Efficacy of tranexamic acid for the prevention of bleeding in patients with von Willebrand disease and Glanzmann thrombasthenia: a controlled, before and after trial

Melikof, L; Taherahmadi, Hassan; Bagheri, Bahador

doi:10.1111/hae.13051

Cited by 19 publications

(21 citation statements)

References 20 publications

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“…We conducted 3 systematic reviews of case series, 1 for each of the intervention arms. We included 21 case series: 8 in which patients underwent a desmopressin trial and were treated based on results, [60][61][62][63][64][65][66][67] 9 in which patients did not undergo a desmopressin trial and were treated with VWF concentrate or tranexamic acid, 26,38,45,[68][69][70][71][72][73] and 4 in which patients did not undergo a desmopressin trial but were treated with desmopressin empirically. [74][75][76][77] The EtD framework for this recommendation is available online at https://guidelines.ash.gradepro.org/profile/fnGuafOe504.…”

Section: Recommendation 2bmentioning

confidence: 99%

“…38,45,69 When used for long-term prophylaxis, the strategy of using tranexamic acid only reduced annual oropharyngeal bleeding episodes from 30 to 16. 68 For the strategy of empiric treatment with desmopressin for surgical prophylaxis without the results of a desmopressin trial, 1 study reported that hemostasis was excellent in 93% of patients with mild type 1 VWD and 73% in patients with moderate type 1 VWD, with a mean hospitalization length of 6.3 days. 76 This strategy, when used to manage acute bleeding episodes, was judged to have excellent efficacy in 83% and good efficacy in 14% of 254 bleeding episodes in patients with mild type 1 VWD.…”

Section: Recommendation 2bmentioning

confidence: 99%

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ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Connell

Flood

Brignardello‐Petersen

et al. 2021

Blood Advances

223

331

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Background: von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. Objective: These evidence-based guidelines from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) are intended to support patients, clinicians, and health care professionals in their decisions about management of VWD. Methods: ASH, ISTH, NHF, and WFH formed a multidisciplinary guideline panel. Three patient representatives were included. The panel was balanced to minimize potential bias from conflicts of interest. The University of Kansas Outcomes and Implementation Research Unit and the McMaster Grading of Recommendations Assessment, Development and Evaluation (GRADE) Centre supported the guideline development process, including performing and updating systematic evidence reviews (through November 2019). The panel prioritized clinical questions and outcomes according to their importance to clinicians and patients. The panel used the GRADE approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel agreed on 12 recommendations and outlined future research priorities. Conclusions: These guidelines make key recommendations regarding prophylaxis for frequent recurrent bleeding, desmopressin trials to determine therapy, use of antiplatelet agents and anticoagulant therapy, target VWF and factor VIII activity levels for major surgery, strategies to reduce bleeding during minor surgery or invasive procedures, management options for heavy menstrual bleeding, management of VWD in the context of neuraxial anesthesia during labor and delivery, and management in the postpartum setting.

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Section: Recommendation 2bmentioning

confidence: 99%

Section: Recommendation 2bmentioning

confidence: 99%

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Connell

Flood

Brignardello‐Petersen

et al. 2021

Blood Advances

223

331

View full text Add to dashboard Cite

show abstract

“…In the case of a propensity towards major bleeding after severe trauma, tranexamic acid (1 g iv over 10 min) should be administered as soon as possible, followed by 1 g iv over 8 h [121,122]. For VWD patients with thrombocytopenia, antifibrinolytic therapy may be administered as an adjuvant treatment in cases of oral mucosal bleeding or excessive menstrual bleeding [123,124].…”

Section: Recommendation 17: Antifibrinolytic Therapy Is Required Whenmentioning

confidence: 99%

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Song¹,

Liu

Zhu

et al. 2020

Military Med Res

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Thrombocytopenia is a common complication of critical care patients. The rates of bleeding events and mortality are also significantly increased in critical care patients with thrombocytopenia. Therefore, the Critical Care Medicine Committee of Chinese People's Liberation Army (PLA) worked with Chinese Society of Laboratory Medicine, Chinese Medical Association to develop this consensus to provide guidance for clinical practice. The consensus includes five sections and 27 items: the definition of thrombocytopenia, etiology and pathophysiology, diagnosis and differential diagnosis, treatment and prevention.

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“…22 They have also been administered to people with a variety of hemostatic disorders, including VWD and Glanzmann thrombasthenia, with a clinical assessment of decreased numbers of bleeding episodes when administered prophylactically. 23 Aminocaproic acid was administered to the dog of this report, as drug-related adverse effects are rare 24 , although its efficacy in managing hemorrhage due to intrinsic platelet dysfunction has not been investigated in veterinary medicine. The dog was also treated with fresh whole blood and PRP transfusions shortly after identification of the hemoperitoneum, given the probability of a platelet P2Y12 defect based on the breed.…”

Section: Discussionmentioning

confidence: 99%

Heterozygosity for P2Y12 receptor gene mutation associated with postoperative hemorrhage in a Greater Swiss Mountain dog

Flores

Boudreaux

Vasquez

et al. 2017

Veterinary Clinical Pathol

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A 3-year-old, female Greater Swiss Mountain dog developed a hemoperitoneum following an exploratory laparotomy and ovariohysterectomy. Platelet count, PT, APTT, and plasma von Willebrand factor antigen concentration were within RIs. A buccal mucosal bleeding time (BMBT) was prolonged. Given the probability of a hereditary thrombopathia, the dog was administered desmopressin, fresh platelet transfusions, and aminocaproic acid to control hemorrhage. Subsequently, DNA testing for the P2Y12 receptor gene mutation identified the dog as being a heterozygote (carrier). Further platelet function testing was performed following complete recovery. Results of a repeat BMBT and a point-of-care screening test using the Platelet Function Analyzer-100 (collagen/adenosine-diphosphate [ADP] test cartridge) were within RIs. Flow cytometric studies demonstrated a marked reduction in fibrinogen binding to the dog's platelets in response to ADP -adenosine diphosphate activation. Likewise, turbidimetric aggregometry revealed a complete absence of platelet aggregation in response to ADP. However, there were a normal aggregation response to the platelet agonist convulxin and a mild reduction in amplitude in response to c-thrombin. This is the first report of a dog heterozygous for the P2Y12 receptor gene mutation exhibiting a bleeding tendency and having evidence of impaired platelet function in vitro in response to ADP activation. Given that the mutant allele for the P2Y12 thrombopathia appears to be widespread in the Greater Swiss Mountain dog breed, veterinarians need to be aware that both homozygotes and heterozygotes for this platelet receptor mutation are at risk of developing life-threatening bleeding following trauma or surgery. Case PresentationA 3-year-old, intact female, Greater Swiss Mountain dog was presented to the Matthew J. Ryan Veterinary Hospital of the University of Pennsylvania for gastrointestinal biopsies and a routine ovariohysterectomy (OVH). The dog had a 9-month history of vomiting, large bowel diarrhea, and weight loss that were minimally responsive to metronidazole, tylosin, and a diet change. Initial diagnostic evaluation included a CBC, serum chemistry profile, adrenocorticotropic hormone (ACTH) stimulation test, and serum cobalamin and folate concentrations, with all results within RIs. A zinc sulfate fecal flotation was negative for parasites. An abdominal ultrasonographic examination revealed small intestinal submucosal thickening and mesenteric and iliac lymphadenopathy.Preoperative PCV and total protein (TP) were 45% (RI 40-60%) and 6.0 g/dL (RI 5.5-7.5 g/dL), respectively. The dog was placed under general anesthesia, and a colonoscopy with colonic biopsies was performed, followed by an exploratory laparotomy with full thickness biopsies of the duodenum, jejunum, and ileum, and an OVH. The procedures were uncomplicated; however, mild oozing of blood was noted from the skin incision, body wall, and multiple surfaces within the abdomen. One day postoperatively, tachycardia, pallor, and a moder...

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Efficacy of tranexamic acid for the prevention of bleeding in patients with von Willebrand disease and Glanzmann thrombasthenia: a controlled, before and after trial

Abstract: The results suggest that TXA is safe and effective to reduce bleeding and use of factor concentrates in VWD patients. In addition, TXA therapy can decrease bleeding in GT patients.

Cited by 19 publications

References 20 publications

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease

Expert consensus on the diagnosis and treatment of thrombocytopenia in adult critical care patients in China

Heterozygosity for P2Y12 receptor gene mutation associated with postoperative hemorrhage in a Greater Swiss Mountain dog

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