The Utility of Thromboelastography As a Screening Test for Bleeding Disorders

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4352-4352 ◽  
Author(s):  
Ayesha N Zia ◽  
Muhammad Fawad Bilal ◽  
Madhvi Rajpurkar ◽  
Meera B. Chitlur ◽  
Michael Callaghan ◽  
...  
Keyword(s):  
Clinical Evaluation ◽  
Screening Test ◽  
Laboratory Testing ◽  
Bleeding Disorder ◽  
Factor Vii ◽  
Factor Xii ◽  
Bleeding Disorders ◽  
Factor Xi Deficiency ◽  
Storage Pool ◽  
Storage Pool Disease

Abstract Abstract 4352 Introduction: Accurate assessment of bleeding disorders requires a thorough clinical evaluation and appropriate laboratory testing process. These patients comprise a substantial proportion of consultations carried out by hematologists. The limitations of each screening test, and the heterogeneity of these disorders continue to make the diagnosis cumbersome for the practicing clinician. Plasma clotting times, such as the prothrombin time and activated partial thromboplastin time are the most frequently used screening tests to assess adequacy of hemostasis. Thromboelastography (TEG) lends the ability to assess hemostasis globally while also assessing the effects of platelets, leucocytes and red cells on coagulation. Aims: To determine the utility of TEG as an effective screening test for bleeding disorders Methods: Medical records of patients referred to Hematology Service from August 2006 through July 2011 were retrospectively reviewed after institutional review board approval. Results: One hundred ninety-five patients (125 females, 70 males: ages 0.1 to 20 years) were evaluated for a bleeding disorder based on either bleeding symptoms or abnormal routine clotting tests and had TEG performed with low dose tissue factor (1:190 000 concentration). Most common symptoms were epistaxis (83/195), bruising (67/195), menorrhagia (48/195), surgical bleeding (16/195). Twenty-nine patients were diagnosed with a bleeding disorder based on clinical evaluation and laboratory testing. Type 1 vonWillebrand disease (vWD) was diagnosed in 16 patients, heterozygous factor VII deficiency in 6 patients, factor XII deficiency in 3 patients, factor XI deficiency in 2 patients and platelet delta storage pool disease in 1 patient. One patient was a symptomatic hemophilia carrier. Preliminary analysis revealed that among the vWD patients, only 1 patient demonstrated abnormalities in all parameters of TEG [Prolonged Reaction time (R Time), k time (rate of clot formation) and decreased Maximum amplitude (MA)] and 2 showed prolonged R and k time without concomitant decrease in MA. In patients with heterozygous factor VII deficiency, only 2 of 4 patients showed prolonged R and k times. The symptomatic hemophilia carrier, 1 of 3 with factor XII and 1 of 2 patients with factor XI deficiency had prolonged R as their sole abnormality. TEG was completely normal in the patient with platelet delta storage pool disease. The sensitivity of the R time to diagnose a clotting factor (including low factor VIII with vWD) deficiency was only 58% with a specificity of 78%. R time correlated with PTT and PT in up to 50% (vWD: 12%, FVII deficiency: 33%, FXII: 33%, FXI: 50%) of the patients. R time was also prolonged in 46/166 (28%) patients without a definitive bleeding disorder, however 10 of these patients had a lupus anticoagulant. Conclusion: In our study of 195 patients referred for evaluation of bleeding symptoms or abnormal coagulation tests, TEG was of limited value in identifying congenital coagulation defects with both poor sensitivity and specificity. Future studies could examine different agonists or conditions for TEG that may improve its sensitivity for detection of congenital bleeding disorders. Disclosures: No relevant conflicts of interest to declare.

Frequency of Bleeding Disorders in Women Presenting Menorrhagia in the North of Iran

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4660-4660
Author(s):  
Ghasem Janbabaei ◽  
Samaneh Borhani ◽  
Masoumeh Rashidi ◽  
Touraj Farazmandfar ◽  
Ramin Shekarriz ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Bleeding Disorder ◽  
Reproductive Age ◽  
Factor Vii ◽  
Factor Xii ◽  
Factor V ◽  
Bleeding Disorders ◽  
The North ◽  
Platelet Aggregometry ◽  
North Of Iran

Abstract Abstract 4660 Objectives: Menorrhagia is a common presentation of bleeding disorders, especially VWD in women. We decided to determine the frequency of these disorders in women with menstruation problems. Materials and Methods: 208 patients in reproductive age with menorrhagia were investigated for bleeding disorders in two steps. Step one includes CBC, PT, PTT and BT, which performed for all patients, and for patients who had an abnormality in step one, step two was considered. Step two includes VWAg, RCO, factors level, RIPA and platelet aggregometry. Results: Among 208 patients who investigated for bleeding disorders 53 patients (25%) had abnormalities in coagulation tests or platelet count. In our survey frequencies of bleeding disorder was VWD=14(6.73%), thrombocytopenia=13(6.25%), deficiency of factor II= 2(0.96%), factor V=1(0.48%), factor VII=3(1.44%), factor VIII=2(0.96%), Factor XI =4(1.92%), factor XII=4(1.92%), Bernard Soulier=2(0.96%). Furthermore, we found 18 patients (8.65%) who had abnormal PT, PTT or BT with no definite diagnosis. Conclusion: In this study, the most common bleeding disorder was VWD and thrombocytopenia ranked second disorders. Although other bleeding disorders are rare, in our study a number of them were found. So, we recomment the above coagulation test for women with menorrhagia. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Are Bleeding Scores Predicting Severity and Outcome in Hemophilia and Rare Bleeding Disorders?

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4801-4801
Author(s):  
Mahdi Shahriari ◽  
Mehran Karimi
Keyword(s):  
Hemophilia A ◽  
Conflicts Of Interest ◽  
Bleeding Disorder ◽  
Factor Vii ◽  
Control Group ◽  
Severe Bleeding ◽  
Bleeding Disorders ◽  
Factor Xi Deficiency ◽  
Factor Vii Deficiency ◽  
Plasma Factor

Abstract Background: Hemophilia is an inherited severe bleeding disorder, Factors VIII and IX deficiencies have X linked inheritance. Most rare bleeding disorders (RBD) have autosomal recessive inheritance. The ISTH-BAT is a valuable research tool that is applicable to clinical practice and scores patients' bleeding symptoms from 0 to 4. ISTH-BAT has the potential to avoid unwanted laboratory testing, predict the risk of bleeding, describe symptom severity and inform treatment. Aims: To assess the utility of ISTH-BAT in our bleeding disorder population, compare the bleeding score (BS) in adult and pediatric groups and investigate its association with plasma factor levels.Von Wilebrand disease and thrombasthenia was excluded from this study. One hundred cases of epistaxis whose coagulation workups were within normal limits were enrolled in the control group. Methods: This is an observational analytical study. The ISTH-BAT was used to calculate bleeding scores (BS) in a group of hemophilic patients and healthy controls. Ethics approval and informed consents were secured prior to the study. Results: A total of 100 patients, (38 hemophilia A; FVIII deficiency,22hemophilia B; FIX deficiency, 10 hemophilia C; factor XI deficiency, 10 factor VII deficiency, 8 factor X deficiency, 6 factor V deficiency, 5 Afibrinogenemia, Factor I deficiency) and 100 controls were analyzed using the ISTH BAT. Mean BAT score in hemophilia A, B and C were 12.8±8.2; 11.6±8.6; and 7.5 ±5.52respectively.While in RBD were and 7.97±5.56. BAT score in 30 controls was 5.35±4.48. Bleeding Scores were significantly higher in hemophilia A and B patients as compared to controls (P < 0.05) but it was not significant when BAT Scores of hemophilia C patients compared with controls (P=0.35). ANOVA test revealed BAT Score were significantly different among the mild, moderate and severe hemophilia A and B in both adult and pediatric patients but there was no difference in Hemophilia C patients. BAT scores were linked to hematomas; minor wound bleeding in hemophilia A, B patients in the pediatric group while more linked with epistaxis bleeding after dental extraction and surgical interventions in the adult group. Mean BAT Scores of rare bleeding disorders was significantly higher than control group (10.97± 5.65; compared with 5.35±4.48; P<0.05). BAT Score of 10 cases of factor VII deficiency and 5 cases of Afibrinogenemia were higher than other case of RBD, and control group (14.35 ± 7.87; and 13.54±5.56 compared with 5.35±4.48; P< 0.001 and P<0.05respectively). Conclusions: Our data revealed that the ISTH-BAT can help diagnose the bleeding condition in hemophilia and RBD patients and can be considered a predictor for the bleeding risk or severity. This will decrease need of subspecialty coagulation tests; it ultimately improvesdecision making and the clinical management of patients. Disclosures No relevant conflicts of interest to declare.

Rare Congenital Bleeding Disorders from a Tertiary Haematology Referral Center in Western India

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4519-4519
Author(s):  
Anjali Jaydeep Kelkar ◽  
Shashikant Apte ◽  
Varsha Melinkeri ◽  
Samir Melinkeri ◽  
Ashay Karpe ◽  
...  
Keyword(s):  
Factor Xiii ◽  
Bleeding Time ◽  
Factor Vii ◽  
Western India ◽  
Factor Xii ◽  
Bleeding Disorders ◽  
Factor Xi Deficiency ◽  
Consanguineous Marriages ◽  
Number Of Patients ◽  
History Of

Abstract Introduction: Bleeding disorders are present in varying frequency in a given population. We present our data of rare bleeding disorders (excluding Haemophilia & von Willebrand’d disease) since little data about the same is available from developing countries. Many of the rare bleeding disorders are associated with a strong history of consanguinity, the frequency of consanguineous marriages are between 12 –42 %. Materials and Methods: These cases were part of the referral for evaluation of congenital bleeding disorders; some of the cases were diagnosed as part of pre-operative coagulation screen. All the cases were subject to a detailed history encompassing the timing site and frequency of bleed, history of consanguinity, umbilical cord bleed. Lab. evaluation included bleeding time (Ivy’s method), clotting time., in vivo adhesion and clot retraction. Prothrombin and activated partial thromboplastin time with correction studies as and when required, Factor XIII screen with urea solubility test. Complete blood count was done prior to bleeding time and platelet adequacy was confirmed. All blood samples used for coagulation assays were collected in sterile citrate vaccutainers. Further investigations were performed including factor assays depending upon the results of the basic profile. Results: Feb 2000 till August 2008. Total No. of cases studies I from Year 2000 970 Abnormal cases 75 Consanguinity 25 Disorder No. Of Cases Factor V Deficiency 5 Factor VII Deficiency 4 Factor X Deficiency 4 Factor XI Deficiency 1 Factor XII Deficiency 6 Factor XIII Deficiency 8 Combined factor deficiency 4 Factor I deficiency 11 Dysfibrinogenemia 6 Factor II deficiency 1 Glanzmann’s thrombasthenemia 25 Bernard soullier’s syndrome 2 Conclusion: In our institution which caters to a large population in and around city of Pune, Maharashtra Western India, we have detected a good number of patients with rare bleeding disorders. These disorders can be found on a more frequent basis as we have a significant incidence of consanguineous marriages, as our data shows i.e approx. 33%. This report highlights need for extended family search and molecular characterization so as to make antenatal diagnosis amenable for severe bleeding disorders.

Rab38 Expression in Platelet Dense Granule Storage Pool Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2112-2112
Author(s):  
Ivana Ninkovic ◽  
James G. White ◽  
Kenyatta W. Stephens ◽  
Artur Rangel-Fihlo ◽  
Francsisca C. Wu ◽  
...  
Keyword(s):  
Platelet Aggregation ◽  
Platelet Function ◽  
Flow Cytometric Analysis ◽  
Dense Granule ◽  
Bleeding Disorder ◽  
Coding Region ◽  
Platelet Dysfunction ◽  
Granule Formation ◽  
Storage Pool ◽  
Storage Pool Disease

Abstract Platelet dense granule storage pool disease (SPD) is a bleeding disorder characterized by a lack of normal platelet dense granule function, as evidenced by decreased platelet aggregation in response to ADP, epinephrine and collagen. Platelet SPD has been studied most extensively in humans and rodents with Hermansky-Pudlak syndrome (HPS), whose phenotype is a result of defects in granule trafficking, leading to oculocutanous albinism, lysosomal storage diseases, and platelet dysfunction. We have been characterizing the fawn-hooded hypertensive (FHH) rat, which has been previously shown to have a bleeding disorder consistent with a platelet SPD and some of the features of HPS. While the platelets in the FHH rat have normal alpha granules and lysosomes, they lack dense granules as assessed by transmission electron microscopy. Platelet flow cytometric analysis of GPIb and GPIIb indicated that the FHH platelets have normal surface expression of these adhesion proteins. The FHH rat has a mutation in the Rab38 gene at the ATG start site, which is associated with the bleeding disorder. Rab38 is part of a large family of GTPases, which are involved in granule formation and secretion. Western blotting of FHH tissues revealed that there is no expression of Rab38 protein. We have used confocal immunomicroscopy to assess Rab38 in platelet formation and function. In normal rat and human platelets, there was punctate expression of Rab38. There was no Rab38 staining detected in FHH platelets. In human megakaryocytic cell lines, Dami and HEL cells, there was punctate staining of Rab38 that was mainly in the periphery of the cells, with a variable amount of perinuclear staining. There was partial colocalization of Rab38 with serotonin and VWF, and with Lamp-3, a marker of lysosomes. The degree of colocalization varied between cells. There was no clear association of Rab38 with actin and tubulin in megakaryocytes. We also examined a cohort of patients with SPD, but not HPS, for mutations in Rab38. The entire coding region and intron-exon boundaries of the Rab38 gene were sequenced in 18 patient samples collected at Emory University for the CDC Women with Bleeding Disorders and Menorrhagia Study. Ten of the patients had platelet function defects documented by standard platelet aggregation studies, and eight had no identifiable platelet function defect. No mutations in Rab38 were detected. Whereas numerous known polymorphisms were identified and confirmed, there was no association of any of them with platelet function abnormalities. In conclusion, Rab38 is expressed in platelets and megakaryocytes and may interact with other granule proteins during megakaryocyte development. Failure to express Rab38 is associated with platelet dysfunction. Further studies are needed to determine its function in megakaryocytes and platelets, and to determine whether defects in Rab38 are a cause of platelet SPD in humans.

Inherited Platelet δ-Storage Pool Disease in Dogs Causing Severe Bleeding: An Animal Model for a Specific ADP Deficiency

1995 ◽  
Vol 74 (03) ◽  
pp. 949-953 ◽  
Author(s):  
Mary Beth Callan ◽  
Joel S Bennett ◽  
Deborah K Phillips ◽  
Mark E Haskins ◽  
James E Hayden ◽  
...  
Keyword(s):  
Adenine Nucleotide ◽  
Bleeding Disorder ◽  
Serotonin Uptake ◽  
Severe Bleeding ◽  
Minor Trauma ◽  
Dense Granules ◽  
Storage Pool ◽  
Normal Number ◽  
Platelet Disorder ◽  
Storage Pool Disease

SummaryThe nature of a disorder producing moderate to severe bleeding after minor trauma, venipuncture, and surgery was studied in 3 families of American cocker spaniel dogs. In the 5 affected dogs tested, platelet counts and measurements of plasma coagulant function and von Willcbrand factor were normal. However, bleeding times were prolonged in 4 of the 5 affected dogs tested, and platelet aggregation in response to ADP and collagen was consistently abnormal in 3, suggesting that the bleeding disorder was due to abnormal platelet function. Measurements of 14C-serotonin uptake and retention by the affected platelets were normal. However, their ADP content was decreased, while their ATP content was normal, resulting in a mean ATP/ADP ratio of 8.32, compared to a mean ratio of 1.9 in normal canine platelets. Electron microscopy revealed that the number and appearance of the dense granules in the affected platelets were indistinguishable from those of normal controls. These studies suggest that this bleeding disorder results from a deficient δ-granule storage pool of ADP; given the normal serotonin uptake and retention by affected platelets and the apparently normal number of dense granules, the ADP deficiency may be the consequence of a selective defect in δ-granule ADP transport. Additional studies of this unique platelet disorder will provide an opportunity to understand the mechanism of adenine nucleotide storage in platelet δ granules.

scholarly journals Factor IX is activated in vivo by the tissue factor mechanism

Blood ◽  
1990 ◽  
Vol 76 (4) ◽  
pp. 731-736 ◽  
Author(s):  
KA Bauer ◽  
BL Kass ◽  
H ten Cate ◽  
JJ Hawiger ◽  
RD Rosenberg
Keyword(s):  
Tissue Factor ◽  
Factor Ix ◽  
Factor Vii ◽  
Factor Xii ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Factor Ixa ◽  
Coagulant Activity ◽  
The Mean

Abstract Despite significant progress in elucidating the biochemistry of the hemostatic mechanism, the process of blood coagulation in vivo remains poorly understood. Factor IX is a vitamin K-dependent glycoprotein that can be activated by factor XIa or the factor VII-tissue factor complex in vitro. To investigate the role of these two pathways in factor IX activation in humans, we have developed a sensitive procedure for quantifying the peptide that is liberated with the generation of factor IXa. The antibody population used for the immunoassay was raised in rabbits and chromatographed on a factor IX-agarose immunoadsorbent to obtain antibody populations with minimal intrinsic reactivity toward factor IX. We determined that the mean level of the factor IX activation peptide (FIXP) in normal individuals under the age of 40 years was 203 pmol/L and that levels increased significantly with advancing age. The mean concentration of FIXP was markedly reduced to 22.7 pmol/L in nine patients with hereditary factor VII deficiency (factor VII coagulant activity less than 7%) but was not significantly different from normal controls in nine subjects with factor XI deficiency (factor XI coagulant activity less than 8%). These data indicate that factor IXa generation in vivo results mainly from the activity of the tissue factor mechanism rather than the contact system (factor XII, prekallikrein, high molecular-weight kininogen, factor XI). Our results may also help to explain the absence of a bleeding diathesis in many patients with deficiencies of the contact factors of coagulation.

How to use a coagulation screen

2021 ◽  
pp. edpract-2020-320925
Author(s):  
Sarah Kapur ◽  
Mark Gilmore ◽  
Christine Macartney ◽  
Andrew Thompson
Keyword(s):  
Screening Test ◽  
Sampling Error ◽  
Clinical Information ◽  
Bleeding Disorder ◽  
Bleeding Disorders ◽  
Optimal Conditions ◽  
Normal Ranges ◽  
Sample Technique

A coagulation screen is an important screening test when investigating a child who presents with easy bruising or bleeding. Interpretation of a coagulation screen can be challenging for clinicians. Evolution of the haemostasis system during childhood means normal ranges vary with age and needs to be interpreted alongside the clinical information. It is essential to consider preanalytical variables when interpreting a coagulation screen, and the reason for the investigation must always be considered. It is important that the sample is taken under optimal conditions, including sample technique, use of the correct bottle and prompt transport to the laboratory. An abnormal coagulation screen may indicate an underlying congenital bleeding disorder or an acquired bleeding disorder, or may be due to sampling error. Limitations of the coagulation screen are essential to be aware of, as some children with normal coagulation screen results may have bleeding disorders. Conversely, an abnormal coagulation screen does not always indicate a 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.

Obstetric analgesia and anaesthesia in women with inherited bleeding disorders

2009 ◽  
Vol 101 (06) ◽  
pp. 1104-1111 ◽  
Author(s):  
Claudia Chi ◽  
Christine Lee ◽  
Adrian England ◽  
Jaishree Hingorani ◽  
James Paintsil ◽  
...  
Keyword(s):  
Von Willebrand Disease ◽  
Factor Vii ◽  
Bleeding Disorders ◽  
Factor X ◽  
Factor Xi Deficiency ◽  
Obstetric Analgesia ◽  
Regional Block ◽  
Platelet Function Disorders ◽  
Coagulation Defects ◽  
Von Willebrand

SummaryA retrospective review was carried out on the methods of obstetric analgesia/anesthesia used in 80 pregnancies amongst 63 women with inherited bleeding disorders (19 factor XI deficiency, 16 carriers of haemophilia, 15 von Willebrand disease, seven platelet function disorders, four factor VII deficiency, one factor VII and XI deficiency and one factor X deficiency). In 72 pregnancies, the woman was seen antenatally in a multidisciplinary clinic to discuss and plan pain relief options. Regional block was performed for 41 pregnancies. The mothers were known to have a bleeding disorder in 35 of these pregnancies. Prophylactic cover was given in 10 pregnancies prior to the insertion of regional block but not required in the remaining 25 pregnancies because the coagulation defects had spontaneously normalised at term. There were six reported adverse effects from regional block similar to that found in the general population: inadequate anesthesia/analgesia (2), bloody tap (2), hypotension and a possible dural puncture which was treated conservatively. There were no reports of long-term complications. The findings show that it is possible to offer women with inherited bleeding disorders the option of regional block provided their coagulation defects have normalised, either spontaneously during pregnancy or following adequate haemostatic cover.

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