Tuberc Respir Dis.  2014 Dec;77(6):274-278. 10.4046/trd.2014.77.6.274.

A Case of Antiphospholipid Syndrome Refractory to Secondary Anticoagulating Prophylaxis after Deep Vein Thrombosis-Pulmonary Embolism

Affiliations
  • 1Division of Allergy and Pulmonary Medicine, Department of Internal Medicine, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea. iwpark@cau.ac.kr

Abstract

Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by a combination of clinical criteria, including vascular thrombosis or pregnancy morbidity and elevated antiphospholipid antibody titers. It is one of the causes of deep vein thrombosis and pulmonary embolism that can be critical due to the mortality risk. Overall recurrence of thromboembolism is very low with adequate anticoagulation prophylaxis. The most effective treatment to prevent recurrent thrombosis is long-term anticoagulation. We report on a 17-year-old male with APS, who manifested blue toe syndrome, deep vein thrombosis, pulmonary thromboembolism, and cerebral infarction despite adequate long-term anticoagulation therapy.

Keyword

Antiphospholipid Syndrome; Venous Thrombosis; Pulmonary Embolism; Cerebrovascular Disorders; Blue Toe Syndrome

MeSH Terms

Adolescent
Antibodies, Antiphospholipid
Antiphospholipid Syndrome*
Blue Toe Syndrome
Cerebral Infarction
Cerebrovascular Disorders
Embolism*
Humans
Male
Mortality
Pregnancy
Pulmonary Embolism
Recurrence
Thromboembolism
Thrombosis
Veins*
Venous Thrombosis
Antibodies, Antiphospholipid

Figure

  • Figure 1 (A) Doppler ultrasonography (US). Uncompressed distal superficial femoral and popliteal veins with internal iso- to hyperechoic material. (B) No blood signal or flow was detected on Doppler US, suggesting a deep vein thrombosis.

  • Figure 2 A lung perfusion scan was performed, and large-size perfusion defects were found in the anterior segment of the right upper lobe and superior and inferior segments of the left upper lobe. ANT: anterior; POST: posterior; LT: left; RT: right; LAT: lateral; RPO: right posterior oblique; LPO: left posterior oblique; RAO: right anterior oblique; LAO: left anterior oblique.

  • Figure 3 (A) A lesion was detected in the left lower lobar pulmonary artery on a chest computed tomography (CT) scan taken 11 months after discharge, which may have been the remaining thrombus. (B) No evidence of deep vein thrombosis or pulmonary thromboembolism was detected on a CT scan 5 years after anticoagulation therapy was initiated.

  • Figure 4 Brain magnetic resonance image. Hyper-intense foci are seen in the left centrum semiovale on T2-weighted image (A) and diffusion-weighted images (B).


Reference

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