Abstract
Purpose
Catastrophic antiphospholipid syndrome (CAPS) is a variant of antiphospholipid syndrome and presents with life-threatening symptoms of multiorgan failure due to thrombosis. We present a patient with CAPS secondary to an ovarian cancer. In such cases, it is believed that the thrombotic risk disappears after surgical removal of the cancer. The intraoperative management was challenging because of the risks of two opposing complications: catastrophic exacerbation of the thrombotic tendency triggered by the surgical stimulus and major bleeding due to the necessary anticoagulation. We describe the intraoperative management of hemostasis in a patient with CAPS.
Clinical features
A 44-yr-old female patient with CAPS underwent resection of an ovarian cancer, which was suspected to be associated with her coagulation abnormality. She had both arterial and venous thromboembolism, including cerebral infarction, embolic gangrene, and pulmonary emboli. Serological examinations revealed increased anticardiolipin IgG antibody and decreased protein C activity. Before surgery, an inferior vena cava filter was placed to prevent perioperative pulmonary embolism. Prostaglandin E1 (PGE1; 100 ng·kg-1·min-1) was given intraoperatively to suppress platelet aggregation and thrombin generation and to maintain arterial blood flow. No apparent coagulation abnormalities were observed during surgery, neither hypercoagulation nor a tendency to bleed. No additional thrombotic symptoms developed during a six-month follow-up.
Conclusion
The use of PGE1, an inhibitor of thrombin formation and platelet function, and placement of an inferior vena cava filter were associated with the uneventful surgical resection of an ovarian cancer in a patient with CAPS.
Résumé
Objectif
Le syndrome antiphospholipidique catastrophique (SAPC), variante du syndrome antiphospholipidique, présente des symptômes graves de défaillance multiorganique causés par une thrombose. Nous présentons un cas de SAPC secondaire à un cancer ovarien. Le traitement peropératoire a été difficile à cause des risques de deux complications opposées: l’exacerbation catastrophique de la tendance à la thrombose déclenchée par le stimulus chirurgical et l’important saignement lié à la nécessaire anticoagulation. Nous décrivons la prise en charge peropératoire de l’hémostase.
Éléments cliniques
Une patiente de 44 ans atteinte du SAPC, qu’on croyait associé à une anomalie de la coagulation, a subi la résection d’un cancer ovarien. Elle présentait une thrombo-embolie artérielle et veineuse, comprenant un infarctus cérébral, de la gangrène embolique et une embolie pulmonaire. Les examens sériques ont révélé une augmentation d’anticorps anticardiolipine IgG et une réduction de l’activité des protéines C. Avant l’opération, un filtre a été placé dans la veine cave inférieure pour prévenir l’embolie pulmonaire périopératoire. De la prostaglandine E1 (PGE1; 100 ng·kg-1min-1) a été administrée pendant l’opération pour supprimer l’agrégation plaquettaire et la génération de thrombine et maintenir le débit sanguin artériel. Aucune anomalie apparente de la coagulation n’a été observée pendant l’opération, ni d’hypercoagulation ou de tendance au saignement. Aucun symptôme thrombotique additionnel ne s’est développé pendant le suivi postopératoire de six mois.
Conclusion
L’usage de PGE1, un inhibiteur de la formation de thrombine et de la fonction plaquettaire, et la mise en place d’un filtre parapluie dans la veine cave inférieure ont été combinés à la résection chirurgicale sans incident d’un cancer ovarien chez une patiente atteinte du SAPC.
Article PDF
Similar content being viewed by others
References
Asherson RA. The catastrophic antiphospholipid syndrome (Editorial). J Rheumatol 1992; 19: 508–12.
Triplett DA, Asherson RA. Pathophysiology of the catastrophic antiphospholipid syndrome (CAPS). Am J Hematol 2000; 65: 154–9.
Shames DS, Broderick PA. Catastrophic antiphospholipid antibody syndrome. Conn Med 2001; 65: 3–5.
Ruffatti A, Aversa S, Del Ross T, Tonetto S, Fiorentino M, Todesco S. Antiphospholipid antibody syndrome associated with ovarian cancer. A new paraneoplastic syndrome? J Rheumatol 1994; 21: 2162–3.
Muir DF, Stevens A, Napier-Hemy RO, Fath-Ordoubadi F, Curzen N. Recurrent stent thrombosis associated with lupus anticoagulant due to renal cell carcinoma. Int J Cardiovasc Intervent 2003; 5: 44–6.
Soltesz P, Szekanecz Z, Vegh J, et al. Catastrophic antiphospholipid syndrome in cancer. Haematologia (Budap) 2000; 30: 303–11.
Rickles FR, Edwards RL. Activation of blood coagulation in cancer: Trousseau’s syndrome revisited. Blood 1983; 62: 14–31.
Menon G, Allt-Graham J. Anaesthetic implications of the anti-cardiolipin antibody syndrome. Br J Anaesth 1993; 70: 587–90.
Yamamoto T, Ito M, Nagata S, et al. Catastrophic exacerbation of antiphospholipid syndrome after lung adenocarcinoma biopsy. J Rheumatol 2000; 27: 2035–7.
Erkan D, Yazici Y, Peterson MG, Sammaritano L, Lockshin MD. A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome. Rheumatology (Oxford) 2002; 41: 924–9.
Langer F, Eifrig B, Marx G, Stork A, Hegewisch-Becker S, Hossfeld DK. Exacerbation of antiphospholipid antibody syndrome after treatment of localized cancer: a report of two cases. Ann Hematol 2002; 81: 727–31.
Madan R, Khoursheed M, Kukla R, Al-Mazidi M, Behbehani A. The anaesthetist and the antiphospholipid syndrome. Anaesthesia 1997; 52: 72–6.
Golden BD, Belmont HM. The role of microvasculopathy in the catastrophic antiphospholipid syndrome: comment on the article by Neuwelt et al (Letter). Arthritis Rheum 1998; 41: 751–3; author reply 753-4, 739.
Permpikul P, Rao LV, Rapaport SI. Functional and binding studies of the roles of prothrombin and beta 2-glycoprotein I in the expression of lupus anticoagulant activity. Blood 1994; 83: 2878–92.
Oosting JD, Preissner KT, Derksen RH, de Groot PG. Autoantibodies directed against the epidermal growth factor-like domains of thrombomodulin inhibit protein C activation in vitro. Br J Haematol 1993; 85: 761–8.
Ozaki M, Minami K, Shigematsu A. Myocardial ischemia during emergency anesthesia in a patient with systemic lupus erythematosus resulting from undiagnosed antiphospholipid syndrome (Letter). Anesth Analg 2002; 95: 255.
Petrovich CT. Hemostasis and hemotherapy.In:Barash PG, Cullen BF, Stoelting R (Eds). Clinical Anesthesia, 3rd ed. Philadelphia, PA: J.B. Lippincott Company; 1996: 189–217.
Morrow JD, Roberts LJ II. Lipid derived autacoids. Eicosanoids and platelet-activating factor.In:Hardman JD, Limbird LE (Eds). Goodman and Gilman’s The Pharmacological Basis of Therapeutics, 10th ed. New York, NY: McGraw-Hill Companies Inc.; 2001: 669–85.
Goto F, Otani E, Kato S, Fujita T. Prostaglandin E1 as a hypotensive drug during general anaesthesia. Anaesthesia 1982; 37: 530–5.
Yukioka H, Asada K, Fujimori M, Shimazu A. Prostaglandin E1 as a hypotensive drug during general anesthesia for total hip replacement. J Clin Anesth 1993; 5: 310–4.
Kozek-Langenecker SA, Wanzel O, Berger R, Kettner SC, Coraim F. Increased anticoagulation during cardiopulmonary bypass by prostaglandin E1. Anesth Analg 1998; 87: 985–8.
Abe K, Demizu A, Kamada K, Morimoto T, Sakaki T, Yoshiya I. Local cerebral blood flow with prostaglandin E1 or trimethaphan during cerebral aneurysm clip ligation. Can J Anaesth 1991; 38: 831–6.
Suzuki H, Asada M, Tateyama T, et al. Myocardial metabolism, oxygen demand and oxygen supply during prostaglandin E1 induced hypotension (Japanese). Masui 1994; 43: 680–3.
Koga T, Az-ma T, Yuge O. Prostaglandin E1 at clinically relevant concentrations inhibits aggregation of platelets under synergic interaction with endothelial cells. Acta Anaesthesiol Scand 2002; 46: 987–93.
Seto A, Fukuyama H, Niijima K, Takenaka I, Kadoya T. Anesthetic management of a patient with deep venous thrombosis using temporary inferior vena cava filter (Japanese). Masui 2000; 49: 302–4.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ozaki, M., Ogata, M., Yokoyama, T. et al. Prevention of thrombosis with prostaglandin E1 in a patient with catastrophic antiphospholipid syndrome. Can J Anaesth 52, 143–147 (2005). https://doi.org/10.1007/BF03027719
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF03027719