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 |
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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 | |
State | Published - 2021 |
Externally published | Yes |