病例报告 Open Access
Copyright ©The Author(s) 2006. Published by Baishideng Publishing Group Inc. All rights reserved.
世界华人消化杂志. 2006-07-28; 14(21): 2146-2148
在线出版日期: 2006-07-28. doi: 10.11569/wcjd.v14.i21.2146
胃恶性间质瘤合并继发性血小板增多症1例
马向涛, 余力伟, 付静
马向涛, 余力伟, 北京市海淀医院外科 北京市 100080
付静, 北京市海淀医院病理科 北京市 100080
通讯作者: 马向涛, 100080, 北京市海淀区中关村大街29号, 北京市海淀医院外科. xiangtao_ma@pku.org.cn
电话: 010-62583013 传真：010-62653601
收稿日期: 2006-04-28
修回日期: 2006-05-20
接受日期: 2006-05-24
在线出版日期: 2006-07-28

胃恶性间质瘤患者1例, 临床表现为继发性血小板增多症. 手术后血小板数量恢复正常, 血小板增多症可能是反应肿瘤负荷的重要指标.

关键词: 血小板增多症; 胃肠道间质瘤
引文著录: 马向涛, 余力伟, 付静. 胃恶性间质瘤合并继发性血小板增多症1例. 世界华人消化杂志 2006; 14(21): 2146-2148
N/A
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Correspondence to: N/A
Received: April 28, 2006
Revised: May 20, 2006
Accepted: May 24, 2006
Published online: July 28, 2006

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Key Words: N/A
0 引言

血小板增多症(thrombocythemia)分原发性和继发性两类, 原发性血小板增多症原因不明, 多见于成人, 血小板计数常在800×109/L-1000×109/L以上且形态功能异常, 有出血倾向和栓塞症状. 继发性血小板增多症的原因很多, 如病毒、细菌感染、外伤、出血、营养缺乏、肾病、肿瘤与遗传性疾病等[1-3], 我院收治1例胃恶性间质瘤患者合并继发性血小板增多症, 现报告如下.

1 病例报告
1.1 材料

本组男19例, 女10例, 平均年龄31岁; 住院1次18例, 住院2次10例, 住院3次1例, 平均住院天数47 d, 所有病例均经B超或CT诊断, 最大囊肿25 cm×16 cm, 最小11 cm×10 cm, 平均15 cm×13 cm, 急性胰腺炎引起15例, 慢性胰腺炎引起4例, 外伤引起6例, 其他原因4例. 头部囊肿14例, 体部囊肿6例, 尾部囊肿7例, 2例患者同时有2个囊肿.

1.2 方法

女, 56岁, 因左上腹胀痛伴坠胀感8 wk, 于2005-07-13入院.患者8 wk前无诱因出现左上腹胀痛伴坠胀感, 左侧卧位时胀痛感明显.开始持续数秒钟, 逐渐持续几分钟, 可自行缓解.患者自发病以来无烦躁易怒, 无多饮多食, 无多汗心悸, 无恶心呕吐, 无返酸嗳气.患者既往无特殊病史.查体: 腹平软, 左上腹肋下可触及约8 cm×6 cm大小质硬肿物, 无压痛, 与皮肤无黏连.肝、脾肋下未触及.实验室检查: 血白细胞4.2×109/L, 中性粒细胞0.553, 血红蛋白126 g/L, 血小板623×109/L, PT 12.8 s, APTT 40.6 s. 总蛋白68.3 g/L, 白蛋白41 g/L, 血电解质正常, CEA 0.45 μg/L(正常值0-3.4 μg/L), CA19-9 4.6 kU/L (正常值0-35 kU/L). 胸片: 双肺未见异常. 腹部B超: 肝、胆、胰、脾及双肾未见异常. B超: 左上腹可见18 cm×15 cm×13 cm混合回声, 自胰尾部向左, 脾脏受压向下移位. 腹部MRI:左上腹巨大占位, 位于肝左叶及脾胃之间, 可见包膜. 术前诊断为胃间质瘤, 完善术前准备后于2005-07-18全麻下行剖腹探查术. 术中见肿瘤位于脾胃之间, 直径约18 cm, 色暗红, 表面遍布曲张血管, 有宽3 cm蒂连接于胃体大弯侧, 探查腹腔内其余脏器未见异常, 术中诊断为胃间质瘤, 完整切除肿瘤, 术后病理报告: 胃壁恶性间质瘤Ⅳ级, 直径约18 cm, 质量2000 g, 伴广泛出血坏死及囊性变, 累及浆膜层及深肌层, 免疫组化提示主要向神经分化. 胃壁黏膜可见脐形溃疡形成, 黏膜及黏膜下未见肿瘤累及. 术后患者恢复好, 血小板降至224×109/L, 患者目前仍在随访中.

2 讨论

临床上引起继发性血小板增多症的原因很多, 如病毒、细菌感染、外伤、出血、营养缺乏、肾病、肿瘤与遗传性疾病等, 其中肿瘤导致的继发性血小板增多症占所有病因的1/3[4-6]. 引起血小板增多症的常见肿瘤包括: 肺癌、肾癌、乳腺癌、卵巢癌与白血病等[7-11], 而继发于胃肠道间质瘤的病例国内外未见报道. 胃肠道间质瘤(gastrointestinal stromal tumor, GIST)过去曾被诊为平滑肌源或神经源性肿瘤. 近年来随着电镜和免疫组织化学等技术的应用和研究证实GIST是消化道独立的一类间叶肿瘤, 可能起源于Cajal间质细胞[12-14]. 继发性血小板增多症中血小板形态、功能与生存时间一般正常, 血小板计数大多在400×109/L-1000×109/L[15-16]. 本例患者诊断为胃恶性间质瘤的同时发现血小板增多症, 最高达623×109/L, 手术切除肿瘤后血小板计数下降至224×109/L.

本例继发性血小板增多症考虑为肿瘤本身分泌或代谢产物导致的副肿瘤综合征(paraneoplastic syndrome). 副肿瘤综合征是指由肿瘤产生的生物活性物质导致的, 与肿瘤原发灶或转移部位无直接关系的各种症状和体征[17-19]. 其中肿瘤导致的继发性血小板增多症与多种细胞因子的产生有关, 包括血小板生成素(TPO)、IL-6以及巨噬细胞集落刺激因子等[20-23]. 血小板生成素作为巨核细胞生长因子主要于肝脏合成, 肾、骨髓与脾也可以合成TPO. 循环中TPO水平与调节巨核细胞及血小板生成的TPO受体水平成反比[24-25]. 当血小板减少时, TPO受体下调, TPO水平升高, 促进血小板生成. 血小板富含血小板衍生生长因子(platelet-derived growth factor, PDGF)与血小板反应蛋白[26-27]. PDGF与血小板反应蛋白在介导肿瘤细胞黏附过程中起重要作用, 并且可以通过尿激酶型纤溶酶原激活因子(urokinase-type plasminogen activator)促进肿瘤细胞转移[28-30]. 研究发现, 合并有血小板增多症的肝癌患者血清中TPO水平也同步增高. 手术切除病灶或给与肝动脉栓塞化疗后, 血小板与TPO水平均下降, 血小板增多症可能是反应肿瘤负荷的重要指标[31-33].

评论
背景资料

血小板增多症分原发性和继发性两类, 原发性血小板增多症原因不明, 多见于成人. 继发性血小板增多症的原因包括: 病毒、细菌感染、外伤、出血、营养缺乏、肾病、肿瘤与遗传性疾病等. GIST发病与转移机制是近期研究的重点.

应用要点

血小板增多症可能是反应GIST肿瘤负荷的重要指标.

同行评价

GIST报道逐年增多, 但是合并血小板增多症的病例尚不多见, 对临床有一定参考价值.

电编:李琪 编辑:潘伯荣

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