Glanzmann’s thrombasthenia is a rare autosomal recessive bleeding syndrome characterised by a lack of platelet aggregation [ 1 ]. Mucocutaneous bleeding with absent platelet aggregation, in the presence of a normal platelet count is diagnostic for this condition [ 1 ]. Treatment is usually through platelet transfusion, however the severe onset of menorrhagia for women is a frequent clinical problem and is treated with high doses of progesterone [ 1 ]. Pregnancy, especially the delivery, accounts for severe haemorrhagic risk; requiring platelet transfusion prior
disorder were to report an annual bleed rate of 12, clinical alarm bells would ring; however, women tend not to receive the same degree of attention when experiencing heavy periods. Table 1 Prevalence of menorrhagia in women with bleeding disorders [ 1 ] DISORDER PREVALENCE (%) Von Willebrand disease 32-100 Platelet dysfunction Glanzmann’s thrombasthenia 51 Bernard-Soulier syndrome 13-98 Haemophilia 10-57 Factor XI deficiency 59 Factor XIII deficiency 35-64 Rare factor deficiencies 35-70 Talking about heavy periods There are cultural taboos that inhibit
number of patients (n=7) [ 38 ]. Tran et al. also reported the safety of a combination of TXA and bypassing agents [ 39 ]. Menorrhagia is common in women with bleeding disorders and is an extremely common presenting symptom in a number of disorders, including, vWD, platelet function disorders, Factor VII and Factor XI deficiencies, and in carrier states of haemophilia A and haemophilia B. Kouides et al. demonstrated that TXA is more effective in reducing menorrhagia compared to DDAVP [ 40 ]. The safety and effectiveness of TXA in menorrhagia and pregnancy has been
Introduction: In childhood, thrombocytopenia caused by transient antibody-mediated thrombocyte destruction is most frequently diagnosed as immune thrombocytopenic purpura (ITP). We report the case of a girl with ITP associated with autoimmune thyroiditis.
Case presentation: A 11-year-old female patient with Hashimoto’s thyroiditis presented with clinical signs of petechiae and ecchymoses on the extremities. Laboratory tests showed remarkable thrombocytopenia with a platelet count of 44,500/μL, hence she was referred to a hematologic consultation. The peripheral blood smear showed normal size platelets in very low range. The bone marrow examination exposed hyperplasia of the megakaryocyte series with outwardly morphologic abnormalities. The patient was diagnosed with ITP, and her first-line treatment was pulsed steroid and immunoglobulin therapy. The thrombocytopenia was refractory to these first-line medications. After 6 months of corticotherapy and a period of severe menorrhagia, azathioprine immunosupression was initiated as a second-line treatment. Her platelet count rapidly increased, and the evolution was good, without bleeding complications.
Conclusion: In case of a medical history of autoimmune diseases and treatment-resistant ITP, attention must be focused on detecting coexisting autoimmune diseases and adjusting the treatment in accordance with the chronic evolution of the disease.
, Bullerdiek J. Karyotype evolution in a case of uterine angioleiomyoma. Cancer Genet Cytogenet. 1999; 108:79-80. 10.1016/S0165-4608(98)00123-X 8. Hsieh CH, Lui CC, Huang SC, Ou YC, Chang Chien CC, Lan KC, et al. Multiple uterine angioleiomyomas in a woman presenting with severe menorrhagia. Gynecol Oncol. 2003; 90:348-52. 10.1016/S0090-8258(03)00263-4
Gynecol. 1999;163:1650. 5. Ghazizadeh S, Bakhtiari F, Rahmanpour H, Davari-Tanha F, Ramezanzadeh F. A randomized clinical trial to compare levonorgestrel-releasing intrauterine system (Mirena) vs transcervical endometrial resection for treatment of menorrhagia. Int J Womens Health. 2011;3:207-11. 6. Caspi B, Elchalal U, Dgani R et al. Invasive mole and placental site trophoblastic tumor.Two entities of gestational trophoblastic disease with a common ultrasonographic appearance. J Ultrasound Med. 1991;10:517.
awareness of bleeding disorders. So, we did a survey with the Royal College of Obstetricians to see how aware gynaecologists were of underlying bleeding disorders in menorrhagia. Our results showed that very few gynaecologists test or screen for von Willebrand’s disease and underlying bleeding disorders in their patients with menorrhagia [ 5 ]. Similar results were published in the United States [ 6 ]. As a result of this, we worked with the Haemophilia Society and set up the “Women Bleed Too” project, with the aim of raising awareness and providing support to women with
Surgery in women with von willebrand disease or factor VII deficiency TThe recorded prevalence in Slovakia of von Willebrand disease (VWD) and, in particular, factor VII deficiency is among the highest in the world [ 1 ] . Gynaecological problems are more common among young women with VWD and other bleeding disorders than unaffected women [ 2 , 3 ] . Women with a bleeding disorder may need to undergo surgery for menorrhagia (dilatation and curettage, endometrial ablation, hysterectomy), ovulatory bleeding and endometriosis (laparoscopy, laparotomy, oophorectomy
important issue and online comments cannot be independently verified. Indications and doses The indications and doses licensed in the UK [ 13 , 17 ] have been used for the purpose of this review, on the basis that the data are reliable and the disposition of tranexamic acid is not affected by ethnicity. Licensed indications in the UK Cyklokapron tablets are licensed for short-term use for haemorrhage or risk of haemorrhage in increased fibrinolysis or fibrinogenolysis, and local fibrinolysis as it occurs in prostatectomy and bladder surgery, menorrhagia, epistaxis
Surveyor, DNA Variant Analysis Software (Softgenetics, Pennsylvania, US). Case report Family 1: A young woman aged 21 (Proband 1) was referred to the haemophilia centre for investigation of menorrhagia, with onset at the menarche. It transpired that all of the female members of her family had menorrhagia, which affected a mother, aged 46, and all three of her daughters, aged 21, 19 and 16 years. On further questioning, it was ascertained that the mother had bled for several days following a dental extraction, but had not bled following three spontaneous vaginal