THROMBOSIS OF UNUSUAL SITES AND THROMBOPHILIA
Abstract
Thrombosis of unusual sites (hepatic vein thrombosis, splenic vein thrombosis, mesenteric vein thrombosis, ovarian vein thrombosis, retinal vein thrombosis, cerebral vein thrombosis, portal vein thrombosis, axillary and subclavian vein thrombosis) are life-threatening disorders that often goes unrecognized. Most often thrombosis of
atypical locations develops in patients with inherited hemostasis defects - genetic thrombophilia. Thrombosis of unusual sites often accompany acquired abnormalities of hemostasis, which include antiphospholipid syndrome, hypercoagulability associated with pregnancy, oral contraceptives use, malignant tumors, in conditions of ovarian
hyperstimulation in IVF programs. In case of thrombosis of unusual sites screening for hereditary forms of thrombophilia and antiphospholipid syndrome should be performed and timely administration of anticoagulant therapy is critical. Analysis of genetic and acquired abnormalities of hemostasis allows you to select a group of patients requiring longterm anticoagulation therapy and mandatory anticoagulant prophylaxis in situations with a high risk of thromboembolic complications (pregnancy, surgery).
atypical locations develops in patients with inherited hemostasis defects - genetic thrombophilia. Thrombosis of unusual sites often accompany acquired abnormalities of hemostasis, which include antiphospholipid syndrome, hypercoagulability associated with pregnancy, oral contraceptives use, malignant tumors, in conditions of ovarian
hyperstimulation in IVF programs. In case of thrombosis of unusual sites screening for hereditary forms of thrombophilia and antiphospholipid syndrome should be performed and timely administration of anticoagulant therapy is critical. Analysis of genetic and acquired abnormalities of hemostasis allows you to select a group of patients requiring longterm anticoagulation therapy and mandatory anticoagulant prophylaxis in situations with a high risk of thromboembolic complications (pregnancy, surgery).
About the Authors
S. V. Akinshina
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
Russian Federation
A. D. Makatsariya
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
Russian Federation
References
1. Sherlok Sh., Dzhulii Dzh. Diseases of the liver and biliary tract: A Practical Guide. Lane. with Engl. / Rev. Ed. Z.G. Aprosin, N.A. Mukhin [Zabolevaniya pecheni i zhelchnykh putei: Prakticheskoe rukovodstvo. Per. s angl./ Pod. Red. Z.G. Aprosinoi, N.A. Mukhina.] moscow. 2002; 864 s.
2. Abu el-Asrar A.M., al-Momen A.K., al-Amro S., Abdel Gader A.G., Tabbara K.F. Prothrombotic states associated with retinal venous occlusion in young adults. Int Ophthalmol. 1996-1997; 20 (4): 197-204.
3. Ağaoğlu N., Mustafa N.A., Türkyilmaz S. Prothrombotic disorders in patients with mesenteric vein thrombosis. J. Invest. Surg . 2003 Sep-Oct; 16 (5): 299-304.
4. Al-toma A., Heggelman B.G., Kramer M.H. Postpartum ovarian vein thrombosis: report of a case and review of literature. Neth. J. Med. 2003 Oct; 61 (10): 334-6.
5. Amitrano L., Brancaccio V., Guardascione M.A., Margaglione M., Iannaccone L., Dandrea G., Ames P.R., Marmo R., Mosca S., Balzano A. High prevalence of thrombophilic genotypes in patients with acute mesenteric vein thrombosis. Am. J. Gastroenterol. 2001 Jan; 96 (1): 146-9.
6. Amitrano L., Guardascione M.A., Scaglione M., Pezzullo L., Sangiuliano N., Armellino M.F., Manguso F., Margaglione M., Ames P.R., Iannaccone L., Grandone E., Romano L., Balzano A. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am. J. Gastroenterol. 2007 Nov; 102 (11): 2464-70.
7. Andre M., Delevaux I., Amoura Z., Corbi P., Courthaliac C., Aumaitre O., Piette J.C. Ovarian vein thrombosis in the antiphospholipid syndrome. Arthritis Rheum. 2004 Jan; 50 (1): 183-6.
8. Avecillas J.F., Falcone T., Arroliga A.C. Ovarian hyperstimulation syndrome. Crit. Care Clin. 2004 Oct; 20 (4): 679-95.
9. Bahnson R.R., Wendel E.F., Vogelzang R.L. Renal vein thrombosis following puerperal ovarian vein thrombophlebitis. Am. J. Obstet. Gynecol. 1985; 152 (3): 290-1.
10. Beigi R.H., Wiensenfeld H.C. Enoxaparin for postpartum ovarian vein thrombosis. A case report. J. Reprod. Med. 2004 Jan; 49 (1): 55-7.
11. Bergqvist D., Svensson P.J. Treatment of mesenteric vein thrombosis. Semin. Vasc. Surg. 2010 Mar; 23 (1): 65-8.
12. De Stefano V., Fiorini A., Rossi E., Za T., Farina G., Chiusolo P., Sica S., Leone G. Incidence of the JAK2 V617F mutation among patients with splanchnic or cerebral venous thrombosis and without overt chronic myeloproliferative disorders. J. Thromb. Haemost. 2007 Apr; 5 (4): 708-14.
13. De Stefano V., Leone G., Mastrangelo S., Tripodi A., Rodeghiero F., Castaman G., Barbui T., Finazzi G., Bizzi B., Mannucci P.M. Clinical manifestations and management of inherited thrombophilia: retrospective analysis and follow-up after diagnosis of 238 patients with congenital deficiency of antithrombin III, protein C, protein S. Thromb. Haemost. 1994 Sep; 72 (3): 352-8.
14. Dentali F., Ageno W., Witt D., Malato A., Clark N., Garcia D., McCool K., Siragusa S., Dyke S., Crowther M. WARPED consortium. Natural history of mesenteric venous thrombosis in patients treated with vitamin K antagonists: a multi-centre, retrospective cohort study. Thromb. Haemost. 2009 Sep; 102 (3): 501-4.
15. Etoh Y., Ohsawa I., Fujita T., Fuke Y., Endo M., Ohi H., Kumasaka K. Nephrotic syndrome with portal, splenic and renal vein thrombosis. A case report. Nephron. 2002; 92 (3): 680-4.
16. Glueck C.J., Bell H., Vadlamani L., Gupta A., Fontaine R.N., Wang P., Stroop D., Gruppo R. Heritable thrombophilia and hypofibrinolysis. Possible causes of retinal vein occlusion. Arch. Ophthalmol. 1999 Jan; 117 (1): 43-9.
17. Glueck C.J., Wang P. Ocular vascular thrombotic events: a diagnostic window to familial thrombophilia (compound factor V Leiden and prothrombin gene heterozygosity) and thrombosis. Clin. Appl. Thromb. Hemost. 2009 Feb; 15 (1): 12-8.
18. Harris R.C., Ismail N. Extrarenal complications of the nephrotic syndrome. Am. J. Kidney Dis. 1994 Apr; 23 (4): 477-97
19. Heller C., Schobess R., Kurnik K., Junker R., Günther G., Kreuz W., Nowak-Göttl U. Abdominal venous thrombosis in neonates and infants: role of prothrombotic risk factors – a multicentre case-control study. For the Childhood Thrombophilia Study Group. Br. J. Haematol. 2000 Nov; 111 (2): 534-9.
20. Janssen H.L., Meinardi J.R., Vleggaar F.P. et al. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. Blood. 2000 Oct 1; 96 (7): 2364-8.
21. Joffe G.M., Aisenbrey G.A., Argubright K.F. Budd-Chiari syndrome, systemic lupus erythematosus, and secondary antiphospholipid antibody syndrome in pregnancy. Obstet Gynecol. 2005 Nov; 106 (5 Pt 2): 1191-4. Erratum in: Obstet Gynecol. 2006 Jan; 107 (1): 209
22. Joffe H.V., Goldhaber S.Z. Upper-extremity deep vein thrombosis. Circulation. 2002 Oct 1; 106 (14): 1874-80.
23. Kitchens K.S., Alving B.M., Kessler C.M. Consultative haemostasis and thrombosis. Elsivier Science. 2002; P. 617.
24. Kitchens G.S., Kessler C.M., Konkle B.A. Consultative hemostasis and thrombosis. 3rd addition. Elsivier. 2013.
25. Klein R., Klein B.E., Moss S.E., Meuer S.M. The epidemiology of retinal vein occlusion: the Beaver Dam Eye Study. Trans. Am. Ophthalmol. Soc. 2000; 98: 133-41.
26. Kucher N. Clinical practice. Deep-vein thrombosis of the upper extremities. N. Engl. J. Med. 2011 Mar 3; 364 (9): 861-9.
27. Kuhle S., Massicotte P., Chan A., Mitchell L. A case series of 72 neonates with renal vein thrombosis. Data from the 1-800-NO-CLOTS Registry. Thromb. Haemost. 2004 Oct; 92 (4): 729-33.
28. Kumar S., Sarr M.G., Kamath P.S. Mesenteric venous thrombosis. N. Engl. J. Med. 2001 Dec 6; 345 (23): 1683-8.
29. Leebeek F.W., Stadhouders N.A., van Stein D., Gómez-García E.B., Kappers-Klunne M.C. Hypercoagulability states in upper-extremity deep venous thrombosis. Am. J. Hematol. 2001 May; 67 (1): 15-9.
30. Linnemann B., Meister F., Schwonberg J., Schindewolf M., Zgouras D., Lindhoff-Last E. MAISTHRO registry. Hereditary and acquired thrombophilia in patients with upper extremity deep-vein thrombosis. Results from the MAISTHRO registry. Thromb Haemost. 2008 Sep; 100 (3): 440-6.
31. Martinelli I., Battaglioli T., Bucciarelli P., Passamonti S.M., Mannucci P.M. Risk factors and recurrence rate of primary deep vein thrombosis of the upper extremities. Circulation. 2004 Aug 3; 110 (5): 566-70.
32. Martinelli I., De Stefano V. Rare thromboses of cerebral, splanchnic and upper-extremity veins. A narrative review. Thromb. Haemost. 2010 Jun; 103 (6): 1136-44.
33. Menon K.V., Shah V., Kamath P.S. The Budd-Chiari syndrome. N. Engl. J. Med. 2004 Feb 5; 350 (6): 578-85.
34. Pabinger I., Schneider B. Thrombotic risk in hereditary antithrombin III, protein C, or protein S deficiency. A cooperative, retrospective study. Gesellschaft fur Thrombose- und Hamostaseforschung (GTH) Study Group on Natural Inhibitors. Arterioscler. Thromb. Vasc. Biol. 1996 Jun; 16 (6): 742-8.
35. Salomon O., Apter S., Shaham D., Hiller N., Bar-Ziv J., Itzchak Y., Gitel S., Rosenberg N., Strauss S., Kaufman N., Seligsohn U. Risk factors associated with postpartum ovarian vein thrombosis. Thromb. Haemost. 1999 Sep; 82 (3): 1015-9.
36. Senzolo M., Cholongitas E.C., Patch D., Burroughs A.K. Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome. Nat. Clin. Pract. Gastroenterol. Hepatol. 2005 Apr; 2 (4): 182-90.
37. Twickler D.M., Setiawan A.T., Evans R.S., Erdman W.A., Stettler R.W., Brown C.E., Cunningham F.G. Imaging of puerperal septic thrombophlebitis: prospective comparison of MR imaging, CT, and sonography. AJR Am. J. Roentgenol. 1997 Oct; 169 (4): 1039-43.
38. Valla D.C. Hepatic vein thrombosis (Budd- Chiari syndrome). Semin. Liver. Dis. 2002 Feb; 22 (1): 5-14.
39. Weber S.M., Rikkers L.F. Splenic vein thrombosis and gastrointestinal bleeding in chronic pancreatitis. World J. Surg. 2003 Nov; 27 (11): 1271-4.
40. Weger M., Stanger O., Deutschmann H., Leitner F.J., Renner W., Schmut O., Semmelrock J., Haas A. The role of hyperhomocysteinemia and methylenetetrahydrofolate reductase (MTHFR) C677T mutation in patients with retinal artery occlusion. Am. J. Ophthalmol. 2002 Jul; 134 (1): 57-61.
41. Witlin A.G., Sibai B.M. Postpartum ovarian vein thrombosis after vaginal delivery: a report of 11 cases. Obstet. Gynecol. 1995 May; 85 (5 Pt 1): 775-80.
42. Zigman A., Yazbeck S., Emil S., Nguyen L. Renal vein thrombosis: a 10-year review. J. Pediatr. Surg. 2000 Nov; 35 (11): 1540-2.
43. Zimmerman M.A., Cameron A.M., Ghobrial R.M. Budd-Chiari syndrome. Clin. Liver. Dis. 2006 May; 10 (2): 259-73
44.
45.
46.
Review
For citation:
Akinshina S.V., Makatsariya A.D. THROMBOSIS OF UNUSUAL SITES AND THROMBOPHILIA. Obstetrics, Gynecology and Reproduction. 2014;8(2):97-111. (In Russ.)
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