Gynaecological management of women with inherited bleeding disorders. A UK Haemophilia Centres Doctors' Organisation Guideline
Heavy menstrual bleeding
Inherited bleeding disorder and fertility
Inherited bleeding disorders
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AbstractWomen with inherited bleeding disorders (IBDs) may present to healthcare professionals in a variety of ways and commonly will be encountered by either haematology or gynaecology services. Heavy menstrual bleeding is very often the first manifestation of an IBD. There is a wide variation in severity of bleeding for women with IBD and diagnosis and subsequent management of their condition requires multidisciplinary specialised care which is tailored to the individual and includes excellent cross-specialty communication between gynaecology and haematology teams. This guideline is intended for both haematologists and gynaecologists who are involved in the diagnosis and management of women with bleeding disorders. It sets out recommendations about how to investigate heavy menstrual bleeding (HMB), the commonest presentation for women with IBD to hospital services, to guide physicians about how to diagnose an IBD and covers the management of women with known IBD and HMB. The second section sets out recommendations for patients known to have IBD and covers management of patients with IBD in the setting of gynaecological surgery and management for all other non-surgical gynaecological situations.
CitationCurry, N., Bowles, L., Clark, T. J., Lowe, G., Mainwaring, J., Mangles, S., Myers, B., & Kadir, R. A. (2022). Gynaecological management of women with inherited bleeding disorders. A UK Haemophilia Centres Doctors' Organisation Guideline. Haemophilia : the official journal of the World Federation of Hemophilia, 28(6), 917–937. https://doi.org/10.1111/hae.14643
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Trends in variceal bleeding: A single centre experience from 2006-2013Grant, C; Kemp, D; Austin, Andrew (2014-06)Introduction Over the last decade, the numbers of patients presenting with chronic liver disease has risen. During this period the approach to the treatment of variceal bleeding has undergone important changes both internationally (adoption of early TIPSS in high risk cases), and locally with the development of a 24 h endoscopy service (2006), movement to single site hospital with enlarged intensive care capacity (2009), adoption of the DanisTM stent (2009) and a shift to carvedilol as the primary agent for prophylaxis (2013). We reviewed all episodes of variceal bleeding in the last 8 years to describe patient outcomes. Methods All episodes of bleeding from oesophageal varices managed in the Liver Unit at Royal Derby Hospital from 2005 to mid 2013 were identified from clinical coding data - population served approx. 650,000. A retrospective review of the patient records identified the aetiology and severity of liver disease, morbidity, mortality, endoscopy findings and episodes of rebleeding. Results Each year between 17 and 31 patients presented with variceal bleeding. 5 day mortality fluctuated between 3-22% whereas 30 day mortality fell steadily from a peak in 2006 of 41% to 5% in 2012 (Figure 1). The reduction in mortality was in Child's B/C cirrhosis. Interestingly, the proportion of episodes in Child's A cirrhosis increased from 2009 onwards (7% of all bleeding episodes in 2009 to above 30% in 2013). 30 day mortality rates for Child's A did not improve but remained lower than for those with Child's B/C cirrhosis (mean 9.8% compared to 22.8% (2009-2013)). From 2007, there was a fall in frequency of rebleeding from 35% to below 10% in 2013. Only 3 high risk patients underwent an early TIPSS procedure, all after 2012. (Figure presented) Conclusion Variceal bleeding rates have remained surprisingly constant over 8 years despite the rise in admissions with chronic liver disease. Outcomes for acute variceal bleeding have improved which is likely the result of several organisational changes. Notably, rebleeding rates and 30 day mortality decreased even before the adoption of early TIPSS.
Late discovery of a case with TAR syndrome who presented with an additional potential bleeding risk other than thrombocytopeniaVan Staden, Bernhard; Ahmad, Humayun (2013-04)The name of this syndrome based on an acronym describing the so called 'most obvious' clinical features: Absent Radii & Thrombocytopenia (TAR). There may however be more to TAR cases than what the name says. Development of molecular genetics has provided us with more information and given us a better understanding of these rare conditions. The deletion of chromosome 1q21.1 associated with TAR syndrome, involves the RBM8A gene which is associated with the production of certain cellular proteins. A newly referred 33 year old woman presented with conspicuous upper limb deformities in the presence of thrombocytopenia (platelets 63) at one of our clinics. The patient was never diagnosed nor given a specific name for her abnormal physical appearance in the past. Plain x-rays of the forearms confirmed absent radii. An ultrasound of the abdomen to exclude splenic sequestration of the platelets surprisingly revealed lesions in keeping with arterio-venous malformations/ haemangioma of the liver. It was uncertain if this finding was related to TAR syndrome, however not unlikely as these cases may present with other arterio-venous malformations, like facial haemangiomas and intracranial vascular malformations. The bleeding risk associated with this finding and particularly in this patient with thrombocytopenia was of great concern. The case was also referred to the hepatology team for further management of the hepatic haemangioma. One can conclude from this case that patients with suspected TAR syndrome may require more detailed imaging as there are many other features, some quite life threatening, which are not included in the short acronym based name.