Risk of recurrence of unusual site venous thromboembolism

Summary

The term unusual site venous thrombosis defines uncommon clinical manifestations of venous thromboembolism occurring in sites different from the lower limbs or the lungs, with peculiar pathophysiological features and clinical history. Information on long-term outcomes of unusual site thrombosis is generally scant, because most studies are small and usually retrospective.

Recurrence rate of cerebral vein thrombosis is about 2/100 patient-years; the only identified predisposing factors have been male gender and personal history of thrombosis. Retinal vein occlusion showed a recurrence in the same eye of 2.5% and in the fellow eye of 11.9% within four years. Hypercholesterolemia, hypertriglyceridaemia and hyperhomocysteinaemia were significantly associated with recurrent events. Recurrence rates of splanchnic vein thrombosis are difficult to estimate given the heterogeneity of patient populations; higher recurrence rates are reported in the cirrhotic population (from 27% to 38.5%). Hormone therapy, myeloproliferative neoplasm or other prothrombotic states, and absence of anticoagulant therapy emerged as independent prognostic factors. Future studies should aim at better assessing the risk of recurrence in different patients subgroups and at identifying more accurate prognostic markers.

Zusammenfassung

Die Bezeichnung Venenthrombosen an ungewöhnlicher Lokalisation steht für Manifestationen venöser Thromboembolien, die außerhalb von den unteren Extremitäten oder der Lunge auftreten, und besondere pathophysiologische Eigenschaften und eine spezielle Anamnese aufweisen. Langzeitdaten über den klinischen Verlauf von Venenthrombosen an ungewöhnlicher Lokalisation sind im Allgemeinen sehr spärlich, da es sich oft um wenig umfangreiche und meistens retrospektive Studien handelt Die Rezidivrate zerebraler Venenthrombosen liegt bei ungefähr 2 pro 100 Patientenjahre; die einzigen identifizierten Prädispositionsfaktoren sind männliches Geschlecht und Thrombose in der Vorgeschichte. Bei retinalen Venenverschlüssen wurden in vier Jahren bei 2.5% Rezidive im selben Auge und bei 11.9% Rezidive im anderen Auge beobachtet. Ein signifikanter Zusammenhang bestand zwischen Rezidiven und Hypercholesterinämie, Hypertriglyzeridämie und Hyperhomocysteinämie. Die Rezidivraten splanchnischer Venenthrombosen sind schwer einzuschätzen, da die Patienten sehr heterogen sind; bei Patienten mit Zirrhose werden höhere Rezidivraten berichtet (27–38,5%). Als unabhängige prognostische Faktoren kristallisierten sich Hormontherapien, myeloproliferative Neoplasien oder andere prothrombotische Zustände sowie fehlende Antikoagulationstherapie heraus. Ziel künftiger Studien sollte sein, das Rezidivrisiko in verschiedenen Patientengruppen besser einzuschätzen und präzise Prognosemarker zu identifizieren.

Keywords

Recurrence - cerebral vein thrombosis - retinal vein occlusion - splanchnic vein thrombosis

Schlüsselwörter

Rezidiv - zerebrale Venenthrombose - retinaler Venenverschluss - splanchnische Venenthrombose

• References

• 1 Iorio A, Kearon C, Filippucci E. et al. Risk of recurrence after a first episode of symptomatic venous thromboembolism provoked by a transient risk factor: a systematic review. Arch Intern Med 2010; 170: 1710-1716.
• 2 Baglin T, Luddington R, Brown K, Baglin C. Incidence of recurrent thromboembolism in relation to clinical and thrombophilic risk factors: prospective cohort study. Lancet 2003; 362: 523-526.
• 3 Kearon C, Akl EA, Comerota AJ. et al. American College of Chest Physicians. Antithrombotic therapy for VTE disease: antithrombotic therapy and prevention of thrombosis, 9th ed. American College of Chest Physicians evidence-based clinical practice guidelines. Chest 2012; 141 (Suppl. 02) e419S-e494S.
• 4 Rodger MA, Kahn SR, Wells PS. et al. Identifying unprovoked thromboembolism patients at low risk for recurrence who can discontinue anticoagulant therapy. CMAJ 2008; 179: 417-426.
• 5 Eichinger S, Heinze G, Jandeck LM, Kyrle PA. Risk assessment of recurrence in patients with unprovoked deep vein thrombosis or pulmonary embolism: the Vienna prediction model. Circulation 2010; 121: 1630-1636.
• 6 Tosetto A, Iorio A, Marcucci M. et al. Predicting disease recurrence in patients with previous unprovoked venous thromboembolism: a proposed prediction score (DASH). J Thromb Haemost 2012; 10: 1019-1025.
• 7 Dentali F, Ageno W. Cerebral vein thrombosis. Intern Emerg Med 2010; 05: 27-32.
• 8 Dentali F, Squizzato A, Marchesi C. et al. D-dimer testing in the diagnosis of cerebral vein thrombosis: a systematic review and a meta-analysis of the literature. J Thromb Haemost 2012; 10: 582-589.
• 9 Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005; 352: 1791-1798.
• 10 Martinelli I, De Stefano V. Rare thromboses of cerebral, splanchnic and upper-extremity veins. A narrative review. Thromb Haemost 2010; 103: 1136-1144.
• 11 Dentali F, Crowther M, Ageno W. Thrombophilic abnormalities, oral contraceptives, and risk of cerebral vein thrombosis: a meta-analysis. Blood 2006; 107: 2766-2773.
• 12 Passamonti SM, Biguzzi E, Cazzola M. et al. The JAK2 V617F mutation in patients with cerebral venous thrombosis. J Thromb Haemost 2012; 10: 998-1003.
• 13 Bellucci S, Cassinat B, Bonnin N. et al. The V617F JAK 2 mutation is not a frequent event in patients with cerebral venous thrombosis without overt chronic myeloproliferative disorder. Thromb Haemost 2008; 99: 1119-1120.
• 14 Dentali F, Gianni M, Crowther MA, Ageno W. Natural history of cerebral vein thrombosis: a systematic review. Blood 2006; 108: 1129-1134.
• 15 Sacco RL, Adams R, Albers G. et al. Guidelines for prevention of stroke in patients with ischemic stroke or transient ischemic attack: a statement for healthcare professionals from the American Heart Association/American Stroke Association Council on Stroke: co-sponsored by the Council on Cardiovascular Radiology and Intervention: the American Academy of Neurology affirms the value of this guideline. Circulation 2006; 113: e409-e449.
• 16 Einhäupl K, Stam J, Bousser MG. et al. European Federation of Neurological Societies. EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. Eur J Neurol 2010; 17: 1229-1235.
• 17 Ageno W, Dentali F, Squizzato A. et al. Evidence and clinical judgment: Treatment of cerebral vein thrombosis. Thromb Haemost 2010; 103: 1109-1115.
• 18 Preter M, Tzourio C, Ameri A, Bousser MG. Longterm prognosis in cerebral venous thrombosis. Follow-up of 77 patients. Stroke 1996; 27: 243-246.
• 19 Ferro JM, Canhão P, Stam J. ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004; 35: 664-670.
• 20 Gosk-Bierska I, Wysokinski W, Brown Jr RD. et al. Cerebral venous sinus thrombosis: Incidence of venous thrombosis recurrence and survival. Neurology 2006; 67: 814-819.
• 21 Martinelli I, Bucciarelli P, Passamonti SM. et al. Long-term evaluation of the risk of recurrence after cerebral sinus-venous thrombosis. Circulation 2010; 121: 2740-2746.
• 22 Dentali F, Poli D, Scoditti U. et al. CErebral Vein Thrombosis International Study Investigators. Long-term outcomes of patients with cerebral vein thrombosis: a multicenter study. J Thromb Haemost 2012; 10: 1297-1302.
• 23 Hayreh SS. Occlusion of the central retinal vessels. Br J Ophthalmol 1965; 49: 626-645.
• 24 Cheung N, Klein R, Wang JJ. et al. Traditional and novel cardiovascular risk factors for retinal vein occlusion: the multi-ethnic study of atherosclerosis. Invest Ophthalmol Vis Sci 2008; 49: 4297-4302.
• 25 Hayreh SS, Zimmerman MB, Podhajsky P. Incidence of various types of retinal vein occlusion and their recurrence and demographic characteristics. Am J Ophthalmol 1994; 117: 429-441.
• 26 Rogers S, McIntosh RL, Cheung N. et al. International Eye Disease Consortium. The prevalence of retinal vein occlusion: pooled data from population studies from the United States, Europe, Asia, and Australia. Ophthalmology 2010; 117: 313-319.
• 27 The Central Vein Occlusion Study Group. Baseline and early natural history report. Arch Ophthalmol 1993; 111: 1087-1095.
• 28 Yau JW, Lee P, Wong TY. et al. Retinal vein occlusion: an approach to diagnosis, systemic risk factors and management. Intern Med J 2008; 38: 904-910.
• 29 Marcucci R, Sofi F, Grifoni E. et al. Retinal vein occlusions: a review for the internist. Intern Emerg Med 2011; 06: 307-314.
• 30 Hayreh SS. Prevalent misconceptions about acute retinal vascular occlusive disorders. Prog Retin Eye Res 2005; 24: 493-519.
• 31 Cole MD, Dodson PM, Hendeles S. Medical conditions underlying retinal vein occlusion in patients with glaucoma or ocular hypertension. Br J Ophthalmol 1989; 73: 693-698.
• 32 Dodson PM, Kritzinger EE, Clough CG. Diabetes mellitus and retinal vein occlusion in patients of Asian, West Indian and White European origin. Eye (Lond) 1992; 06: 66-68.
• 33 Eye Disease Case-Control Study Group. Risk factors for central retinal vein occlusion. Arch Ophthalmol 1996; 114: 545-554.
• 34 Eye Disease Case-Control Study Group. Risk factors for branch retinal vein occlusion. Am J Ophthalmol 1993; 116: 286-296.
• 35 Hayreh SS, Zimmerman B, McCarthy MJ, Podhajsky P. Systemic diseases associated with various types of retinal vein occlusion. Am J Ophthalmol 2001; 131: 61-77.
• 36 Klein R, Klein BE, Moss SE, Meuer SM. The epidemiology of retinal vein occlusion: the Beaver Dam Eye Study. Trans Am Ophthalmol Soc 2000; 98: 133-141.
• 37 McGrath MA, Wechsler F, Hunyor AB, Penny R. Systemic factors contributory to retinal vein occlusion. Arch Intern Med 1978; 138: 216-220.
• 38 Janssen MC, den Heijer M, Cruysberg JR. et al. Retinal vein occlusion: a form of venous thrombosis or a complication of atherosclerosis? A meta-analysis of thrombophilic factors. Thromb Haemost 2005; 93: 1021-1026.
• 39 Glacet-Bernard A, Coscas G, Chabanel A. et al. Prognostic factors for retinal vein occlusion: prospective study of 175 cases. Ophthalmology 1996; 103: 551-560.
• 40 Rehak J, Dusek L, Chrapek O. et al. Initial visual acuity is an important prognostic factor in patients with branch retinal vein occlusion. Ophthalmic Res 2011; 45: 204-209.
• 41 Michels RG, Gass JD. The natural course of retinal branch vein obstruction. Trans Am Acad Ophthalmol Otolaryngol 1974; 78: OP166-OP177.
• 42 Rogers SL, McIntosh RL, Lim L. et al. Natural history of branch retinal vein occlusion: an evidencebased systematic review. Ophthalmology 2010; 117: 1094-1101.
• 43 McIntosh RL, Rogers SL, Lim L. et al. Natural history of central retinal vein occlusion: an evidencebased systematic review. Ophthalmology 2010; 117: 1094-1123.
• 44 Sodi A, Giambene B, Marcucci R. et al. Atherosclerotic and thrombophilic risk factors in patients with recurrent central retinal vein occlusion. Eur J Ophthalmol 2008; 18: 233-238.
• 45 Cugati S, Wang JJ, Knudtson MD. et al. Retinal vein occlusion and vascular mortality: pooled data analysis of 2 population-based cohorts. Ophthalmology 2007; 114: 520-524.
• 46 Khan Z, Almeida DR, Rahim K. et al. 10-Year Framingham risk in patients with retinal vein occlusion: a systematic review and meta-analysis. Can J Ophthalmol 2013; 48: 40-45.
• 47 De Stefano V, Martinelli I. Splanchnic vein thrombosis: clinical presentation, risk factors and treatment. Intern Emerg Med 2010; 05: 487-494.
• 48 Almdal TP, Sorensen TI. Incidence of parenchymal liver diseases in Denmark, 1981 to 1985: analysis of hospitalization registry data. The Danish Association for the Study of the Liver. Hepatology 1991; 13: 650-655.
• 49 Ögren M, Bergqvist D, Björck M. et al. Portal vein thrombosis: Prevalence, patient characteristics and lifetime risk: A population study based on 23 796 consecutive autopsies. World J Gastroenterol 2006; 12: 2115-2119.
• 50 Acosta S, Alhadad A, Svensson P, Ekberg O. Epidemiology, risk and prognostic factors in mesenteric venous thrombosis. Br J Surg 2008; 95: 1245-1251.
• 51 Rajani R, Melin T, Björnsson E. et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18-year experience. Liver Int 2009; 29: 253-259.
• 52 Valla D. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol 2004; 19: S204-S211.
• 53 Thatipelli MR, McBane RD, Hodge DO, Wysokinski WE. Survival and recurrence in patients with splanchnic vein thromboses. Clin Gastroenterol Hepatol 2010; 08: 200-205.
• 54 Ageno W, Squizzato A, Togna A. et al. Incidental diagnosis of a deep vein thrombosis in consecutive patients undergoing a computed tomography scan of the abdomen: a retrospective cohort study. J Thromb Haemost 2012; 10: 158-160.
• 55 Ikeda M, Sekimoto M, Takiguchi S. et al. Total splenic vein thrombosis after laparoscopic splenectomy: a possible candidate for treatment. Am J Surg 2007; 193: 21-25.
• 56 Kralovics R, Passamonti F, Buser AS. et al. A gainof-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med 2005; 352: 1779-1790.
• 57 Vannucchi AM. JAK2 mutation and thrombosis in the myeloproliferative neoplasms. Curr Hematol Malig Rep 2010; 05: 22-28.
• 58 Dentali F, Squizzato A, Brivio L. et al. JAK2V617F mutation for the early diagnosis of Phmyeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis. Blood 2009; 113: 5617-5623.
• 59 Dentali F, Galli M, Gianni M, Ageno W. Inherited thrombophilic abnormalities and risk of portal vein thrombosis. A meta-analysis. Thromb Haemost 2008; 99: 675-682.
• 60 Janssen HL, Meinardi JR, Vleggaar FP. 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; 96: 2364-2368.
• 61 De Stefano V, Za T, Ciminello A. et al. Causes of adult splanchnic vein thrombosis in the mediterranean area. Mediterr J Hematol Infect Dis 2011; 03: e2011063.
• 62 Van Bijnen ST, Van Heerde WL, Muus P. Mechanisms and clinical implications of thrombosis in paroxysmal nocturnal hemoglobinuria. J Thromb Haemost 2012; 10: 1-10.
• 63 Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd-Chiari syndrome: a common complication of Behçet’s disease. Am J Gastroenterol 1997; 92: 858-862.
• 64 Amitrano L, Guardascione MA, Scaglione M. et al. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol 2007; 102: 2464-2470.
• 65 Condat B, Pessione F, Hillaire S. et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001; 120: 490-497.
• 66 Amitrano L, Guardascione MA, Menchise A. et al. Safety and efficacy of anticoagulation therapy with low molecular weight heparin for portal vein thrombosis in patients with liver cirrhosis. J Clin Gastroenterol 2010; 44: 448-451.
• 67 Delgado MG, Seijo S, Yepes I. et al. Efficacy and safety of anticoagulation on patients with cirrhosis and portal vein thrombosis. Clin Gastroenterol Hepatol 2012; 10: 776-783.
• 68 Senzolo MM, Sartori T, Rossetto V. et al. Prospective evaluation of anticoagulation and transjugular intrahepatic portosystemic shunt for the management of portal vein thrombosis in cirrhosis. Liver Int 2012; 32: 919-927.
• 69 Dentali F, Ageno W, Witt D. et al. WARPED consortium. Natural history of mesenteric venous thrombosis in patients treated with vitamin K antagonists: a multi-centre, retrospective cohort study. Thromb Haemost 2009; 102: 501-504.
• 70 Darwish SMurad, Plessier A, Hernandez-Guerra M. et al. EN-Vie (European Network for Vascular Disorders of the Liver). Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151: 167-175.
• 71 Cruz E, Ascher NL, Roberts JP. et al. High incidence of recurrence and hematologic events following liver transplantation for Budd-Chiari syndrome. Clin Transplant 2005; 19: 501-506.