脾切除术揭示了伴有肝外门静脉阻塞的骨髓增生性肿瘤潜在的血小板增多症。

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL
Tetsuya Shimizu, Hiroshi Yoshida, Nobuhiko Taniai, Ryuji Ohashi, Yoichi Kawano, Junji Ueda, Takuma Iwai, Akira Matsushita, Masato Yoshioka, Takahiro Murokawa, Toshiyuki Irie, Takashi Ono, Takahiro Haruna, Daigo Yoshimori, Akira Hamaguchi
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引用次数: 0

摘要

肝外门静脉阻塞(EHPVO)是一种罕见疾病,骨髓增生性肿瘤(MPN)是最常见的病因。我们报告称,MPN 可消除 EHPVO 中的脾功能亢进血液学改变。通过脾切除术控制 EHPVO 静脉曲张的经验,我们怀疑脾脏可能会掩盖 MPN 诱导的血小板增多,而 MPN 可能对脾切除术后血小板过多有重要影响。为了明确 MPN 和脾脏对血小板趋势的影响,我们进行了一项回顾性医院数据库分析,评估了 8 例接受脾脏切除术的 EHPVO 患者(2 男 6 女;年龄从 17 岁到 64 岁,平均 38.3 岁)。8人中有3人(37.5%)因JAK2V617F突变而被诊断为骨髓增生性疾病。无 MPN 的 EHPVO 患者术前、术后 1 周和 3 周的围手术期血清血小板计数分别为 10.5、35.4 和 36.6 (x104/μL)。伴有MPN的EHPVO的血小板计数分别为34.2、86.4和137.0(x104/μL)。脾切除术与 MPN 对血小板增加的影响呈正相关,具有统计学意义。我们还研究了脾脏体积指数(SpVI:脾脏体积(cm3)/体表面积(m2))和术后血小板升高比值(PER:术后 3 周血小板计数/术前血小板计数)。然而,SpVI 和 PER 在有无 MPN 的情况下没有明显差异。组织学检查显示,所有8例EHPVO病例均存在脾充血,而3例MPN病例中有2例存在脾髓外造血。在伴有 MPN 的 EHPVO 患者中,脾功能亢进会掩盖 MPN 引起的血小板增多,从而导致血小板计数假性正常化;然而,脾切除术会揭示术后血小板增多。伴有 MPN 的 EHPVO 患者的脾脏也参与髓外造血。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Splenectomy unveils thrombocytosis in underlying myeloproliferative neoplasms with extrahepatic portal vein obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease with myeloproliferative neoplasm (MPN) as the most common cause. We report that hypersplenic hematologic changes in EHPVO might be eliminated by MPN. Through experience with splenectomy for variceal control with EHPVO, we suspected that spleen might mask MPN-induced thrombocytosis, and that MPN might have a significant influence on excessive thrombocytosis after splenectomy. To clarify the influence of MPN and spleen on platelet trends, we conducted a retrospective hospital database analysis, evaluating 8 EHPVO patients with splenectomy (2 males, 6 females; from 17 years to 64 years, mean 38.3 years). Three (37.5%) of 8 were diagnosed as MPN by JAK2V617F mutation. The perioperative serum platelet counts in EHPVO without MPN were 10.5, 35.4, and 36.6 (x104/μL) preoperatively, after 1 week and 3 weeks, respectively. The platelet counts in EHPVO with MPN were 34.2, 86.4, and 137.0 (x104/μL), respectively. Splenectomy and MPN showed positive interaction on platelet increasing with statistical significance. We also examined the spleen volume index (SpVI: splenic volume (cm3) / body surface area (m2) and postoperative platelet elevations ratio (PER: 3-week postoperative platelet counts / preoperative platelet counts). However, both SpVI and PER showed no significant difference with or without MPN. Histological examination revealed splenic congestion in all 8 EHPVO cases, and splenic extramedullary hematopoiesis in 2 of 3 MPN. In EHPVO with MPN, hypersplenism causes feigned normalization of platelet count by masking MPN-induced thrombocytosis; however, splenectomy unveils postoperative thrombocytosis. Spleen in EHPVO with MPN also participates in extramedullary hematopoiesis.

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来源期刊
Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
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29
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