5. Treatment


The patient was kept on 90 mg enoxaparin (Lovenox), which was administered subcutaneously once a day through the diagnostic phase. Mr. H was also referred to a vascular surgeon. After a two-month interval, the patient returned to the hematology clinic.

Mr. H had been noncompliant with his enoxaparin treatments and did not follow up with the vascular surgeon. At that time, a repeat ultrasound was ordered, and a persistent partial thrombosis of the subclavian, axillary and basilica veins was noted.




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6. Discussion


Venous thromboembolic disease is the most common manifestation of antiphospholipid antibody syndrome (APS). APS is characterized by arterial and venous thrombosis, with the presence of antiphospholipid antibodies or positive lupus anticoagulant.1 The deep veins of the extremities are most often affected, followed by axillary, retinal, hepatic and cerebral sinus vessels.2

It is common for antiphospholipid antibodies to be seen in conjunction with other autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. This patient’s condition was complicated by the concurrent diagnosis of thoracic outlet syndrome (TOS). TOS is a cause of neck and shoulder pain with varying etiologies, primarily neurological from nerve or vascular compression with related edema and cyanosis of the limb.3 Most cases of TOS are treated with physical or occupational therapy, with palliative and supportive treatments.3 It is unlikely that TOS was the cause of these thrombolytic events in this patient, but rather was confounded by the APS.


The diagnostic testing performed to confirm APS includes immune and coagulation studies—specifically lupus anticoagulant (LA), IgG or IgM cardiolipin antibodies, or IgG or IgM anti-beta-2 glucoproteins.2 Most patients with laboratory findings of LA do not have SLE. The dRVVT is a test to detect LA, and is prolonged if LA antibodies are identified in the serum.4 Serial testing is encouraged after 12 weeks to confirm the diagnosis of APS. 


Treatment can be individualized based upon symptomatology and gender, as APS results in high pregnancy-morbidity and fetal mortality. Asymptomatic patients with positive laboratory tests may not require any specific treatment. Patients with thrombolytic events or obstetric complications are treated with anticoagulant therapy.

Because recurrence of venous or arterial thrombosis can be as high as 70%, long term anticoagulant therapy is indicated.1,2 Oral anticoagulants are the best available and most effective treatments for the prevention of recurrent venous or arterial thrombosis. Long-term anticoagulant therapy is recommended to prolong the international normalized ratio to 2.0-3.0.5 


Low-molecular-weight heparin in combination with low-dose aspirin is recommended to avoid pregnancy loss in women with APS. Steroids or intravenous immunoglobulin are also known to be effective in those who have an increase in bleeding or who require surgery.2 



7. Summary


As individual diagnoses, APS and TOS can be managed with minimal long-term sequelae for the patient with appropriate pharmacologic and therapeutic maintenance interventions. Multiorgan failure secondary to widespread thrombotic disease known as catastrophic APS (CAPS) is a risk for any patient with APS due to the effects on the microvasculature system, and is a rare and serious complication.6 Patients diagnosed with CAPS require intensive treatment with corticosteroids, immunosuppression, intravenous IgG, and/or plasma exchange.2

Due to his lack of compliance with the medication regimen and specialty follow-up, Mr. H has been made aware of these risks. In addition, he was counseled to follow up appropriately going forward.  


Margaret Quinn, DNP, CPNP, is a clinical assistant professor at Rutgers College of Nursing in New Brunswick, N.J.


References


  1. Acikel S, Akdemir R, Dogan M, et al. Antiphospholipid syndrome: 
coexistence of left ventricular apical thrombosis and deep vein thrombosis causing pulmonary thromboembolism in a patient with systemic lupus erythematosus. Echocardiography. 2010;27:198-201.

  2. Sangle NA, Smock KJ. Antiphospholipid antibody syndrome. Arch Pathol Lab Med. 2011;135:1092-1096. Available at www.archivesofpathology.org/doi/full/10.5858/2010-0325-RSR.1.

  3. Laulan J, Fouquet B, Rodaix C, et al. Thoracic outlet syndrome: definition, aetiological factors, diagnosis, management and occupational impact. J Occup Rehabil. 2011;21:366-373. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC3526474/.

  4. Sood R, ed. Textbook of Medical Laboratory Technology. London, England: Jaypee Brothers Medical Publishers; 2006:296-307.

  5. Horner KE, Phillips BB, Newkirk E, et al. Evaluation of anticoagulation in patients with antiphospholipid syndrome. Am J Health Syst Pharm. 2008;65:964-967.

  6. Katikireddi VS, Kandiah DA. Progression of antiphospholipid antibody syndrome to catastrophic antiphospholipid antibody syndrome acutely with cessation of antithrombotic therapy. Intern Med J. 2012;42:585-591.


All electronic documents accessed October 15, 2013.