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Abstract
Antiphospholipid syndrome (APS) is an autoimmune multisystemic disease characterized by the presence of persistent laboratory evidence of antiphospholipid antibodies associated to clinical manifestations(1).
The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) requires both of the
following criteria(2): - pulmonary hypertension must be present, defined as a mean pulmonary arterial pressure (mPAP) >20 mmHg at rest and the pulmonary vascular resistance is ≥3 Woods units in the absence of an elevated pulmonary capillary wedge pressure (ie, PCWP is ≤15 mmHg).
- thromboembolic occlusion of the proximal or distal pulmonary vasculature must exist and be the presumed cause of the pulmonary hypertension.
Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of exposure to heparin (unfractionated heparin or low molecular weight heparin) that occurs in a small percentage of patients exposed, regardless of the dose, schedule, or route of administration. HIT results from an autoantibody directed against endogenous platelet factor 4 (PF4) in complex with heparin. This antibody activates platelets and can cause catastrophic arterial or venous thrombosis with a mortality rate as high as 20 percent; although with improved recognition and early intervention, mortality rate has been reported as below 2 percent.
We present the case of a 41 years old female patient from Peru. She was sent to our Hospital to realize thromboendarterectomy because of chronic throm boembolic pulmonary hypertension secondary to
antiphospholipid syndrome. She was anticoagulated with anti-vitamin K anticoagulants. At hospital this was changed to unfractionated heparin; and at the fifth day she developed HIT. Because of this, the treatment was rotated to bivalirudin, with TT controls. The surgery was postponed and she left the institution with oral anticoagulation.
Because of the lack of bivalirudin in our country, in a multidisciplinary discussion, we decided to utilize unfractionated heparin only during the extracorporeal circulation bomb. After the surgery, we used the few blisters of bivalirudin left in Argentine.
References
2. Simonneau G, Montani D, Celermajer DS et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan 24;53(1).
3. Cervera R, Piette J, Font J et al. Antiphospholipid Syndrome Clinical and Immunologic Manifestations and Patterns of Disease Expression in a Cohort of 1,000 Patients. Arthritis and Rheumatism. 2002 April;46(4):1019-1027.
4. Ruiz-Irastorza G, Crowther M, Branch W. Antiphospholipid syndrome. Lancet. 2010;376(9751):1498.
5. Tektonidou MG, Varsou N, Kotoulas G et al. Cognitive deficits in patients with antiphospholipid syndrome: association with clinical, laboratory, and brain magnetic resonance imaging findings. Arch Intern Med. 2006;166(20):2278.
6. Abreu MM, Danowski A, Wahl DG et al.The relevance of "non-criteria" clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force
Report on Antiphospholipid Syndrome Clinical Features. Autoimmun Rev. 2015 May;14(5):401-14.
7. Galli M, Luciani D, Bertolini G et al. Lupus anticoagulants are stronger risk factors for thrombosis than
anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood.
2003 Mar 1;101(5):1827-32.
8. Cervera R, Serrano R, Pons-Estel GJ et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2015 Jun;74(6):1011-8.
9. Pengo V, Ruffatti A, Legnani C et al. Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost. 2010;8(2):237.
10. Kearon C, Parpia S, Spencer FA et al. Antiphospholipid antibodies and recurrent thrombosis after a first unprovoked venous thromboembolism. Blood. 2018;131(19):2151.
11. Forastiero R, Martinuzzo M, Pombo G et al. A prospective study of antibodies to beta2-glycoprotein I
and prothrombin, and risk of thrombosis. J Thromb Haemost. 2005 Jun;3(6):1231-8.
12. Ruffatti A, Tonello M, Del Ross T et al. Antibody profile and clinical course in primary antiphospholipid syndrome with pregnancy morbidity. Thromb Haemost. 2006 Sep;96(3):337-41.
13. Khamashta MA, Cuadrado MJ, Mujic F et al. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med. 1995;332(15):993.
14. Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A et al. Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature. Eur Respir J. 2017;49(2).
15. Lang IM, Pesavento R, Bonderman D et al. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: a current understanding. Eur Respir J. 2013;41(2):462.
16. Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364(4):351.
17. Neal D, Jonathan H, Milo E et al. Pulmonary Endarterectomy Under Hypothermic Circulatory Arrest in
a Patient With Heparin-Induced Thrombocytopenia. J Cardiothorac Vasc Anesth. 2016 Jun;30(3):741-5.
18. Warkentin TE, Anderson JA. How I treat patients with a history of heparin-induced thrombocytopenia. Blood. 2016;128(3):348.
19. Pötzsch B, Klövekorn WP, Madlener K. Use of heparin during cardiopulmonary bypass in patients with a
history of heparin-induced thrombocytopenia. N Engl J Med. 2000;343(7):515.
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