Post-partum hemorrhage in women with rare bleeding disorders (original) (raw)
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Inherited Bleeding Disorders in the Obstetric Patient
Transfusion Medicine Reviews, 2018
Inherited bleeding disorders increase the risk of bleeding in the obstetric patient. Randomized controlled trials to compare prophylactic or therapeutic interventions are rare, and guidance documents rely heavily on expert opinion. Here we report the results of a systematic review of the literature for the treatment and prevention of peripartum bleeding in women with an inherited bleeding disorder. The highest quality evidence is for the use of tranexamic acid (TXA) in postpartum hemorrhage (PPH), which has been shown to decrease bleeding-related mortality in women without bleeding disorders. There is limited evidence for prophylactic use of this agent in women with inherited bleeding disorders. Desmopressin has also been used in observational studies of patients with VWD and carriers of hemophilia A with some success, although concerns about the risk of hyponatremia persist. In patients with deficiencies of specific factors, replacement is generally the preferred approach and concentrates have been studied in deficiencies of VWF and factors VII, VIII, IX, XI, and XIII as well as in patients with fibrinogen deficiency. Due to the small size of these studies, neither safety nor efficacy are well-established, although the literature suggests that bleeding history may be more predictive of outcomes than factor levels in many cases. Goal factor levels have not been studied or systematically established in any of these diseases, although observational data suggest that achieving normal levels may be inadequate, particularly for VWF and factor VIII, which are physiologically elevated in pregnancy. For factor deficiencies in which no specific concentrate is available, such as factors II (prothrombin) and V, prothrombin complex concentrate or fresh frozen plasma may be used and for platelet defects or deficiencies, such as GT or BSS, platelet transfusion is generally first line, although use of recombinant FVIIa has been reported in patients with GT to avoid development of, or treat patients with, antibodies to platelet GpIIbIIIa. Ultimately, data are lacking to definitively support an evidencebased approach to management in any of these disorders and prospective, controlled studies are desperately needed.
Gynaecological and obstetrical problems in women with different bleeding disorders
Haemophilia, 2009
The aim of this case-control study was to analyse the prevalence of gynaecological, obstetrical and other more general bleeding symptoms in 114 women affected by various inherited bleeding disorders, who were compared with 114 apparently healthy women. Retrospective information were collected by means of two specific questionnaires, one on gynaecological and obstetrical bleeding symptoms, with special focus on the presence of menorrhagia as defined by a pictorial blood loss assessment chart (PBAC); and the other on general bleeding symptoms, whose severity was graded by means of the bleeding score (BS). Compared to normal women, the whole group of women with inherited bleeding disorders had a higher prevalence of excessive bleeding at menarche (25% vs. 5%, P < 0.0001) and menorrhagia (59% vs. 46%, P = 0.06). Affected women also had a higher frequency than controls of general bleeding symptoms that scored as severe by a BS ‡ 12 (49% vs. 0%, P < 0.0001). In affected women, the BS increased according to the severity of the haemostasis defect. In conclusions, the BS and the PBAC are simple tools to evaluate the severity of general bleeding symptoms and menorrhagia in women with inherited bleeding disorders. These instruments may help to identify those women for whom a therapeutic intervention is warranted.
Prenatal diagnosis in rare bleeding disorders-An unresolved issue?
International Journal of Laboratory Hematology, 2018
Rare bleeding disorders (RBD) account for 3%-5% of all inherited coagulation factor deficiencies, including deficiencies of coagulation factors I (fibrinogen), factor (F) II, FV, combined FV and FVIII, FVII, FX, FXI, FXIII and congenital deficiency of vitamin K-dependent clotting factors (VKCFD). These disorders most often inherited in autosomal recessive manner, except for some cases of FXI deficiency (FXID) and FID. Prevalence of RBD varies from 1 per 300 000 to 500 000 for FVIID to 1 per 2 million for FXIIID and FIID. 1 Bleeding tendency significantly varies between these disorders, and most patients with combined FV-FVIIID (CFV-VIIID) and FVD have mild bleeding and patients with FXID bleeds only after trauma or surgery but most patients with FXIIID have severe life-threatening bleeding. 2,3 Intracranial haemorrhage (ICH) as the most dreadful and the main cause of death among patients with RBD is commonly observed in congenital FXIIID. About one-third of these patients without appropriate prophylaxis treatment experience this diathesis. 4 ICH was less frequently reported in FVIID, FID and FXD, and rarely was observed in FIID and FVD. 5 Although ICH is the main cause of morbidity and mortality, timely diagnosis and appropriate management of these bleeding disorders can significantly reduce the rate of ICH and related adverse consequences. 4,5 In severely affected patients with RBD, ICH most often spontaneously occurred early in life. This presentation can occur intrauterine, immediately after
Proceedings of Shaikh Zayed Postgraduate Medical Institute, 2019
Introduction: Bleeding issues specific to women and adolescent females are common across the world. These issues can present either due to gynecological causes or obstetric one; however these can result in significant morbidity and work up needs to be done to rule out any underlying bleeding diathesis. Aims & Objectives: To determine frequency of various bleeding disorders among gynecological and obstetric patients presenting with bleeding. Place and duration of study: It is a cross sectional study which was conducted in the Hematology Department, Services Institute of Medical Sciences, Lahore. The duration of study was 6 months i.e from 21 st Oct, 2018 to 30 th April, 2019. Material & Methods: Females with age range of 15 to 50 years irrespective of pregnancy presenting as menorrhagia, metrorrhagia or obstetrical bleeding were included. Results: In the present study there were total 140 cases with mean age of 30.14±9.59 years. There were 98 (70%) cases that were pregnant. There were 35 (25%) cases presented with PPH (Post Partum Hemorrhage), 42 (30%) with IPH (Intra Partum Hemorrhage), 49 (35%) with menorrhagia and 14 (10%) with metrorrhagia. HELLP (Hemolysis, Elevated Liver enzymes and Low Platelet count) syndrome was seen in 13 (9.29%) cases, DIC (Disseminated Intravascular Coagulation) in 2 (1.43%), gestational thrombocytopenia in 7 (5%), while factor deficiency in 34 (24.29%) cases; and 84 (60%) of cases revealed none of the bleeding disorder. Out of total 34 cases of factor deficiencies, vWD was the most common, seen in 20 (58.82%) cases. There was none of the cases seen having deficiency of factor II, VII, VIII, XI, XII. HELLP syndrome was seen in 11 (84.62%) of the cases with age group 15 to 32 years and similarly both cases of DIC were seen in same age group; but the difference in both the age groups regarding these bleeding disorders was not statistically significant with p= 0.38. There was no significant difference in terms of pregnancy; however, both cases of DIC were seen in pregnancy with insignificant p value of 0.51. HELLP syndrome was more seen in IPH affecting 5 (38.40%), factor deficiency in menorrhagia affecting 13 (38.2%) with p value of 0.61. vWD and factor V Leiden presented in 44.4% cases each with PPH, and vWD affected 87.5% of cases in IPH without any significant difference with p= 0.42. Conclusion: Bleeding disorders are not uncommon in gynecological and obstetric conditions. Factor deficiency were the most common causes and amongst these vWD was the most deficient factor. There was no significant association with any confounding variable; however, DIC was only seen in obstetrical conditions.
Postpartum Hemorrhage; Incidence and Prognosis
During the study period a total of 1000 deliveries; 74 parturients presented with PPH, an incidence of 7.4% [95% CI 6.3-6.5] with that of severe PPH at 2.7% [95% CI 1.6-1.8]. maternal age varied between 30 and 42 years. The average age in our sample was: 34.33 ± 6.030 years. Mean term: 38.69 ± 1.25 weeks gestation mean was 3.8 ± 1.6. The oldest parity was 3.2 ± 2 with extremes of 1 and 8. 73.7% were multiparous (2 deliveries and more). RBC transfusion was done in 97.5%. Oxytocics were administered in 95.8%. Uterine inertia following a placentation anomaly (accreta, increta and percreta) remains the most frequent etiology in 79.8%, and coagulation disorders 15.2% (congenital or acquired) and 5% of entangled causes (ATCD from Covid19, death in utero, help). As for the mode of delivery, the upper route is the most frequent with 70% of cases RBC transfusion was done in 97.5%. Oxytocics were administered in 95.8%. Uterine revision was performed in 80.6%. , artificial delivery was done in 26.4%. Vascular ligation was done in 20.8%. Hemostatic hysterectomy was required in 19.4%. Hospitalization in the maternal intensive care unit was necessary in 70.9% with an average duration of 3.5 days. There were 73% cases of disseminated intravascular coagulation (DIC), 50% renal failure (IR) of which two were hemodialysis. The death rate was 18%. Three risk factors associated with a poor maternal prognosis: severe hypovolaemia (p = 0.0001), disseminated intravascular coagulation abnormalities (p = 0.006) and organic renal failure (p = 0.0013).
Risk factors for severe postpartum hemorrhage: a case-control study
BMC pregnancy and childbirth, 2017
In high-income countries, the incidence of severe postpartum hemorrhage (PPH) has increased. This has important public health relevance because severe PPH is a leading cause of major maternal morbidity. However, few studies have identified risk factors for severe PPH within a contemporary obstetric cohort. We performed a case-control study to identify risk factors for severe PPH among a cohort of women who delivered at one of three hospitals in Norway between 2008 and 2011. A case (severe PPH) was classified by an estimated blood loss ≥1500 mL or the need for blood transfusion for excessive postpartum bleeding. Using logistic regression, we applied a pragmatic strategy to identify independent risk factors for severe PPH. Among a total of 43,105 deliveries occurring between 2008 and 2011, we identified 1064 cases and 2059 random controls. The frequency of severe PPH was 2.5% (95% confidence interval (CI): 2.32-2.62). The most common etiologies for severe PPH were uterine atony (60%) ...
“Post partum hemorrhage: causes and management”
BMC Research Notes, 2013
Background Post partum hemorrhage is defined as blood loss of 500 ml or above. It is the most common cause of pre-mature mortality of women world wide. Our objective was to evaluate the most common etiology and method of management of Post partum Hemorrhage in a tertiary care hospital of Karachi. Findings It was a cross sectional study conducted at Liaquat National Hospital Karachi, during the period of July 2011 to May 2012. Review include mode of delivery, possible cause of postpartum hemorrhage, supportive, medical and surgical interventions. All the women admitted with post partum hemorrhage or develop PPH in hospital after delivery were included in our study. Bleeding disorder and use of anticoagulants were set as exclusion criteria. Diagnosis was made on the basis of blood loss assessment which was made via subjective and objective evaluation. During the targeted months, out of total 1493 deliveries (26/1493 = 1.741%) 26 cases of post partum hemorrhage were reported with a mea...