Inherited bleeding disorders in Indian women with menorrhagia

Abstract

In order to define the prevalence of haemostatic defects in women presenting with menorrhagia in our region, the coagulation data on women bleeders investigated in the Department of Haematology, AIIMS, were analysed. A total of 337 of the 2200 menorrhagic women investigated were characterized to have an inherited bleeding disorder; 221 of these 337 women presented with menorrhagia alone while 116 also had other associated bleeding manifestations as prolonged bleeding from injury site, ecchymotic patches in the skin, epistaxis, haematomas, haemarthroses and major bleeds like intracerebral bleeding. The tests performed included bleeding time (BT), platelet count, prothrombin time (PT), prothrombin consumption index (PCI), activated partial thromboplastin time (APTT), PF3 release with adenosine diphosphate (ADP) at 0 and 20 min, total PF3 assay and platelet Aggregation studies with collagen, ADP, adrenaline, arachidonic acid and ristocetin. Coagulation factor assays, von Willebrand antigen estimation, ristocetin cofactor assay and electron microscopy were performed wherever necessary. Inherited platelet dysfunction was seen in 283 (83.9%) of the patients. Amongst these, isolated PF3 availability defect was seen in 163 (48.4%) cases. Glanzman's thrombasthenia was seen in 30 (8.9%) patients, Storage pool disease in eight (2.4%) patients, arachidonic acid pathway defect in five (1.5%) patients and Bernard-Soulier Syndrome in six (1.8%) patients. In 71 (21.1%) patients, the platelet function defect could not be classified into any specific subtypes. Inherited defects of coagulation were observed in 54 (16%) of the cases. Amongst these, von Willebrand disease (vWD) was the most frequent being seen in 40 (11.9%) of the cases. Factor XIII deficiency was seen in one (0.3%), factor X deficiency in four (1.2%), factor VII deficiency in one (0.3%) and factor XII deficiency in one (0.3%) of the patients. It is concluded that although hereditary platelet function defects constitute a large majority of women bleeders in India but among the coagulation defects, vWD is the commonest as reported from the caucasian population. It is thus suggested that in women presenting with menorrhagia, screening tests for haemostasis especially for vWD and inherited platelet function defects must be performed.

Gynecological Problems of Adolescent Girls Attending Outpatient Department at Tertiary Care Center with Evaluation of Cases of Puberty Menorrhagia Requiring Hospitalization

“…Khosla AH et al showed 27 % patients in their study had bleeding disorders [8]. Bevan et al and Saxena R et al showed 13-15 % incidence of thrombocytopenia among girls presented with menorrhagia, which was associated with bruising, petechiae, and mucosal bleeding [20,21]. In our institute, one patient of ITP was managed with blood transfusion and oral progestogen and tranexamic acid.…”

The condensed MCMDM-1 VWD bleeding questionnaire as a predictor of bleeding disorders in women with unexplained menorrhagia

“…The prevalence of VWD in women presenting with menorrhagia ranges between 10 and 20% in Caucasian women, with a lower (1-2%) prevalence in African-American women [3,8,10,11]. In addition, platelet function defects are reported to be the second common cause of bleeding disorders in women presenting with menorrhagia [8,12]. Single coagulation factor deficiencies have also been reported, although at lower frequency than VWD and platelet function defects [3,6,8,12].…”

“…In addition, platelet function defects are reported to be the second common cause of bleeding disorders in women presenting with menorrhagia [8,12]. Single coagulation factor deficiencies have also been reported, although at lower frequency than VWD and platelet function defects [3,6,8,12].…”

Age and the Prevalence of Bleeding Disorders in Women With Menorrhagia

“…9 Single coagulation factor deficiencies, particularly factors XI, VII, and V, have also been identified in studies of women with menorrhagia, although at lower frequencies than von Willebrand's disease and platelet function defects. 3,7,9,15 Menorrhagia occurring during adolescence and perimenopause is presumed to be associated with anovulation or, during perimenopause, also anatomic causes, such as submucosal uterine myoma. 1 The frequency of bleeding disorders in women diagnosed with menorrhagia at the extremes of menstruating age, ie, adolescence and perimenopause, are not known.…”