術前にプロトロンビン複合体製剤の予防投与を行ったProthrombin Himiの1例

Yusuke Kamihara; Shohei Kikuchi; Akinori Wada; Jun Murakami; Nana Sugie; Hideki Niimi; Akitoshi Nakashima; Tsutomu Sato

doi:10.11406/rinketsu.62.1412

術前にプロトロンビン複合体製剤の予防投与を行ったProthrombin Himiの1例

Translated title of the contribution: Successful preoperative replacement of prothrombin with prothrombin complex concentrate for Prothrombin Himi

Yusuke Kamihara, Shohei Kikuchi, Akinori Wada, Jun Murakami, Nana Sugie, Hideki Niimi, Akitoshi Nakashima, Tsutomu Sato

Research output: Contribution to journal › Article › peer-review

Abstract

Dysprothrombinemia is the rarest inherited bleeding disorder that is characterized by a decrease in the prothrombin activity, but normal antigen levels. In this study, we report the case of a compound heterozygote of two mutations in prothrombin; Met337Thr and Arg388His, which has previously been identified as "Prothrombin Himi." A systemic blood coagulation evaluation revealed a prolonged prothrombin time (39%) and activated partial thromboplastin (64.4 sec), with an isolated severe decrease in the prothrombin activity (8.6%). Preoperative replacement of prothrombin with prothrombin complex concentrate, PPSB-HT "Nichiyaku," successfully prevented abnormal postoperative bleeding after laparoscopic hysterectomy for cervical cancer. This is the second reported case of Prothrombin Himi.

Translated title of the contribution	Successful preoperative replacement of prothrombin with prothrombin complex concentrate for Prothrombin Himi
Original language	Japanese
Pages (from-to)	1412-1414
Number of pages	3
Journal	[Rinshō ketsueki] The Japanese journal of clinical hematology
Volume	62
Issue number	9
DOIs	https://doi.org/10.11406/rinketsu.62.1412
State	Published - 2021
Externally published	Yes

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@article{a8fe4250ea1842109d564ee74dff56bd,

title = "術前にプロトロンビン複合体製剤の予防投与を行ったProthrombin Himiの1例",

abstract = "Dysprothrombinemia is the rarest inherited bleeding disorder that is characterized by a decrease in the prothrombin activity, but normal antigen levels. In this study, we report the case of a compound heterozygote of two mutations in prothrombin; Met337Thr and Arg388His, which has previously been identified as {"}Prothrombin Himi.{"} A systemic blood coagulation evaluation revealed a prolonged prothrombin time (39%) and activated partial thromboplastin (64.4 sec), with an isolated severe decrease in the prothrombin activity (8.6%). Preoperative replacement of prothrombin with prothrombin complex concentrate, PPSB-HT {"}Nichiyaku,{"} successfully prevented abnormal postoperative bleeding after laparoscopic hysterectomy for cervical cancer. This is the second reported case of Prothrombin Himi.",

keywords = "Blood Coagulation Factors, Humans, Prothrombin",

author = "Yusuke Kamihara and Shohei Kikuchi and Akinori Wada and Jun Murakami and Nana Sugie and Hideki Niimi and Akitoshi Nakashima and Tsutomu Sato",

year = "2021",

doi = "10.11406/rinketsu.62.1412",

language = "日本",

volume = "62",

pages = "1412--1414",

journal = "[Rinshō ketsueki] The Japanese journal of clinical hematology",

issn = "0485-1439",

publisher = "Nihon Rinsho Ketsueki Gakkai/Japan Society of Clinical Hematology",

number = "9",

}

TY - JOUR

T1 - 術前にプロトロンビン複合体製剤の予防投与を行ったProthrombin Himiの1例

AU - Kamihara, Yusuke

AU - Kikuchi, Shohei

AU - Wada, Akinori

AU - Murakami, Jun

AU - Sugie, Nana

AU - Niimi, Hideki

AU - Nakashima, Akitoshi

AU - Sato, Tsutomu

PY - 2021

Y1 - 2021

N2 - Dysprothrombinemia is the rarest inherited bleeding disorder that is characterized by a decrease in the prothrombin activity, but normal antigen levels. In this study, we report the case of a compound heterozygote of two mutations in prothrombin; Met337Thr and Arg388His, which has previously been identified as "Prothrombin Himi." A systemic blood coagulation evaluation revealed a prolonged prothrombin time (39%) and activated partial thromboplastin (64.4 sec), with an isolated severe decrease in the prothrombin activity (8.6%). Preoperative replacement of prothrombin with prothrombin complex concentrate, PPSB-HT "Nichiyaku," successfully prevented abnormal postoperative bleeding after laparoscopic hysterectomy for cervical cancer. This is the second reported case of Prothrombin Himi.

AB - Dysprothrombinemia is the rarest inherited bleeding disorder that is characterized by a decrease in the prothrombin activity, but normal antigen levels. In this study, we report the case of a compound heterozygote of two mutations in prothrombin; Met337Thr and Arg388His, which has previously been identified as "Prothrombin Himi." A systemic blood coagulation evaluation revealed a prolonged prothrombin time (39%) and activated partial thromboplastin (64.4 sec), with an isolated severe decrease in the prothrombin activity (8.6%). Preoperative replacement of prothrombin with prothrombin complex concentrate, PPSB-HT "Nichiyaku," successfully prevented abnormal postoperative bleeding after laparoscopic hysterectomy for cervical cancer. This is the second reported case of Prothrombin Himi.

KW - Blood Coagulation Factors

KW - Humans

KW - Prothrombin

U2 - 10.11406/rinketsu.62.1412

DO - 10.11406/rinketsu.62.1412

M3 - 学術論文

C2 - 34615802

SN - 0485-1439

VL - 62

SP - 1412

EP - 1414

JO - [Rinshō ketsueki] The Japanese journal of clinical hematology

JF - [Rinshō ketsueki] The Japanese journal of clinical hematology

IS - 9

ER -

術前にプロトロンビン複合体製剤の予防投与を行ったProthrombin Himiの1例

Abstract

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