同，以外周血细胞减少的增生低下型MDS多见，幼稚细胞增多向白细胞转化的MDS相对少见。
|
[
{
"id": 0,
"entity": "血细胞",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
},
{
"id": 1,
"entity": "幼稚细胞",
"start_offset": 49,
"end_offset": 53,
"label": "bod"
},
{
"id": 2,
"entity": "白细胞",
"start_offset": 56,
"end_offset": 59,
"label": "bod"
}
] |
幼年型慢性粒单核细胞白血病（juvenilemyelomonocyticleukemia，JMML）是儿童特有的MDS亚类。
|
[
{
"id": 0,
"entity": "幼年型慢性粒单核细胞白血病",
"start_offset": 0,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "juvenilemyelomonocyticleukemia",
"start_offset": 14,
"end_offset": 44,
"label": "dis"
},
{
"id": 2,
"entity": "JMML",
"start_offset": 45,
"end_offset": 49,
"label": "dis"
}
] |
1.JMML也称JCMML在临床血液学、细胞生物学和分子学等方面与成人慢性髓系白血病（CML）明显不同。
|
[
{
"id": 0,
"entity": "JMML",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "JCMML",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "白血病",
"start_offset": 39,
"end_offset": 42,
"label": "dis"
},
{
"id": 3,
"entity": "CML",
"start_offset": 43,
"end_offset": 46,
"label": "dis"
}
] |
外周血中白细胞计数及单核细胞绝对数增多，贫血、血小板减少，血液中胎儿血红蛋白（HbF）持续性的明显增高细胞及单核细胞增多，巨核细胞减少，病态造血的特征常不明显，6%～24%的患儿表现有7号染色体单体（-7），体外培养CFU-GM呈自发性生长，对GM-CSF刺激敏感性增高，患儿对化疗反应不敏感，生存期短，但急性白血病转化率相对较低，多数患儿死于骨髓衰竭并发症。
|
[
{
"id": 0,
"entity": "外周血",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "白细胞",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "单核细胞",
"start_offset": 10,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "贫血",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
},
{
"id": 4,
"entity": "血小板减少",
"start_offset": 23,
"end_offset": 28,
"label": "sym"
},
{
"id": 5,
"entity": "血液",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 6,
"entity": "血红蛋白",
"start_offset": 34,
"end_offset": 38,
"label": "bod"
},
{
"id": 7,
"entity": "HbF",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
},
{
"id": 8,
"entity": "血液中胎儿血红蛋白（HbF）持续性的明显增高",
"start_offset": 29,
"end_offset": 51,
"label": "sym"
},
{
"id": 9,
"entity": "细胞",
"start_offset": 51,
"end_offset": 53,
"label": "bod"
},
{
"id": 10,
"entity": "单核细胞",
"start_offset": 54,
"end_offset": 58,
"label": "bod"
},
{
"id": 11,
"entity": "巨核细胞",
"start_offset": 61,
"end_offset": 65,
"label": "bod"
},
{
"id": 12,
"entity": "急性白血病",
"start_offset": 153,
"end_offset": 158,
"label": "dis"
},
{
"id": 13,
"entity": "骨髓衰竭并发症",
"start_offset": 172,
"end_offset": 179,
"label": "dis"
}
] |
患儿经常发生感染，肝、脾、淋巴结增大Down综合征、Fanconi综合征、神经纤维瘤Ⅰ型（NF-1）、Bloom综合征、先天性中性粒细胞减少、血小板储存池病、家族性-7综合征、线粒体细胞病、非特异性免疫缺陷以及不能分类的其他先天性异常等，这些患儿发病年龄大多大于2岁，AML的转化率较原发性儿童MDS为低。
|
[
{
"id": 0,
"entity": "感染",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "肝",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "脾",
"start_offset": 11,
"end_offset": 12,
"label": "bod"
},
{
"id": 3,
"entity": "淋巴结",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 4,
"entity": "肝、脾、淋巴结增大",
"start_offset": 9,
"end_offset": 18,
"label": "sym"
},
{
"id": 5,
"entity": "Down综合征",
"start_offset": 18,
"end_offset": 25,
"label": "dis"
},
{
"id": 6,
"entity": "Fanconi综合征",
"start_offset": 26,
"end_offset": 36,
"label": "dis"
},
{
"id": 7,
"entity": "神经纤维瘤Ⅰ型",
"start_offset": 37,
"end_offset": 44,
"label": "dis"
},
{
"id": 8,
"entity": "NF-1",
"start_offset": 45,
"end_offset": 49,
"label": "dis"
},
{
"id": 9,
"entity": "Bloom综合征",
"start_offset": 51,
"end_offset": 59,
"label": "dis"
},
{
"id": 10,
"entity": "先天性中性粒细胞减少",
"start_offset": 60,
"end_offset": 70,
"label": "sym"
},
{
"id": 11,
"entity": "血小板储存池病",
"start_offset": 71,
"end_offset": 78,
"label": "dis"
},
{
"id": 12,
"entity": "家族性-7综合征",
"start_offset": 79,
"end_offset": 87,
"label": "dis"
},
{
"id": 13,
"entity": "线粒体细胞病",
"start_offset": 88,
"end_offset": 94,
"label": "dis"
},
{
"id": 14,
"entity": "非特异性免疫缺陷",
"start_offset": 95,
"end_offset": 103,
"label": "sym"
}
] |
成人WHOMDS诊断分型标准中按骨髓原始粒细胞比例将RAEB再分为RAEB-Ⅰ（骨髓原始细胞5%～9%）和RAEB-Ⅱ（骨髓原始细胞10%～19%）两型，此外，将MDS和AML骨髓原始细胞的分界降低为0.20，取消了RAEB-t亚型，但现有资料表明这并不适合儿童MDS。
|
[
{
"id": 0,
"entity": "骨髓原始粒细胞",
"start_offset": 16,
"end_offset": 23,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓原始细胞",
"start_offset": 40,
"end_offset": 46,
"label": "bod"
},
{
"id": 2,
"entity": "骨髓原始细胞",
"start_offset": 60,
"end_offset": 66,
"label": "bod"
}
] |
对于那些骨髓原始细胞比例在20%～30%的患儿，如无临床和儿童MDS特征性7号染色单体异常或前述原发性AML特征性染色体核型异常，应在几周后重复骨髓检查，如果骨髓原始细胞比例超过30%则诊断为AML，如果骨髓原始细胞比例保持稳定则诊断为RAEB-t。
|
[
{
"id": 0,
"entity": "骨髓原始细胞",
"start_offset": 4,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓",
"start_offset": 79,
"end_offset": 81,
"label": "bod"
},
{
"id": 2,
"entity": "骨髓",
"start_offset": 102,
"end_offset": 104,
"label": "bod"
}
] |
【诊断】（一）外周血象常表现为一系或一系以上血细胞减少，部分患儿网织红细胞百分率有增高。
|
[
{
"id": 0,
"entity": "血细胞",
"start_offset": 22,
"end_offset": 25,
"label": "bod"
},
{
"id": 1,
"entity": "网织红细胞",
"start_offset": 32,
"end_offset": 37,
"label": "bod"
}
] |
贫血一般呈正细胞、正色素性，红细胞大小不一，可见单个核或多核有核红细胞及卵形大红细胞。
|
[
{
"id": 0,
"entity": "正细胞",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "正色素性",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
}
] |
粒系形态变化较明显，核浆发育不平衡，可出现Pelgen-Huet畸形（分叶减少的中性粒细胞），也可伴分叶过多畸形，或中性粒细胞胞质中颗粒减少，或无颗粒以及其他的形态异常表现。
|
[
{
"id": 0,
"entity": "Pelgen-Huet畸形",
"start_offset": 21,
"end_offset": 34,
"label": "sym"
},
{
"id": 1,
"entity": "分叶减少的中性粒细胞",
"start_offset": 35,
"end_offset": 45,
"label": "bod"
},
{
"id": 2,
"entity": "畸形",
"start_offset": 54,
"end_offset": 56,
"label": "sym"
}
] |
单核细胞常可见增多。
|
[
{
"id": 0,
"entity": "单核细胞",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
}
] |
血小板及其颗粒常减少，可见大型血小板或形态异常，电镜下可呈空泡形成，糖原减少，微小管缺乏，小管系统扩张等变化。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "糖原减少，微小管缺乏，小管系统扩张等变化",
"start_offset": 34,
"end_offset": 54,
"label": "sym"
}
] |
有些患儿血小板计数可正常，但有出血倾向，血小板对胶原、ADP等诱导的聚集作用异常，黏附性降低。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "ADP",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "黏附性降低",
"start_offset": 41,
"end_offset": 46,
"label": "sym"
}
] |
（二）骨髓涂片MDS的骨髓象呈现病态造血的现象。
|
[
{
"id": 0,
"entity": "骨髓涂片",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "骨髓象",
"start_offset": 11,
"end_offset": 14,
"label": "pro"
}
] |
1/2～3/4患儿骨髓有核细胞增生亢进或正常，约1/4左右患儿骨髓增生减低，尤其是继发性MDS骨髓增生常低下，而骨髓增生活跃时常伴有纤维化，因此常出现骨髓不易抽出（“干抽”现象）。
|
[
{
"id": 0,
"entity": "骨髓",
"start_offset": 75,
"end_offset": 77,
"label": "bod"
}
] |
红系病态造血表现为，红系增生过多（＞60%）或过少（＜5%），多数患儿的幼红细胞有巨幼样改变，出现环状铁粒幼红细胞、多核红细胞、核分裂、核凹陷以至核分叶、胞质染色不均匀、多嗜性红细胞及点彩红细胞，尤其MDS转变为白血病前，上述变化为较突出的表现。
|
[
{
"id": 0,
"entity": "环状铁粒幼红细胞",
"start_offset": 49,
"end_offset": 57,
"label": "bod"
},
{
"id": 1,
"entity": "多核红细胞",
"start_offset": 58,
"end_offset": 63,
"label": "bod"
},
{
"id": 2,
"entity": "核分裂",
"start_offset": 64,
"end_offset": 67,
"label": "bod"
},
{
"id": 3,
"entity": "胞质染色不均匀",
"start_offset": 77,
"end_offset": 84,
"label": "bod"
},
{
"id": 4,
"entity": "多嗜性红细胞",
"start_offset": 85,
"end_offset": 91,
"label": "bod"
},
{
"id": 5,
"entity": "点彩红细胞",
"start_offset": 92,
"end_offset": 97,
"label": "bod"
},
{
"id": 6,
"entity": "白血病",
"start_offset": 106,
"end_offset": 109,
"label": "dis"
}
] |
粒系病态造血表现为，颗粒减少或缺如或过大，成熟粒细胞胞质仍嗜碱，呈核浆发育不平衡表现，细胞核分叶过少（Pelger-Hüet异常）或过多。
|
[
{
"id": 0,
"entity": "核浆",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
},
{
"id": 1,
"entity": "细胞核分叶过少",
"start_offset": 43,
"end_offset": 50,
"label": "sym"
},
{
"id": 2,
"entity": "Pelger-Hüet异常",
"start_offset": 51,
"end_offset": 64,
"label": "sym"
}
] |
巨核系病态造血表现为巨核细胞减少，出现小巨核细胞、大单个核巨核细胞、多核巨核细胞、胞质中颗粒加大或形态异常。
|
[
{
"id": 0,
"entity": "巨核细胞",
"start_offset": 10,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "巨核细胞",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 2,
"entity": "大单个核巨核细胞",
"start_offset": 25,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "多核巨核细胞",
"start_offset": 34,
"end_offset": 40,
"label": "bod"
}
] |
小巨核细胞及巨大血小板偶尔出现在外周血中。
|
[
{
"id": 0,
"entity": "小巨核细胞",
"start_offset": 0,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "巨大血小板",
"start_offset": 6,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "外周血",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
}
] |
（三）骨髓活检除了观察骨髓中细胞学改变之外，还可见到下列主要的组织学变化红系前体细胞成熟过程障碍，常形成分化在同一阶段的幼红细胞岛，伴有早幼红细胞增多，骨髓中原粒细胞和早幼粒细胞离开骨小梁附近呈中心性簇生，这些异位的原粒和早幼粒细胞形成聚集（＞5个粒系前体细胞）或小簇（3～5个粒系前体细胞），称为异位的不成熟前体细胞（abnormallocalizationofimmatureprecursor，ALIP），巨核细胞形态异常，表现为体积有显著的大小不一，细胞核呈低分叶的鹿角样和不规则的过多分叶，小型巨核细胞（体积仅为正常的1/6）普遍多见。
|
[
{
"id": 0,
"entity": "骨髓活检",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "骨髓",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "幼红细胞",
"start_offset": 60,
"end_offset": 64,
"label": "bod"
},
{
"id": 3,
"entity": "红细胞",
"start_offset": 70,
"end_offset": 73,
"label": "bod"
},
{
"id": 4,
"entity": "骨髓",
"start_offset": 76,
"end_offset": 78,
"label": "bod"
},
{
"id": 5,
"entity": "早幼粒细胞",
"start_offset": 111,
"end_offset": 116,
"label": "bod"
},
{
"id": 6,
"entity": "异位的不成熟前体细胞",
"start_offset": 149,
"end_offset": 159,
"label": "bod"
},
{
"id": 7,
"entity": "abnormallocalizationofimmatureprecursor",
"start_offset": 160,
"end_offset": 199,
"label": "bod"
},
{
"id": 8,
"entity": "ALIP",
"start_offset": 200,
"end_offset": 204,
"label": "bod"
},
{
"id": 9,
"entity": "巨核细胞",
"start_offset": 206,
"end_offset": 210,
"label": "bod"
},
{
"id": 10,
"entity": "小型巨核细胞",
"start_offset": 250,
"end_offset": 256,
"label": "bod"
}
] |
骨髓组织内细胞增生活跃者（造血组织＞50%）约60%～70%，部分患者增生正常（造血组织30%～50%），少数患者骨髓造血细胞增生减低（＜30%）。
|
[
{
"id": 0,
"entity": "骨髓组织",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "细胞",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "骨髓造血细胞",
"start_offset": 57,
"end_offset": 63,
"label": "bod"
}
] |
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