|LETTER TO EDITOR
|Year : 2020 | Volume
| Issue : 3 | Page : 264-265
The challenge of concurrent thrombosis and hemorrhage in catastrophic antiphospholipid syndrome
Muneerah M ALBugami
Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
|Date of Submission||26-Jul-2020|
|Date of Acceptance||06-Oct-2020|
|Date of Web Publication||04-Dec-2020|
Muneerah M ALBugami
Department of Medicine, King Faisal Specialist Hospital and Research Centre, P.O. 3354, Riyadh 11211
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
ALBugami MM. The challenge of concurrent thrombosis and hemorrhage in catastrophic antiphospholipid syndrome. Saudi J Health Sci 2020;9:264-5
To the Editor,
Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening autoimmune disease. CAPS occurs in 1% of patients with antiphospholipid syndrome, and it is associated with a high mortality (50%)., It is characterized by disseminated intravascular thrombosis causing multiorgan failure due to positive antiphospholipid antibodies.
We present the case of a 64-year-old male who had hypertension and diabetes. He had unprovoked left lower limb deep-venous thrombosis (DVT), which was treated with apixaban 5 mg twice daily. One week later, he had acute renal failure. His creatinine was 365 μmol/L (74.3–107) with normal electrolytes. His partial thromboplastin time was 139.7 s (60–70), prothrombin time was 26.2 s (11–13.5), and international normalized ratio was 2.4 (0.8–1.1). He had normal platelet count and hepatic profile. D-dimer was 3.6 μg/mL (<0.4 μg/mL) and fibrinogen was 9.2 g/l (1.5–4.5). The patient was treated with intravenous heparin (IH). He had a sudden onset of numbness and weakness of the upper and lower limbs with hypoxia and hypotension. He was intubated and started on mechanical ventilation, high-dose inotropic support, hemodialysis, and broad-spectrum antibiotics. All his septic workup were negative. Magnetic resonance imaging and magnetic resonance angiography brain showed multiple diffuse bilateral arterial infarctions. Echocardiography showed thrombosis at the mitral valve. Antiphospholipid antibodies and lupus anticoagulant came positive. He had primary CAPS involving the kidneys, brain, and heart in less than a week with lower-limb DVT. He was treated with intravenous immunoglobulin (IVIG), intravenous dexamethasone for 5 days, and then prednisone 60 mg daily with hydroxychloroquine. His platelets dropped to 40 (150,000–450,000/μL). Heparin-induced thrombocytopenia study was negative. He had pericardial effusion, and hemorrhagic pericardial fluid (350 mL) was removed. Bronchoscopy showed diffuse alveolar hemorrhage, which is the rarest pulmonary complication of CAPS with high mortality risk. He had fast atrial fibrillation which was treated with amiodarone. He was on high-dose glucocorticoids, IVIG, rituximab, hemodialysis, and platelet transfusion, as needed. Due to the benefit of anticoagulation outweighing the bleeding risk, the patient was started on IH to keep the anti-Xa level between 0.3 and 0.7. The outcome was renal, hematological, and cardiopulmonary recovery over 8 weeks of management. He was off hemodialysis.
This rare case highlights the challenge to manage concurrent thrombosis and hemorrhage with profound thrombocytopenia in CAPS. Rapid early diagnosis and an aggressive therapy lead to complete recovery. Physicians need to recognize this syndrome in a patient presenting with multiorgan failure.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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