Coagulopathies

2020 ◽  
Author(s):  
Michael Levine
Keyword(s):  
Key Words ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Coagulation Cascade ◽  
Bleeding Disorders ◽  
Platelet Disorders ◽  
Von Willebrand

Coagulopathy can be caused by numerous hereditary or acquired etiologies. Although some of these conditions are known and the patient is aware of the bleeding disorder, other bleeding disorders are diagnosed only after the onset of excessive hemorrhage. This review discusses both hereditary and acquired disorders of coagulopathy. Platelet disorders are discussed elsewhere. This review contains 2 figures, 7 tables, and 72 references. Key words: Coagulopathies; Coagulopathy; Bleeding disorder; Hereditary bleeding disorder; Acquired bleeding disorder; von Willebrand disease; Hemophilia; Coagulation cascade; Hemorrhage; Anticoagulant-associated hemorrhage

scholarly journals Von Willebrand Disease Screening in Women Undergoing Hysterectomy for Heavy Menstrual Bleeding

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 674-674
Author(s):  
Amanda E. Jacobson ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. O'Brien
Keyword(s):  
Surrogate Marker ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Heavy Menstrual Bleeding ◽  
Menstrual Bleeding ◽  
Common Symptom ◽  
Metropolitan Statistical Area ◽  
Bleeding Disorders ◽  
Nationally Representative ◽  
Von Willebrand

Abstract Background Bleeding disorders in women are under-recognized and under-treated. Women are equally as likely as men to have bleeding disorders other than hemophilia and are disproportionately affected by these diseases due to the bleeding challenges of menstruation and childbirth. The most common bleeding disorder identified in women is von Willebrand disease (VWD). Heavy menstrual bleeding (HMB) is the most common symptom in women with VWD, occurring in up to 93% of patients. Among women with HMB, the reported prevalence of VWD ranges from 5─20%. Women with VWD are also more likely to be diagnosed with hemorrhagic ovarian cysts due to ovulation-associated bleeding and endometriosis due to increased retrograde menstruation. As a result, women with bleeding disorders are more likely to undergo hysterectomy and also undergo hysterectomy at an earlier age than women without bleeding disorders. In 2001, the American College of Obstetrics and Gynecology (ACOG) recommended VWD screening prior to hysterectomy in women with HMB. The actual frequency of VWD screening in clinical practice is unknown. Objectives In this study, we assess patterns of VWD screening in a nationally representative sample of women undergoing hysterectomies for HMB. Methods We used the Truven Health MarketScan® Research Databases which include the medical prescription claims of over 109 million covered lives as well as Medicaid data on 8.6 million patients from 14 states. The MarketScan Databases contain patient demographics, physician and facility claims and pharmacy claims. Procedure codes were used to identify women ages 10-44 years undergoing hysterectomy or hysterectomy alternative (HA) from 2011-2013. Subjects were required to have 12 months of continuous enrollment prior to surgery date. We utilized ICD-9 codes to categorize hysterectomy indications and only included women with a diagnosis of excessive bleeding as the indication for surgery. Women with fibroids, genital tract malignancy, and previously diagnosed bleeding disorders were excluded. We defined VWD screening as a laboratory claim for either VWF:Antigen and/or VWF:Activity within the 12 months preceding hysterectomy. To determine if patient and facility level characteristics impacted access to specialty hematology care and/or screening for VWD, we collected the following information: 1) known bleeding disorder diagnosis and/or endometriosis prior to surgery; 2) age; 3) whether patient was living in metropolitan statistical area (MSA; used as a surrogate marker for urban vs rural inhabitance); 4) number of miles and approximate travel time to nearest Hemophilia Treatment Center (HTC). We used ArcMAP® software to calculate distance between the MSA and nearest HTC. MSA data was only available for commercially-insured patients. (Figure 1) Logistic regression was used to assess factors related to the occurrence of VWD screening. Results We identified 13,790 women who underwent hysterectomy/HA for HMB. We excluded 138 with known bleeding disorders leaving 13,652 women in our final analysis (Table 1). Of these, 74 (0.5%) were screened for VWD within 12 months preceding surgery. There were 2,000 women (15%) who underwent other coagulation tests, most commonly prothrombin time and partial thromboplastin time. We had MSA data on 11,557 commercially-insured women, of whom 72.4% lived within a MSA. Women living in a MSA were screened more often than those outside of a MSA (p=0.013). For those living within a MSA, the odds of being screened for VWD was lower in women with endometriosis (OR=0.54, 95% CI 0.31, 0.97; p=0.038) and women living >100 miles from the nearest HTC (OR=0.29, 95% CI 0.11, 0.81; p=0.017). Discussion This study demonstrated that despite ACOG expert recommendations, the frequency of VWD screening in a nationally-representative population of publically and commercially-insured women undergoing hysterectomy for HMB was very low. Greater distance from a HTC or a prior diagnosis of endometriosis further reduced the likelihood of VWD screening. It is important to increase awareness that a diagnosis of endometriosis does not rule out the presence of a bleeding disorder. This study brings to light the need for the hematology community to improve education and awareness among women's health providers in order to identify women with bleeding disorders and allow for optimal medical management of HMB prior to surgical consideration. Disclosures No relevant conflicts of interest to declare.

scholarly journals Puberty menorrhagia Requiring Inpatient Admission

2010 ◽  
Vol 49 (178) ◽  
Author(s):  
AH Khosla ◽  
L Devi ◽  
P Goel ◽  
PK Saha
Keyword(s):  
Blood Transfusion ◽  
Medical Condition ◽  
Severe Anaemia ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Age Group ◽  
Idiopathic Thrombocytopenia Purpura ◽  
Von Willebrand ◽  
Significant Health

INTRODUCTION: Puberty menorrhagia is a significant health problem in adolescent age group and severe cases may require admission and blood transfusion. Aim of this study was to evaluate the causes, associated complications and management of puberty menorrhagia. METHODS: Hospital records of all patients of puberty menorrhagia requiring admission were analyzed for etiology, duration since menarche, duration of bleeding, investigation profile and management. RESULTS: There were 18 patients of puberty menorrhagia requiring hospital admission. Etiology was anovulatory bleeding in 11 patients, bleeding disorders in five which included idiopathic thrombocytopenia purpura in three and one each with Von-Willebrand disease and leukemia. Two patients had hypothyroidism as the cause. Fourteen patients presented with severe anaemia and required blood transfusion. All except one responded to oral hormonal therapy. CONCLUSIONS: Puberty menorrhagia can be associated with severe complications and requiring blood transfusion. Although most common cause is anovulation but bleeding disorder, other medical condition and other organic causes must be ruled out in any patient of Puberty menorrhagia.KEYWORDS: anovulation, bleeding disorder, puberty, menorrhagia, anaemia.

scholarly journals Do We Need To Evaluate Patients With Spontaneous Subconjunctival Hemorrhage For Bleeding Disorders?

2021 ◽  
Author(s):  
Selim Sayın ◽  
Arif Yener
Keyword(s):  
Fibrinogen Level ◽  
Normal Population ◽  
Young Patients ◽  
The Elderly ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Etiologic Factor ◽  
Type I ◽  
Bleeding Disorders ◽  
Von Willebrand

Aim of the study: Subconjunctival hemorrage (SCH) is a frequent bleeding manifestation and a common cause of visits to the primary care. Trauma in young patients and vascular damage such as hypertension in the elderly are the most common causes of SCH and the prevalence of hematological diseases is less than 1%. We aimed to evaluate the prevalence of congenital or acquired bleeding disorders in patients with once or recurrent SCH. Methods used to conduct the study: It is a retrospective study and included fifty-two patients with SCH whose etiologic factor was not detected. Hemostatic tests were studied in 52 patients (25 male and 27 females) with. All patients included were evaluated for congenital or acquired bleeding disorder and SCH with once and those with 2 or more were compared for the laboratory results. Results of the study: One patient with once and one patient with recurrent SCH (3.8%) were diagnosed a type I von Willebrand disease (vWD). The prevalence of patients with type 1 vWD in the study was not statistically significant when compared with the frequency of von Willebrand favctor (vWF) deficiency in the normal population. Fibrinogen level was found to be statistically higher in patients who had SCH once than those who had recurrent SCH. But fibrinogen level was in normal range in all patients. Conclusions drawn from the study and clinical implications: There was no increase in the incidence of congenital or acquired bleeding disorder in SCH patients compared to normal population. Therefore, it was considered that spontaneous SCH patients do not need to be evaluated for bleeding disorder.

Is a Pbac-Score a Good Tool to Quantify Menorrhagia in Women with Von Willebrand Disease and Rare Bleeding Disorders?

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1133-1133
Author(s):  
Susan Halimeh ◽  
Hannelore Rott ◽  
Manuela Siebert ◽  
Guenther Kappert
Keyword(s):  
Blood Loss ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Factor Vii ◽  
Menstrual Blood ◽  
Cycle Period ◽  
Coagulation Disorder ◽  
Bleeding Disorders ◽  
Menstrual Blood Loss ◽  
Von Willebrand

Abstract Abstract 1133 Introduction: Von Willebrand disease (VWD) is the most common inherited bleeding disorder. VWD and other autosomal inherited bleeding disorders equally affect women and men. Menorrhagia or severe menstrual bleeding (HMB) is the most common symptom of women with bleeding disorders. HMB is defined as bleeding that lasts for more than seven days or as the loss of more than 80 mL of blood per menstrual cycle. The menstrual blood loss can be quantified by the use of a pictorial bleeding assessement chart (PBAC). Samples and methods: In 195 women with menorrhagia and in 45 controls menstrual blood loss was quantified using pictorial blood assesment charts (PBAC) and results were compared. Results: In 169 of 195 women (86%) a bleeding disorder could be detected. In those with a bleeding disorder, the distribution was as followed: 62% had a von Willebrand disease, 14,4% had a factor-VII-deficiency (F7D), 5% had a factor-XIII-deficiency (F13D) and the remaining 18,6% had other beedling disorders (e. g. hypofibrinogenaemia and other mild factor deficiencies). The median PBAC-Score of all patients was 268 (range: 10–4212). In our controi group of 45 women the median PBAC-Score was 46,5 (3- 137).ROC-Analysation shows that the PBAC (AUC=0.977) is much more useful than the number of bleeding days (AUC=0.855) in order to distingish controls from patients suffering from menorrhagia due to a coagulation disorder. We found that the best cutoff for the PBAC is 100 with an sensitifity of 88% and a specifity of 97%. Discussion: Attempts to measure the quantity of menstrual blood loss can be useful in clinic practice. One study found that variables predicting a blood loss higher than 80ml per menses were clots greater than one inch, low ferritin levels, or changing a pad or tampon more than hourly (flooding). A prospective method of quantifying menstrual blood loss includes the use of a pictorial bleeding assessement calendar (PBAC). We are of the opinion, that we would have detected more bleeding disorder also in the patients, where we did not find any diagnosis until now, if we would have controlled them more than one time during the cycle period. Conclusions: Women with hyermenorrhagia frequently suffer from a bleeding disorder, in 86% of our patients an abnormal coagulation was found. The PBAC-Score is an easy tool to quantify menstrual blood loss in women. In our study a PBAC-Score above 100 was suspicious of having a bleeding disorder. Disclosures: No relevant conflicts of interest to declare.

Menorrhagia and bleeding disorders in adolescent females

2012 ◽  
Vol 32 (01) ◽  
pp. 45-50 ◽  
Author(s):  
S. Halimeh
Keyword(s):  
Screening Tool ◽  
Treatment Options ◽  
Adolescent Females ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Menstrual Bleeding ◽  
Common Symptom ◽  
Bleeding Disorders ◽  
Factor Deficiency ◽  
Von Willebrand

SummaryIn women, von Willebrand disease (VWD) is the most common inherited bleeding disorder. Since VWD and other inherited bleeding disorders are autosomal disorders, they affect women and men. Menorrhagia, or heavy menstrual bleeding (HMB), is the most common symptom of women with bleeding disorder experience. Objectively, it is defined as bleeding that lasts for more than seven days or results in the loss of more than 80 ml of blood per menstrual cycle. The prevalence of menorrhagia in a woman with a bleeding disorder ranges from 32 to 100% in patients with VWD, from 5 to 98% in patients with a platelet dysfunction and from 35 to 70% in women with a rare factor deficiency. A detailed history and a careful physical exam are the first steps towards a diagnosis in adolescents, adding a PBAC > 100 increased the sensitivity of the screening tool further to 95%. Laboratory testing should be made at the time of menstrual bleeding in an effort to capture the lowest level of VWF : Ag and FVIII : C. Treatment options for menorrhagia in VWD: antifibrinolytic therapy with tranexamic acid, (2) the non-transfusional agent desmopressin (DDAVP), (3) purified blood products that contain factor VIII and VWF concentrated from plasma and (4) hormonal preparations.

scholarly journals Resolving Differential Diagnostic Problems in von Willebrand Disease, in Fibrinogen Disorders, in Prekallikrein Deficiency and in Hereditary Hemorrhagic Telangiectasia by Next-Generation Sequencing

Life ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 202
Author(s):  
Réka Gindele ◽  
Adrienne Kerényi ◽  
Judit Kállai ◽  
György Pfliegler ◽  
Ágota Schlammadinger ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Von Willebrand Disease ◽  
Differential Diagnostics ◽  
Bleeding Disorders ◽  
Next Generation ◽  
Illumina Platform ◽  
Clinical Patient ◽  
Von Willebrand ◽  
Generation Sequencing

Diagnosis of rare bleeding disorders is challenging and there are several differential diagnostics issues. Next-generation sequencing (NGS) is a useful tool to overcome these problems. The aim of this study was to demonstrate the usefulness of molecular genetic investigations by summarizing the diagnostic work on cases with certain bleeding disorders. Here we report only those, in whom NGS was indicated due to uncertainty of diagnosis or if genetic confirmation of initial diagnosis was required. Based on clinical and/or laboratory suspicion of von Willebrand disease (vWD, n = 63), hypo-or dysfibrinogenemia (n = 27), hereditary hemorrhagic telangiectasia (HHT, n = 10) and unexplained activated partial thromboplastin time (APTT) prolongation (n = 1), NGS using Illumina platform was performed. Gene panel covered 14 genes (ACVRL1, ENG, MADH4, GDF2, RASA1, F5, F8, FGA, FGB, FGG, KLKB1, ADAMTS13, GP1BA and VWF) selected on the basis of laboratory results. We identified forty-seven mutations, n = 29 (6 novel) in vWD, n = 4 mutations leading to hemophilia A, n = 10 (2 novel) in fibrinogen disorders, n = 2 novel mutations in HHT phenotype and two mutations (1 novel) leading to prekallikrein deficiency. By reporting well-characterized cases using standardized, advanced laboratory methods we add new pieces of data to the continuously developing “bleeding disorders databases”, which are excellent supports for clinical patient management.

Heavy Menstrual Bleeding in the Adolescent

2019 ◽  
Author(s):  
Kimberly Huhmann ◽  
Andrea Zuckerman
Keyword(s):  
Differential Diagnosis ◽  
Aminocaproic Acid ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Heavy Menstrual Bleeding ◽  
Menstrual Bleeding ◽  
Oral Contraceptive Pills ◽  
Sexually Transmitted ◽  
Contraceptive Pills ◽  
Von Willebrand

Heavy menstrual bleeding is a common presenting problem in the adolescent population. The average age of menarche is between 12 and 13 years. The most common reason for heavy menstrual bleeding soon after menarche is from an immature hypothalamic ovarian access, which spontaneously resolves once cycles become ovulatory. However, the broad differential diagnosis for heavy menses in adolescents includes coagulopathy, thyroid disease, sexually transmitted infections, specifically chlamydia, and chronic medical conditions. Von Willebrand disease is the most common bleeding disorder that can present with heavy menstrual bleeding at menarche or shortly after. A thorough history and physical exam with occasional labs needs to be completed and can assist in narrowing the differential diagnosis. Treatment of heavy menstrual bleeding consists of hormonal and nonhormonal options: combination oral contraceptive pills, patches, or rings taken continuously or cyclically; progesterone-only pills; progesterone implants; progesterone intrauterine devices; cyclic tranexamic acid; cyclic aminocaproic acid; and GnRH agonists with add-back therapy. This review contains 3 tables, and 28 references. Key Words: adolescent menses, anovulation, bleeding disorder, heavy menstrual bleeding, immature hypothalamic ovarian axis, menarche, treatment of heavy menses, Von Willebrand disease

Regulation der primären Hämostase durch von-Willebrand-Faktor und ADAMTS13

2011 ◽  
Vol 31 (04) ◽  
pp. 275-280 ◽  
Author(s):  
U. Budde ◽  
R. Schneppenheim
Keyword(s):  
Platelet Adhesion ◽  
Coagulation Factor ◽  
Von Willebrand Disease ◽  
Coagulation Cascade ◽  
Platelet Glycoprotein ◽  
Platelet Adhesion And Aggregation ◽  
Hydrodynamic Shear ◽  
Specific Protease ◽  
Multiple Domains ◽  
Von Willebrand

SummaryVon Willebrand factor (VWF) is an adhesive, multi-functional huge multimerized protein with multiple domains harboring binding sites for collagen, platelet glycoprotein receptors and coagulation factor VIII (FVIII). The functional domains enable VWF to bind to the injured vessel wall, to recruit platelets to the site of injury by adhesion and aggregation and to bind and protect FVIII, an important cofactor of the coagulation cascade. VWF function in primary haemostasis is located in particular in the arterial and micro-circulation. This environment is exposed to high shear forces with hydrodynamic shear rates ranging over several orders of magnitude from 10–1 to 105 s-1 and requires particular mechanisms to enable platelet adhesion and aggregation under these variable conditions. The respective VWF function is strictly correlating with its multimer size. Lack or reduction of large VWF multimers is seen in patients with von Willebrand disease (VWD) type 2A which correlates with reduction of both VWF:platelet GPIb-binding and VWF:collagen binding and a bleeding phenotype. To prevent unlimited platelet adhesion and aggregation which is the cause of the microangiopathic disorder thrombotic thrombocytopenic purpura (TTP), VWF function is regulated by its specific protease ADAMTS13. Whereas a particular susceptibility of VWF to ADAMTS13 proteolysis is the cause of a frequent VWD type 2A phenotype, lack or dysfunction of ADAMTS13, either acquired by ADAMTS13 antibodies or by inherited ADAMTS13 deficiency (Upshaw-Schulman Syndrome), causes TTP. Therefore VWD and TTP represent the opposite manifestations of VWF related disorders, tightly linked to each other.

scholarly journals Desmopressin: a nontransfusional form of treatment for congenital and acquired bleeding disorders [see comments]

Blood ◽  
1988 ◽  
Vol 72 (5) ◽  
pp. 1449-1455 ◽  
Author(s):  
PM Mannucci
Keyword(s):  
Arginine Vasopressin ◽  
Von Willebrand Disease ◽  
Synthetic Analogue ◽  
Bleeding Disorders ◽  
Spinal Fusion Surgery ◽  
Prolonged Bleeding Time ◽  
Normal Individuals ◽  
Transfusion Requirements ◽  
Coagulant Activity ◽  
Von Willebrand

Abstract Desmopressin (1-deamino-8-D-arginine vasopressin, abbreviated DDAVP) is a synthetic analogue of the antidiuretic hormone L-arginine vasopressin. Because it can raise circulating levels of factor VIII coagulant activity (FVIII) and von Willebrand factor and shorten the prolonged bleeding time, DDAVP is established as a nontransfusional form of treatment for mild and moderate hemophilia and von Willebrand disease. Recently, DDAVP has also been purported to be useful for shortening the prolonged skin bleeding times that occur with uremia, cirrhosis, and platelet dysfunctions of various etiologies. Finally, there is evidence from controlled clinical trials that DDAVP can reduce blood loss and transfusion requirements for hemostatically normal individuals undergoing spinal fusion surgery and for patients undergoing cardiopulmonary bypass surgery. The purpose of this report is to review the therapeutic applications of DDAVP in congenital and acquired bleeding disorders and to discuss areas in which further basic and clinical research is needed.

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