Case 2
A 9-year-old female with a past medical history of nutritional iron deficiency anemia presented to the emergency department with a three week history of subjective fevers, diarrhea, three kilogram weight loss, and abdominal pain. Upon initial presentation, she was febrile, tachycardic, and had right upper quadrant abdominal pain.
Initial lab work was significant for leukocytosis of 23.4 x103/uL, CRP: 152 mg/L and ESR: 47 mm/hr. She had a negative SARS-CoV-2 via RT-PCR nasal swab and a positive anti-SARS-CoV-2 N antibodies. The evaluation for autoimmune, viral infections, acetaminophen level were negative. D-dimer was elevated at 8.5ug/ml, fibrinogen: 424 mg/dl, ferritin: 617 ng/mL. Her liver chemistry panel had normal transaminases but a low albumin at 2.3mg/dL. Abdominal computed tomography (CT) was performed and showed scattered clusters of low-attenuation areas in both liver lobes and opacification of the right and left portal vein, suggestive of possible thrombosis.
Further imaging with MRI MRCP with and without contrast revealed a 1.4 cm inflammatory focus in the periphery of the right hepatic lobe concerning for an abscess as well as diffuse right portal vein thrombosis. Vancomycin and ceftriaxone were initiated and a 7 fr drain was placed by the interventional radiology team in the right-sided liver abscess. Twenty cc of frank pus was drained, which subsequently grewStreptococcus constellatus . Attempt at intervening on the focus on the left lobe did not yield drainable fluid collection. Immune deficiency workup was performed and negative, including a negative CGD test (Dihydrorhodamine Flow Cytometric Test).
Hypercoagulation studies were performed to evaluate the underlying cause of thrombosis, and she was found to have an abnormal LA/APS studies concerning for antiphospholipid syndrome. Anti-phos Serine/PT IGM antibodies were positive. Her Cardiolipin and beta 2 glycoprotein antibodies were negative. DRVVT, aPTT, and dilute prothrombin initial clotting times were all prolonged and continued to be prolonged after mixing tests. The DRVVT screen was elevated at 58.8 seconds (normal range is less than 40.1 seconds), PTTLA screen was elevated at 53.3 seconds (normal range is less than 42.6 seconds), and the Dilute Prothrombin Time Screen was elevated at 71.8 seconds (normal range 46.9 seconds). These results eliminated the possibility of coagulation factor deficiency and were consistent with the presence of factor inhibitors, such as those present in anti-phospholipid syndrome.
The patient was discharged on anticoagulation therapy with low molecular weight heparin and oral amoxicillin/clavulanate to follow up outpatient for repeat testing of LA/APS panel, abdominal imaging and vascular Doppler. The only notable trigger for new-onset antiphospholipid syndrome in this patient was past COVID-19 infection.