Unclassified bleeding disorders: outcome of haemostatic challenges following tranexamic acid and/or desmopressin

Obaji, Samya; Alikhan, Raza; Rayment, Rachel; Carter, P.; Macartney, Nicola J.; Collins, Peter William

doi:10.1111/hae.12811

Cited by 33 publications

(47 citation statements)

References 18 publications

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“…In our collection, 13 childbirths were covered with TxA, desmopressin or platelets with 75% having no abnormal bleeding and no cases needing red cell transfusion. Obaji et al described 4 deliveries where TXA and desmopressin were successfully used to manage delivery . The variability we describe in management with platelets, TXA and desmopressin reflects the uncertainty of managing UBD given the lack of any clinical trials.…”

Section: Discussionmentioning

confidence: 89%

“…Patients with unclassified bleeding disorder (UBD) have been identified in a number of studies yet they remain poorly characterized . Evidence guiding management of patients needing procedures with UBD is scarce, though tranexamic acid (TxA) and desmopressin have been described . The optimal investigational strategy has not been established, and it is unknown whether global assays of haemostasis (viscoelastometry and thrombin generation [TG]) can help with refinement of the clinical picture.…”

Section: Introductionmentioning

confidence: 99%

“…[3][4][5][6][7][8][9][10] Evidence guiding management of patients needing procedures with UBD is scarce, though tranexamic acid (TxA) and desmopressin have been described. 11 The optimal investigational strategy has not been established, and it is unknown whether global assays of haemostasis (viscoelastometry and thrombin generation [TG]) can help with refinement of the clinical picture. These assays have been used at our institution to assess patients with UBD.…”

Section: Introductionmentioning

confidence: 99%

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Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

MacDonald

Wright

Beuche

et al. 2019

Int J Lab Hematology

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Introduction There is an unmet need to characterize the diagnosis and management of patients with an unclassified bleeding disorder (UBD). Methods Retrospective review of registered patients with UBD at our centre. Assessment including rotational thromboelastometry (ROTEM) and thrombin generation (TG) were used. Results A total of 124 patients were identified; 91% female. Mean age of presentation was 38.3 years. Mean bleeding score was 8.8 (standard deviation [SD] 3.8); 6.6 in men (SD 1.4) and 9.7 in women (SD 3.3), which was significantly different (P < .05). In women, after deduction of scores for menorrhagia and postpartum haemorrhage, the mean score was 6.4 which was not significantly different to the male score (P = .11). Twenty‐three percent of patients have been transfused, 61% women had treatment for menorrhagia and 17% for epistaxis. TxA and desmopressin were effective at preventing bleeding in 69 procedures and 13 deliveries. TG revealed 26% patients with a long lag time and 19% with a decreased endogenous thrombin potential but no diagnostic pattern was seen. ROTEM (NATEM) was unable to characterize patients; 9% had a prolonged clot time or maximum lysis. ThromboGenomics was normal in 45 tested patients. Conclusions We provide data which shows the bleeding score is biased towards gynaecological bleeding but which remains elevated even when the bleeding score is deducted. Tranexamic acid and desmopressin are effective as haemostatic prophylaxis but there is an urgent need for clinical trials. In conclusion, we describe the use of the bleeding score in these patients and phenotype, diagnosis (including ThromboGenomic testing) and management with practice recommendations.

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Section: Discussionmentioning

confidence: 89%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

MacDonald

Wright

Beuche

et al. 2019

Int J Lab Hematology

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show abstract

“…There is evidence suggesting that in patients with unclassified bleeding disorders, prophylaxis with TXA ± DDAVP prior to major and minor procedures was successful at ameliorating bleeding in nearly all patients. 10 In our study, TXA alone was often prescribed to patients prior to surgery to prevent abnormal bleeding, the efficacy of which was not explored in the current study.…”

Section: Laboratory Investigation For Vwd Was Normal For All Patientsmentioning

confidence: 97%

The bleeding score: Useful in predicting spontaneous bleeding events in adults with bleeding of unknown cause?

et al. 2019

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“…Bu ilaçların da faktör XI eksikliğine bağlı kanamalarda kullanılabileceği bildirilmiştir. Ayrıca desmopressin'in FXI aktivitesini arttırdığı gösterilmiştir (4,(7)(8)(9). Bizim hastamızda faktör replasmanı için tercih edilen ürün TDP oldu.…”

Section: Olgu Sunumuunclassified

Anaesthesia Management of a Patient with Factor XI Deficiency

Adibelli¹,

Araz²,

Ersoy³

et al. 2016

Turk J Anaesth Reanim

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Faktör XI eksikliği oldukça nadir görülen travma veya cerrahi gibi başlatıcı bir sebep olmadığı durumlarda klinik bulgu vermeyen bir hastalıktır. Sağlıklı bireylerde faktör düzeyi 70-150 U dL-1 arasın-da bulunur. Bu düzeyin altında, klinik bulgu gözlenmeden sadece aPTT yüksekliği görülebilir. Bu uzamanın saptanması durumunda hastalarda faktör XI eksikliğinden şüphelenerek faktör düzeyi çalışıl-malıdır. Eksiklik saptanan hastaların preoperatif hazırlıklarının dikkatli şekilde yapılmasıyla cerrahi sırasında ve sonrasında olası komplikasyonlar azaltılabilir. Bu olgu sunumunda, faktör XI eksikliği bulunan bir hastanın total kalça protezi operasyonu sırasındaki anestezi yöntemi sunulmaktadır.Anahtar kelimeler: Faktör XI eksikliği, anestezi yönetimi, total kalça protezi Factor XI deficiency is an extremely rare disease presenting no clinical symptoms, unless there is an inducing reason such as trauma or surgery. Normally, factor levels are in the range of 70-150 U dL-1 in healthy subjects. Although no clinical symptoms are seen, only high levels of aPTT can be found. Once a prolongation is detected in aPTT, factor XI deficiency should be suspected and factor levels should be analysed. With careful preoperative preparations in factor-deficient people, preoperative and postoperative complications can be decreased. In this case report, management of anaesthesia during total hip arthroplasty of a patient with factor XI deficiency is presented. Keywords GirişF aktör XI eksikliği, nadir olarak görülen bir faktör eksikliği olup, kodlayan geni 4. kromozom üzerindedir. Faktör eksikliği bulunan hastalarda spontan kanamalar nadirdir, ancak cerrahi müdahaleler ve travma sonrası önemli kanamalar mümkündür. İzole uzamış aktive parsiyel tromboplastin zamanı (aPTT) değerleri faktör XI eksikliği için uyarıcı olmalıdır (1). Tedavide temel yöntem eksik olan faktörün yerine koyulmasıdır (2). Bu olgu sunumunda total kalça protezi cerrahisi geçirecek olan ve faktör XI eksikliği bulunan hastanın anestezi yönetimi sunulmaktadır. Olgu SunumuBu olgu sunumu için hasta bilgilendirilmiş ve izini alınmıştır. İki yıldır faktör XI eksikliği nedeniyle izlenmekte olan 59 yaşında kadın hasta, sol total kalça protezi ameliyatı öncesi değerlendirildi. Faktör XI eksikliği yanı sıra, allerjik astım, ve tiroid nodülü olduğunu belirtti. Yapılan incelemelerde hastanın solunum fonksiyonları ve tiroid fonksiyon testlerinin normal sı-nırlarda olduğu görüldü. Faktör eksikliği tanısının daha önceki ameliyatı sırasında aile hikayesi bulunması nedeniyle konulduğu, küçük cerrahi işlem nedeniyle herhangi bir profilaksi uygulanmadığı ve ciddi bir komplikasyon yaşamadığı öğrenildi.Hastanın hematolojik değerlendirilmesinde aPTT değeri 77 saniye ve faktör XI düzeyi 9 U dL -1 (Normal değeri 70-150 U dL -1 ) olduğu gözlendi. Preoperatif hemoglobin değeri 13,6 g dL -1 ve trombosit sayısı 273 bin mm 3-1 olarak belirlendi. Hematoloji bölümü tarafından girişim öncesi 15-20 mL kg -1 , postoperatif 12. saatte 5 mL kg -1 ve kanamanın devamı halinde 12 saat aralıklarla 5 mL kg -1 ...

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Unclassified bleeding disorders: outcome of haemostatic challenges following tranexamic acid and/or desmopressin

Abstract: This is the first study to report on the investigation and treatment of UBD. Future studies are needed to further our understanding of the bleeding phenotype and identify any underlying causes.

Cited by 33 publications

References 18 publications

Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

The bleeding score: Useful in predicting spontaneous bleeding events in adults with bleeding of unknown cause?

Anaesthesia Management of a Patient with Factor XI Deficiency

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