Women's bleeding represents a major public health problem. Current research supports the hypothesis that a significant number of cases of unexplained menorrhagia may be due to an underlying bleeding disorder, the most common of which is von Willebrand disease (VWD). Unnecessary surgical interventions can thus be avoided with appropriate diagnosis, and bleeding can be controlled with medications.
It is thus necessary to improve:
Diagnosis of affected women with high risk of gynaecological bleeding Treatment and prophylaxis of symptoms
Causes of a difficult diagnosis
Women's perceptions of the magnitude of their menstrual flow often are not reliable. In particular, women with a bleeding disorder may underestimate their losses and consider their symptoms "normal", so that they come to attention only after serious bleeding events Physicians may not suspect the diagnosis. (obstetricians and gynaecologists may not be aware of the role that haemostasis plays in reproductive tract bleeding and haematologist may not be appreciate the nature or range of conditions that that can place their patients at risk) - Lack of availability of accurate laboratory diagnostic tests
Differential diagnosis of an underling bleeding disorders in women with menorrhagia [56]
Gynaecological assessment
Gynaecological causes of abnormal vaginal bleeding (e.g., endometrial polyps, submucosal fibroids, cervicitis, cervical and vaginal lesions, etc.) should be excluded. A complete personal and family history as well as a physical examination including a careful pelvic/vaginal examination should be done. An in-depth coagulation investigation should be considered for the patient with menorrhagia in whom gynaecologic causes have been ruled out. However, a high index of suspicion for a possible bleeding abnormality needs to be maintained in all patients with excessive bleeding, as the prevalence of uterine abnormalities in patients with bleeding disorders in whom the gynaecologic abnormality is "unmasked" by the bleeding disorder, is uncertain. Thus, a coagulation screen may be appropriate as part of the initial assessment, even in the absence of a pelvic examination.
Medical assessment
Initial testing should include:
platelet count haemoglobin / haematocrit ferritin prothrombin time (PT) activated partial thromboplastin time (APTT) thyroid and hepatic screens a serum prolactin
Further investigations
A more in-depth investigation should be considered in the following clinical situations: the menorrhagia is present since menarche there is evidence of anaemia or iron deficiency there is a personal or family history of bleeding after haemostatic challenge (dental extraction, surgery, or parturition) or a family history of menorrhagia, there is no local cause for menorrhagia the first screening tests present abnormalities (such as prolonged PT and/or PTT, ….)
This investigation may include:
bleeding time or closure time PFA-100 blood group von Willebrand studies (factor VIII, VWF antigen, and VWF functional assay) if PT, APTT or TT are prolonged: mixing tests (50:50 normal plasma/patient's plasma) single coagulation factor assays to test both activity and antigen levels These tests can be ordered by the gynaecologist or the family physician or, alternatively, the woman may be referred directly to the haematologist. However, interpretation of abnormal or borderline results usually requires referral to a haematologist (or an internal medicine) consultant. As levels of VWF and factor VIII are affected by a number of factors and, therefore, may fluctuate, it may be necessary to test on more than 1 occasion if there is a positive history but normal VWF levels on initial testing. If there is a positive history in the absence of VWD, further investigation may be indicated for other factor deficiencies, platelet dysfunction, and other rare disorders (such as a2-antiplasmin or factor XIII deficiency) that are not evaluable by screening tests. It is important to have a precise haematological diagnosis because the diagnosis may affect such clinical situations as the treatment of both gynaecologic and non-gynaecologic bleeding, the optimal drugs/therapeutic modalities recommended for the management of severe bleeding, guidelines for patient preparation for surgery including preoperative and post-operative management, and information for family counselling.
Multidisciplinary clinics
The ideal management of women with inherited coagulation defects who suffer from menorrhagia is through multidisciplinary clinics. However, at the present time, very few of those clinics exist and they are all located in tertiary care centres. These clinics include a nurse, a clinical haematologist and a gynaecologist who meet with the patient and liaise with the family physician. The ideal multidisciplinary team would have an even broader representation of expertise, and would include a laboratory haematologist, an obstetrician-gynaecologist, an anaesthesiologist, a family physician, a social worker, a pharmacist, a laboratory technician, and a secretary.
Recommendations
Inherited bleeding disorders should be considered in the differential diagnosis of all patients presenting with menorrhagia. The graphical scoring system presented is a validated tool which offers a simple yet practical method that can be used by patients to quantify their blood loss Because underlying bleeding disorders are frequent in women with menorrhagia, physicians should consider performing a haemoglobin/haematocrit, platelet count, ferritin, PT (INR), and APTT in women with menorrhagia. In women who have a personal history of other bleeding or a family history of bleeding, further investigation should be considered, including a VWD workup (factor VIII, VWF antigen, and VWF functional assay).