A <i>CSF3R</i> T618I Mutation in a Patient with Chronic Neutrophilic Leukemia and Severe Bleeding Complications

Summary. Background: To assess the impact of short- vs. long-term anticoagulation in addition to standard dual antiplatelet therapy (DAPT) upon endovascular treatment of (sub)acute thrombembolic occlusions of the lower extremity. Patient and methods: Retrospective analysis was conducted on 202 patients with a thrombembolic occlusion of lower extremities, followed by crirical limb ischemia that received endovascular treatment including thrombolysis, mechanical thrombectomy, or a combination of both between 2006 and 2015 at a single center. Following antithrombotic regimes were compared: 1) dual antiplatelet therapy, DAPT for 4 weeks (aspirin 100 mg/d and clopidogrel 75 mg/d) upon intervention, followed by a lifelong single antiplatelet therapy; 2) DAPT plus short term anticoagulation for 4 weeks, followed by a lifelong single antiplatelet therapy; 3) DAPT plus long term anticoagulation for > 4 weeks, followed by a lifelong anticoagulation. Results: Endovascular treatment was associated with high immediate revascularization (> 98 %), as well as overall and amputation-free survival rates (> 85 %), independent from the chosen anticoagulation regime in a two-year follow up, p > 0.05. Anticoagulation in addition to standard antiplatelet therapy had no significant effect on patency or freedom from target lesion revascularization (TLR) 24 months upon index procedure for both thrombotic and embolic occlusions. Severe bleeding complications occurred more often in the long-term anticoagulation group (9.3 % vs. 5.6 % (short-term group) and 6.5 % (DAPT group), p > 0.05). Conclusions: Our observational study demonstrates that the choice of an antithrombotic regime had no impact on the long-term follow-up after endovascular treatment of acute thrombembolic limb ischemia whereas prolonged anticoagulation was associated with a nominal increase in severe bleeding complications.

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Individualized antithrombotic therapy in high risk patients after coronary stenting. A double-edged sword between thrombosis and bleeding

Thrombosis and Haemostasis ◽

10.1160/th07-11-0680 ◽

2008 ◽

Vol 99 (03) ◽

pp. 487-493 ◽

Cited By ~ 33

Author(s):

Tobias Geisler ◽

Meinrad Gawaz ◽

Andreas May

Keyword(s):

High Risk ◽

Antiplatelet Therapy ◽

Stent Thrombosis ◽

Antithrombotic Therapy ◽

Bleeding Complications ◽

Severe Bleeding ◽

High Risk Patients ◽

Coronary Stent Implantation ◽

Risk Patients ◽

The Individual

SummaryDual antiplatelet therapy with aspirin and clopidogrel is currently the standard therapy after coronary stent implantation to prevent a life-threatening stent thrombosis. However, a variety of procedural and individual factors contribute to the individual patient risk and have to be taken into account to allow for an individual recommendation for both the duration and intensity of the antiplatelet therapy. Obviously, the benefit of the prevention of stent thrombosis by antithrombotic therapy has to outweigh the risk of severe bleeding complications. Depending on the individual clinical situation and procedural characteristics (stent type, length, angiographic result etc.), the recommended duration of the combined antiplatelet therapy currently varies from four weeks to at least one year.These recommendations are mainly based on large, prospective, randomized trials and evidence-based guidelines. However, in a subgroup of high-risk patients there is insufficient evidence for the benefit of conventional dual antiplatelet regimen.These include i) patients with an indication for anticoagulation, ii) patients with urgent need for an operation requiring a perioperative withholding of antiplatelet therapy, as well as iii) clopidogrel low responders.This review aims to provide a stratification to define patient collectives who may benefit from more individualized antithrombotic regimens on behalf of currently available literature and guidelines.

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Bleeding and haemostasis disorders

10.1093/med/9780199687039.003.0070_update_003 ◽

2018 ◽

Author(s):

Pier Mannuccio Mannucci ◽

Maddalena Lettino

Keyword(s):

Artery Bypass ◽

Coronary Intervention ◽

Adverse Outcomes ◽

High Rate ◽

Bleeding Complications ◽

Severe Bleeding ◽

Preventive Strategy ◽

Stent Deployment ◽

Coronary Syndrome ◽

Coronary Syndromes

The main cause of haemostasis defects and related bleeding complications in patients with acute coronary syndromes admitted to the intensive cardiac care unit is the use of multiple antithrombotic drugs, alone or concomitantly with invasive procedures such as percutaneous coronary intervention with stent deployment and coronary artery bypass surgery. These drugs, that act upon several components of haemostasis (platelet function, coagulation, fibrinolysis), are associated with bleeding complications, particularly in elderly patients (more so in women than in men), those who are underweight, and those with comorbid conditions such as renal and liver insufficiency and diabetes. The identification of patients at higher risk of bleeding is the most important preventive strategy. Red cell and platelet transfusions, which may become necessary in patients with severe bleeding, should be used with caution, because transfused patients with acute coronary syndrome have a high rate of adverse outcomes (death, myocardial infarction, and stroke).

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Case 17: Severe bleeding complications, lymphocytosis and leukoerythroblastosis – disseminated intravascular coagulation in a patient with metastasizing carcinoma of the prostate and chronic lymphocytic leukemia

Therapeutische Umschau ◽

10.1024/0040-5930.56.9.533 ◽

1999 ◽

Vol 56 (9) ◽

pp. 533-536 ◽

Cited By ~ 1

Author(s):

Solenthaler ◽

Lämmle

Keyword(s):

Chronic Lymphocytic Leukemia ◽

Disseminated Intravascular Coagulation ◽

Lymphocytic Leukemia ◽

Bleeding Complications ◽

Severe Bleeding ◽

Disseminierte Intravasale Gerinnung ◽

Intravascular Coagulation ◽

Carcinoma Of The Prostate

Blutungskomplikationen bei chronischer lymphatischer Leukämie (CLL) stehen meist in Zusammenhang mit einer Thrombozytopenie, bedingt entweder durch eine direkte Verdrängung der Megakaryopoese bei diffuser Knochenmarksinfiltration oder durch einen vermehrten peripheren Verbrauch im Rahmen einer (sekundären) Immunthrombozytopenie. Eine disseminierte intravasale Gerinnung (DIC) gehört nicht zum Spektrum hämatologischer Komplikationen einer CLL. Das hier geschilderte Fallbeispiel eines Patienten mit schweren Blutungskomplikationen, labormäßigen Zeichen einer DIC und neu entdeckter CLL ließ eine Ursache der DIC vermissen. Das leukoerythroblastäre Blutbild lieferte den Schlüssel zur Diagnose eines metastasierenden Prostatakarzinoms, welches durch die Knochenmarksbiopsie bestätigt wurde und als Trigger für die DIC verantwortlich war.

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Activated Recombinant Factor VII (rFVIIa/NovoSeven®) in the Treatment of Bleeding Complications Following Hematopoietic Stem Cell Transplantation (HSCT).

Blood ◽

10.1182/blood.v104.11.1135.1135 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1135-1135 ◽

Cited By ~ 1

Author(s):

Benjamin Brenner ◽

Markus Pihusch ◽

Andrea Bacigalupo ◽

Jeff Szer ◽

Mario von Depka Prondzinski ◽

...

Keyword(s):

Adverse Events ◽

Initial Dose ◽

Factor Vii ◽

Bleeding Complications ◽

Controlled Study ◽

Severe Bleeding ◽

Trial Period ◽

Hematopoietic Stem ◽

Coagulation Parameters ◽

Transfusion Requirements

Abstract HSCT is a common treatment of hematological or oncological disorders. Many HSCT-associated complications, including prolonged aplasia, infection and graft-versus-host disease (GVHD) predispose to bleeding, resulting in increased morbidity and mortality. Platelet concentrates, fresh frozen plasma, antifibrinolytic agents or prothrombin complex concentrates are often ineffective. Although licensed for use in hemophilia pts with inhibitors, rFVIIa has been reported to be effective for the treatment of bleeding related to thrombocytopenia, thrombasthenia and other coagulation disorders. We conducted a multicenter, randomized, double-blind, placebo-controlled trial to evaluate the efficacy and safety of rFVIIa in treatment of bleeding from days 2 to 180 after HSCT (F7BMT-1360 trial). Patients received rFVIIa (40, 80, 160 μg/kg) or placebo every 6h for 36h and were followed up for 96h after the initial dose. Exclusion criteria were recent thromboembolic events, atherosclerotic disease, DIC, thrombotic microangiopathy, venoocclusive disease and active AML (M3, M4, M5). The primary efficacy endpoint was the change in bleeding from 0h to 38h after initial dose. Secondary endpoints were transfusion requirements, change of coagulation parameters, and adverse events (AEs). One hundred patients (64 m; 36 f; 97 allogeneic; 3 autologous; 67 PBSC; 30 bone marrow; 2 cord blood; 1 unknown) with moderate or severe bleeding were included (38 lower GI; 26 hemorrhagic cystitis; 14 upper GI; 7 pulmonary; 1 intracerebral; 14 other). 80 μg/kg rFVIIa was effective, however no significant reduction in bleeding was seen at 160 μg/kg. There were no differences in transfusion requirements and changes in coagulation parameters across dose groups. Thirteen serious adverse events (SAEs) were reported within the trial period; there was no apparent drug or dose-related trend in the type or number of SAEs. One thromboembolic SAE (catheter thrombosis, 160 μg/kg) was observed within the trial period (2 days after last dosing) and two thromboembolic SAEs (cerebral infarction, 80 μg/kg; myocardial infarction, 40 μg/kg) were reported 4 and 14 days after last dosing. In this first controlled study on the use of rFVIIa after HSCT we found a significant effect of 80 μg/kg rFVII versus the standard hemostatic treatment. The diversity of the hemostatic disturbances and the heterogeneity of the patient population may have contributed to the lack of an increasing effect with increased dose. No safety issues were identified. Further trials with higher numbers of patients should focus upon optimizing the dose regimen of rFVIIa and on severe bleeding complications Bleeding status 38h after initial dose Treatment Gp N Stopped Decreased Unchanged/worse Cumulative odds ratio 97.1% CI p All data based on ITT. p<0.029 considered sig. Overall treatment effect: p=0.003. *Status unknown for 2 pts Placebo 22 5 (23%) 8 (36%) 9 (41%) 1.00 - - 40μg/kg 20 6 (30%) 4 (20%) 10 (50%) 0.94 [0.24; 3.64] 0.923 80μg/kg 26 14 (54%) 7 (27%) 5 (19%) 4.20 [1.05; 16.84] 0.021 160μg/kg 30 4 (13%) 9 (30%) 17 (57%) 0.54 [0.16; 1.83] 0.269 Total 98* 29 (30%) 28 (29%) 41 (42%) - - -

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The Administration of Fondaparinux in Strongly Suspected Heparin-Induced Thrombocytopenia (HIT): Further Evidence of Its Safety and Efficacy, and Need for a Controlled Randomized Study.

Blood ◽

10.1182/blood.v114.22.1072.1072 ◽

2009 ◽

Vol 114 (22) ◽

pp. 1072-1072

Author(s):

Franco Piovella ◽

Stefano Barco ◽

Marisa Barone ◽

Chiara Beltrametti ◽

Mara De Amici

Keyword(s):

Unfractionated Heparin ◽

Bleeding Complications ◽

Controlled Study ◽

Severe Bleeding ◽

Minor Bleeding ◽

Heparin Induced Thrombocytopenia ◽

Safety And Efficacy ◽

Coronary Syndrome ◽

Platelet Number ◽

The Mean

Abstract Abstract 1072 Poster Board I-94 Introduction: Heparin-induced thrombocytopenia (HIT), alone or associated with thrombosis (HITT), may develop during anticoagulant treatment with unfractionated heparin (UFH) or low-molecular weight heparin (LWMH). Fondaparinux is a selective inhibitor of coagulation factor Xa which apparently does not react with anti-PF4/heparin antibodies in in vitro testing. Methods: From january 2005 to december 2007 we treated 44 patients who had strong suspect of HIT (12 patients) or HITT (32 patients, of whom 12 had both DVT and PE). Of these, 30 patients were previously exposed to unfractionated heparin (UFH) for major cardiac surgery. The remaining patients were admitted to medical or general surgery wards. In the 32 patients who developed HITT, we applied therapeutic dosages of fondaparinux, i.e. 7.5 mg QD or lower, in accordance with their bleeding risk. The remaining patients were treated with prophylactic dosages of fondaparinux, i.e. 2.5 mg QD. Switch to warfarin was performed as soon as possible. The mean of our patients 4T's score was 6.2, corresponding to high risk patients; the mean of anticorpal optical density (GTI Enhanced®) was 1.4; the mean platelet number was 45 × 109/L. Results: All patients but four showed sustained normalization of the platelet number. All patients but four showed a significant reduction of their thromboembolic burden. No death related to severe bleeding was recorded. Two episodes of major bleeding were recorded in post-surgical patients (4,5%); 4 episodes of minor bleeding were recorded (9,1%). Four patients underwent dialysis during fondaparinux without bleeding complications. One patient developed acute coronary syndrome during treatment with fondaparinux. Nine patients did not survive (20,5%), with a key role of primitive diseases (i.e. sepsis) causing delay in the diagnostic process of HIT (four of them had a diagnosis of HIT with a mean delay of 7 days). In 16 patients submitted to therapeutic dosages of fondaparinux, anti-PF4/heparin antibody titers were followed over time showing a constant decrease: in one case antibody levels did not decrease up to 6 months after stopping both heparin and fondaparinux. Thirty patients were easily switched from fondaparinux to VKAs without problems in reaching the appropriate INR target. Conclusions: This report provides further evidence supporting the safety and efficacy of fondaparinux in the treatment of HIT and underlines the importance of an early diagnosis. However, it shows the need of a randomized controlled study between fondaparinux and a registered comparator drug. Disclosures: Piovella: GlaxoSmithKline: Honoraria, Speakers Bureau; Bayer: Honoraria, Speakers Bureau; Boehringer Ingelheim: Honoraria, Speakers Bureau. Off Label Use: fondaparinux in the treatment of heparin-induced thrombocytopenia.

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Successful Management of Refractory Severe Thrombocytopenia Associated with Antiphospholipid Syndrome Using rituximab

Blood ◽

10.1182/blood.v112.11.4570.4570 ◽

2008 ◽

Vol 112 (11) ◽

pp. 4570-4570

Author(s):

Soad Al Jaouni

Keyword(s):

B Cell ◽

Antiphospholipid Syndrome ◽

Pulse Therapy ◽

Bleeding Complications ◽

High Dose ◽

Severe Bleeding ◽

Severe Thrombocytopenia ◽

Promising Alternative ◽

Systemic Treatments

Abstract Background: Rituximab, a monoclonal antibody against the pan B-cell antigen CD20, that induces a rapid in vivo depletions of normal B lymphocytes. Although this agent was originally developed for use in patients with B-cell-NHL, its use has been successfully extended to many autoimmune disorders. Thrombocytopenia associated with antiphospholipid syndrome can be mild to severe. Severe cases can be associated with significant morbidity and mortality if refractory to the usual therapy for autoimmune thrombocytopenia. Aim: To report an effective regimen in treating a case of severe bleeding thrombocytopenia associated with antiphospholipid syndrome. Patient and Methods: A 49 year old female was diagnosed with thrombocytopenia associated with antiphospholipid syndrome and antibodies to double-stranded DNA, controlled over the last 15 years. Unfortunately, over the last two years the patient has had frequent admissions for uncontrolled nose bleed, soft palate petechiae and generalized bruising. Many treatments have been tried for this case of difficult refractory bleeding with platelets in the range 2.0–6.0 K/uL. These include systemic treatments such as corticosteroids, high pulse therapy of methylprednisolone, Imuran, i.v. immunoglobulin infusion and anti Rh-D intravenous therapy. We report an adult female patient with severe refractory bleeding thrombocytopenia associated with antiphospholipid syndrome and successful treatment with Rituximab. Her platelets count have been maintained above 200.0K/uL over a 14 months period. Unfortunately, this patient has had side effects of steroid therapy. In conclusion: Rituximab may be effective treatment on patients with refractory, severe thrombocytopenia associated with antiphospholipid syndrome and significant bleeding complications. Rituximab is a promising alternative option for the eradication of the autoantibodies and restoration of normal hemostasis while avoiding the use of high-dose steroid in refractory bleeding thrombocytopenia.

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Mycophenolate Mofetil Therapy Is a Steroid Sparing In Childhood and Young Adult Chronic ITP and Evans Syndrome.

Blood ◽

10.1182/blood.v116.21.1441.1441 ◽

2010 ◽

Vol 116 (21) ◽

pp. 1441-1441

Author(s):

Zaher Naji ◽

Madhvi Rajpurkar ◽

Sureyya Savasan ◽

Roland Chu ◽

Meera Chitlur ◽

...

Keyword(s):

Mycophenolate Mofetil ◽

Side Effects ◽

Bleeding Complications ◽

Severe Bleeding ◽

Evans Syndrome ◽

Corticosteroid Administration ◽

Chronic Itp ◽

Life Threatening ◽

The Mean ◽

Steroid Sparing

Abstract Abstract 1441 Chronic ITP and Evans syndrome are diseases characterized by autoantibody formation with resultant destruction of platelets or both platelets and red blood cells on neutrophils, respectively. Affected patients are at risk of life threatening bleeding complications and/or life threatening anemia. Conventional therapies are often ineffective or transiently effective and have significant toxicity. One of the most commonly used therapeutic strategies employs chronic corticosteroid administration with the attendant weight gain, hypertension, hyperglycemia, bone loss, infection risk, mood changes and protean other undesirable side effects. Mycophenolate Mofetil (MMF) is an immunosuppressive agent with a favorable side effect profile. It is converted to the active metabolite, mycophenolic acid, and interferes with purine metabolism in T-lymphocytes, effectively killing many of these cells and down-regulating autoimmune phenomena. With approval of our local IRB /HIC we retrospectively reviewed the charts of 11 chronic ITP/Evans syndrome patients who had received MMF, all such patients treated at a large urban Children's Hospital. Clinical variables included age, sex, duration of disease, steroid use, IVIG use, Anti-D use, platelet counts, hemoglobin concentrations and reticulocyte percentages. These data were analyzed using paired t-tests, one-sample t-test and descriptive statistics. The 11 patients ranged in age from 9–22 years old, with a mean age of 15 years. The mean time from diagnosis of disease was 41.8 months with a range of 6–95 months. There were 5 female subjects, 6 Evans syndrome patients and 5 with chronic ITP alone. The median platelet count over the 6 months prior to MMF was 70×109/L, (18-223), with a median of 90×109/L with (27-145) during the first 6 months of MMF therapy (p=0.4). In the Evans syndrome group, the mean hemoglobin prior to MMF was 10.9 g/dL, (9.1-14.6), with a mean of 12.1 g/dL, (6.9-16.7) on MMF (p=0.54). Similarly, the mean reticulocyte percentage was 2.7%, (0.5-14) prior to MMF, with a mean of 2.3%, (0.3-8.9) during MMF therapy. The mean total dose of steroids used in the 6 months prior to MMF was 84.2 mg/kg (prednisone equivalent), (11.2-170), compared to 62.2 mg/kg, (0-193.8) on MMF. 9 of 11 patients had reductions in steroid requirement by an average of 49.3% (p=0.0013). During the first 6 months of MMF both IVIG and anti-D usage decreased from total doses for the entire group of 17 and 3 doses to 8 and 1 dose, respectively. None of the patients experienced severe bleeding episodes or side effects more serious than a transient rash while on MMF. These data suggest that MMF may have a role as a steroid sparing therapy in the treatment of chronic ITP and Evans syndrome. Disclosures: Off Label Use: mycophenolate mofetil for ITP Evans syndrome.

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Efficacy and Safety of VTE Prophylaxis with Oral Rivaroxaban Compared to Fondaparinux or Low-Molecular Weight Heparin In a Large Cohort of Consecutive Patients Undergoing Major Orthopaedic Surgery

Blood ◽

10.1182/blood.v118.21.210.210 ◽

2011 ◽

Vol 118 (21) ◽

pp. 210-210

Author(s):

Jan Beyer-Westendorf ◽

Lars Donath ◽

Jörg Lützner ◽

Sebastian Werth ◽

Oliver Radke ◽

...

Keyword(s):

Orthopaedic Surgery ◽

Low Molecular Weight Heparin ◽

Phase Iii ◽

Bleeding Complications ◽

Low Molecular Weight ◽

Severe Bleeding ◽

Efficacy And Safety ◽

Vte Prophylaxis ◽

Superior Efficacy ◽

Major Orthopaedic Surgery

Abstract Abstract 210 Efficacy and safety of VTE prophylaxis with oral rivaroxaban compared to fondaparinux or low-molecular weight heparin in a large cohort of consecutive patients undergoing major orthopaedic surgery. Aim: Patients undergoing major orthopaedic surgery (MOS) have a high risk of postoperative venous thromboembolism (VTE). Several types of pharmacological thromboprophylaxis are approved for this indication. In phase III VTE prevention trials in MOS, the new oral facor Xa inhibitor rivaroxaban proved superior efficacy over LMWH in preventing VTE without increasing bleeding complications. However, study populations consist of selected patients screened for strict exclusion criteria before randomisation. Little is known about efficacy and safety of rivaroxaban prophylaxis in large unselected cohorts of patients undergoing MOS in daily practice. Method: We retrospectively evaluated 5346 consecutive patients undergoing MOS at our centre between January 2005 and June 2011 for the rate of VTE events, bleeding complications and surgical revisions by review of patient charts, complication and transfusion databases and autopsy reports. All events were analyzed according to the type of thromboprophylaxis used. Results: Of the 5346 patients, 1055 patients received thromboprophylaxis with rivaroxaban (R; hospital standard since 2010), 1683 patients received LMWH (hospital standard 2005–2007), 2069 received fondaparinux (F; hospital standard 2007–2009) and 539 patients received other prophylaxis. Symptomatic VTE event rates for patients receiving R, F or LMWH were 2.5%, 5.5% and 3.9%, respectively (table 1). R was significantly more effective to prevent symptomatic VTE compared to F or LMWH, mostly due to significantly lower rates of distal VTE. Overall, the safety of VTE prophylaxis with R was superior over F or LMWH with significantly lower rates of surgical revisions (1.1%, 1.8% and 4.7%, respectively) and lower rates of severe bleeding complications (7.4%, 11.1% and 14.9%, respectively, which also was statistically significant. Conclusion: VTE prophylaxis with rivaroxaban is superior over prophylaxis with fondaparinux or LMWH with regard to the prevention of symptomatic VTE complications. Furthermore, rivaroxaban prophylaxis was also safer with regard to severe bleeding complications and surgical complications compared to fondaparinux or LMWH, which is in contrast to the results of large phase III trials and the current opinion, that superior efficacy of prophylaxis has the downside of higher bleeding complications. We conclude that real world patients undergoing MOS are different from study populations and may especially benefit from rivaroxaban prophylaxis with regard to both efficacy and safety. Disclosures: Beyer-Westendorf: Bayer Healthcare: Research Funding, Speakers Bureau.

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Menorrhagia in Women with Severe Bleeding Disorders

Blood ◽

10.1182/blood.v120.21.4626.4626 ◽

2012 ◽

Vol 120 (21) ◽

pp. 4626-4626

Author(s):

Susan Halimeh ◽

Joanna Davies ◽

Debra Pollard ◽

Rezan Abdul-Kadir

Keyword(s):

Tranexamic Acid ◽

Surgical Intervention ◽

Postoperative Bleeding ◽

Bleeding Complications ◽

Severe Bleeding ◽

Bleeding Disorders ◽

Line Treatment ◽

First Line ◽

First Line Treatment ◽

Factor Concentration

Abstract Abstract 4626 The management of menorrhagia presents a challenge in women with severe bleeding disorders. Conservative medical management is the first line treatment and most women with severe bleeding disorder require combination treatment. Surgical intervention may ultimately be offered to women in whom medical management has failed and whom no longer desire fertility. Women with low factor levels are at risk of perioperative bleeding complications and may require haemostatic support. A total of 50 women with severe factor deficiencies (less than 20iu/dL) were included in this study. 46 women were registered at the Haemophilia Centre at the Royal Free Hospital in London. Four cases were also included from the Rhine-Ruhr Haemophilia Centre in Duisburg, Germany. We reviewed the occurrence of menorrhagia and the management options that were offered. In those that required surgical intervention, the incidence of postoperative bleeding complications and the requirement for factor concentration was also reviewed. The bleeding disorders in these women were 34 (68%) with severe factor XI deficiency, 10 (20%) with severe type 1 and type 3 von Willebrand's disease, 4 (8%) with factor VII deficiency, 2 (4%) had factor V or X deficiencies and one (2%) had a combination of factor VI and VIII deficiency. The ISTH/SSC joint working group bleeding assessment tool was used to assess the severity and frequency of bleeding symptoms among this cohort of women. The bleeding scores ranged from −2 to 30 with a median score of 9.5. In total, 32 out of 50 (64%) women with severe factor deficiency required medical attention for menorrhagia. Medical treatment included hormonal preparations (combined oral contraceptive pill or levonorgestrel intrauterine device), which was used as a first line treatment in 15 out of 32 (46.8%) women. Haemostatic treatment included antifibrinolytic medication such as tranexamic acid, which was used in combination with hormonal therapy. One women required intranasal DDAVP, von Willebrand factor concentrate and tranexamic acid. Failure to control menstrual bleeding occurred in 14 (43.7%) women and surgical intervention was required. 7 out of 14 (50%) women required hysterectomy and the remaining 7 women underwent endometrial ablation. Prophylaxis with factor concentration to cover surgical intervention was given in 8 out of 14 women (64.2%). The remainder received tranexamic acid for 24–48 hours following surgery. Postoperative bleeding occurred in 7 women that had surgical intervention, despite two women receiving prophylaxis. This study highlights the complexity involved in the management of menorrhagia in women with severe bleeding disorders and the high risk of postoperative bleeding. Disclosures: No relevant conflicts of interest to declare.

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