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Esophageal Varices-II: Clinical Features and Management01:28

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Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
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Related Experiment Video

Updated: Jul 12, 2025

Laparoscopic Splenectomy with Pericardial Devascularization for Hypersplenism and Esophageal Variceal Hemorrhage Due to Portal Hypertension
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Laparoscopic Splenectomy with Pericardial Devascularization for Hypersplenism and Esophageal Variceal Hemorrhage Due to Portal Hypertension

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Surgery in rare bleeding disorders: the prospective MARACHI study.

Florence Rousseau1,2, Benoit Guillet3, Thibault Mura4

  • 1Département d'hématologie biologique, CHU Montpellier, France.

Research and Practice in Thrombosis and Haemostasis
|October 23, 2023
PubMed
Summary

Bleeding risk in rare inherited bleeding disorders is complex. Factor levels and surgery type, not bleeding scores, best guide perioperative factor replacement therapy for safe surgical outcomes.

Keywords:
bleeding scorehemorrhagic disordersrare bleeding disorderreplacement therapysurgerytranexamic acid

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Area of Science:

  • Hematology
  • Surgical Management
  • Rare Diseases

Background:

  • Bleeding risk stratification before surgery is challenging for patients with rare inherited bleeding disorders.
  • Existing methods like bleeding scores and clotting factor levels have limitations.

Purpose of the Study:

  • To assess perioperative hemostatic management choices by experts in patients with rare coagulation factor deficiency.
  • To evaluate bleeding outcomes after surgery in this patient population.

Main Methods:

  • A multicenter observational prospective study included 178 patients with low coagulation activity (factor levels <50%).
  • 207 surgical procedures were analyzed, collecting data on bleeding outcomes, Tosetto's bleeding score, and perioperative hemostatic protocols.

Main Results:

  • 27/81 procedures in severe deficiency (≤10% factor level) were performed without factor replacement, including 6 high-risk cases, with no bleeding events.
  • 100/126 procedures in mild deficiency were done without hemostatic treatment.
  • Factor replacement was guided by procedure, bleeding risk, and history, with tranexamic acid used in nearly half of procedures.

Conclusions:

  • Current perioperative hemostatic management for rare bleeding disorders is effective, with only 6/207 procedures experiencing excessive bleeding.
  • Factor level and surgery type are more critical than bleeding scores for guiding perioperative factor replacement therapy.