OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press1110.17650/1818-8346-2014-9-1-47-51RARE DISEASESРЕДКИЕ БОЛЕЗНИCoagulation disoders as a cause of juvenile uterine bleeding in 12–18 years adolescent girlsНарушения свертывания крови как причина ювенильных маточных кровотечений у девочек-подростков 12–18 летDmitrievV. V.ДмитриевВ. В.
Russian Federation
dmitrievhaematol@mail.ruMozheykoL. F.МожейкоЛ. Ф.
Russian Federation

Department of Obstetrics and Gynecology

кафедра акушерства и гинекологии

GuzeyI. A.ГузейИ. А.
Russian Federation

Department of Obstetrics and Gynecology

кафедра акушерства и гинекологии

Republican Scientific Centre of Pediatric Oncology, Hematology and Immunology, Ministry of Health of Republic of BelarusГУ «Республиканский научно-практический центр детской онкологии, гематологии и иммунологии» Министерства здравоохранения Республики БеларусьBelarusian State Medical University«Белорусский государственный медицинский университет»210720149147512107201421072014https://oncohematology.abvpress.ru/ongm/article/view/11

Obligatory detection of vWF blood levels, progesterone and testosterone levels in addition to routine tests are necessary to identify the causes of juvenile uterine bleeding. If progesterone level less than 1.75 ng/ml, and vWF activity in first phase of the cycle varies in the range 30–39 %, hyperpolymenorrhea may be due to coagulation disorders. Diagnostic range of vWF activity, within which hyperpolymenorrhea considered as a clinical sign of latent coagulation “disorders”, should be extended to 35 % for patients with O(I) blood group and to 39 % for patients with A (II), B (III) and AB (IV) blood group.

Для выявления причины ювенильного маточного кровотечения у девочек-подростков кроме выполнения рутинных лабораторных исследований необходимо обязательное определение в крови активности фактора Виллебранда, уровней прогестерона и тестостерона. Если содержание прогестерона < 1,75 нг/мл, а активность фактора Виллебранда в первой фазе цикла изменяется в диапазоне 30–39 %, то изменения свертывания могут быть одной из причин гиперполименореи. Диагностический диапазон активности и содержания фактора Виллебранда, в пределах которого гиперполименорею рассматривают как клинический признак «скрытых нарушений» свертывания, следует расширить до 35 % для пациенток с О(I) групповой принадлежностью и до 39 % для пациенток с A(II), В(III) и АВ(IV) групповой принадлежностью.

adolescent girlsjuvenile uterine bleedingblood coagulationhormonal statusdiagnosticsдевочки-подросткиювенильное маточное кровотечениесвертывание кровигормональный статусдиагностика1. Kouides P.A. Bleeding symptom assessment and Hemostasis evaluation of menorrhagia. Curr Opin Hematol 2008;15:465–72.Kouides P.A. Bleeding symptom assessment and Hemostasis evaluation of menorrhagia. Curr Opin Hematol 2008;15:465–72.2. Demers C., Derzko C., David M. et al. Gynaecological and obstetric management of women with inherited bleeding disorders. J Obstet Gynaecol Can 2005;27(7):707–32.Demers C., Derzko C., David M. et al. Gynaecological and obstetric management of women with inherited bleeding disorders. J Obstet Gynaecol Can 2005;27(7):707–32.3. Toogeh G., Sharifian R., Lak M. et al. Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran. Am J Hematol 2004;77:198–9.Toogeh G., Sharifian R., Lak M. et al. Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran. Am J Hematol 2004;77:198–9.4. Kadir R., Chi C., Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009;15:990–1005.Kadir R., Chi C., Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009;15:990–1005.5. Kadir R.A., Edlund M., von Mackensen S. he impact of menstrual disorders on quality of life in women with inherited bleeding disorders. Haemophilia 2010;16:832–9.Kadir R.A., Edlund M., von Mackensen S. he impact of menstrual disorders on quality of life in women with inherited bleeding disorders. Haemophilia 2010;16:832–9.6. Asahina T., Kobayashi T., Takeuchi K., Kanayama N. Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature. Obstet Gynecol Surv 2007;62:255–60.Asahina T., Kobayashi T., Takeuchi K., Kanayama N. Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature. Obstet Gynecol Surv 2007;62:255–60.7. Peyvandi F., Garagiola I., Menegatti M. Gynecological and obstetrical manifestations of inherited bleeding disorders in women. J Thromb Haemost 2011;9(Suppl 1):236–45.Peyvandi F., Garagiola I., Menegatti M. Gynecological and obstetrical manifestations of inherited bleeding disorders in women. J Thromb Haemost 2011;9(Suppl 1):236–45.8. Diaz A., Laufer M.R., Breech L.L.; American Academy of Pediatrics Committee on Adolescence, American College of Obstetricians and Gynecologists Committee on Adolescent Health Care. Menstruation in girls and adolescents: using the menstrual cycle as a vital sign. PediatricsDiaz A., Laufer M.R., Breech L.L.; American Academy of Pediatrics Committee on Adolescence, American College of Obstetricians and Gynecologists Committee on Adolescent Health Care. Menstruation in girls and adolescents: using the menstrual cycle as a vital sign. Pediatrics2006;118:2245–50.;118:2245–50.9. James A.H., Kouides P.A., Abdul-Kadir R. et al. Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel. Am J Obstet Gynecol 2009;201:12–22.James A.H., Kouides P.A., Abdul-Kadir R. et al. Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel. Am J Obstet Gynecol 2009;201:12–22.10. Federici A.B., Castaman G., Mannucci P.M. et al. Guidelines for the diagnosis and management of von Willebrand disease in Italy. Italian Association of Hemophilia Centers (AICE). HaemophiliaFederici A.B., Castaman G., Mannucci P.M. et al. Guidelines for the diagnosis and management of von Willebrand disease in Italy. Italian Association of Hemophilia Centers (AICE). Haemophilia2002;8(5):607–21.;8(5):607–21.11. National Heart, Lung, and Blood Institute. NHLBI Von Willebrand Disease Expert Panel. The diagnosis, evaluation and management of von Willebrand disease. NIH publication no. 08-5832. Bethesda, Md.: U.S. Department of Health and Human Services; December 2007. http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. Accessed April 26, 2010.National Heart, Lung, and Blood Institute. NHLBI Von Willebrand Disease Expert Panel. The diagnosis, evaluation and management of von Willebrand disease. NIH publication no. 08-5832. Bethesda, Md.: U.S. Department of Health and Human Services; December 2007. http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. Accessed April 26, 2010.12. Pasi K.J., Collins P.W., Keeling D.M. et al. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004;10(3):218–31.Pasi K.J., Collins P.W., Keeling D.M. et al. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004;10(3):218–31.