Table 1.

Chronology of events, anticoagulation, and antiphospholipid antibody titers: eculizumab initiated on 9/4/2019.

EventDatePresentationInterventionPrior anticoagulationCurrent anticoagulationAntiphospholipid antibody titers (U)
1. 7/2016 Right subclavian, axillary, internal jugular, brachiocephalic vein thrombosis Thrombolysis,cervical rib resection None Enoxaparin (1 mg/kg twice daily) bridged to warfarin (INR 2-3) Anti–β-2 GPI: IgG, 84.9 SGU; IgM, 76.5 SMU; IgA, 66.7 SAU.* Antiphosphatidylserine: IgG, 3.9 GPL; IgM, 26.2 MPL; IgA, 2.4 APL. Antiphosphatidylinositol: IgG, 4.5 GPL; IgM, 45.8 MPL; IgA, 0.6 APL. Antiphosphatidylcholine: IgG, 2.4 GPL; IgM, 5.1 MPL; IgA, 0.4 APL. Antiphosphatidylethanolamide: IgG, 6.1 GPL; IgM, 112.6 MPL; IgA, 0.9 APL. Antiphosphatidic acid: IgG, 10.1 GPL; IgM, 211.7 MPL; IgA, 14.1 APL. Antiphosphatidylglycerol: IgG, 16.2 GPL; IgM, 56.5 MPL; IgA, 0.5 APL. Lupus anticoagulant: positive 
2. 11/2016 Left subclavian vein thrombosis Thrombolysis Warfarin (INR 1.1) Warfarin (INR 2-3) + ASA (81 mg daily) Anti–β-2 GPI: IgG, 124.9 SGU; IgM, 112.6 SMU; IgA, 66.0 SAU.* Antiphosphatidylserine: IgG, 21.5 GPL; IgM, 39.7 MPL; IgA, 1.8 APL. Antiphosphatidylinositol: IgG, 2.1 GPL; IgM, 12.3 MPL; IgA, 1.8 APL. Antiphosphatidylcholine: IgG, 4.5 GPL; IgM, 8.9 MPL; IgA, 2.2 APL. Antiphosphatidylethanolamide: IgG, 0.6 GPL; IgM, 2.9 MPL; IgA, 0.4 APL. Antiphosphatidic acid: IgG, >120.0 GPL; IgM, 98.2 MPL; IgA, 4.8 APL. Antiphosphatidylglycerol: IgG, 2.0 GPL; IgM, 11.7 MPL; IgA, 1.7 APL. Lupus anticoagulant: positive 
3. 12/2016 Left common femoral and iliac vein thrombosis Thrombolysis Warfarin (INR 1.7) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) Anti–β-2 GPI: IgG, 111.5 GPL-U/mL; IgM, >112 MPL-U/mL; IgA, 34.8 APL-U/mL.* Antiphosphatidylserine: IgG, 18.7 GPL; IgM, 34.4 MPL; IgA 1.6 APL. Antiphosphatidylinositol: IgG, 2.1 GPL; IgM, 7.5 MPL; IgA, 0.8 APL. Antiphosphatidylcholine: IgG, 0.8 GPL; IgM, 11.5 MPL; IgA, 3.8 APL. Antiphosphatidylethanolamide: IgG, 1.4 GPL; IgM, 2.0 MPL; IgA, 1.3 APL. Antiphosphatidic acid: IgG, >120.0 GPL; IgM, 98.9 MPL; IgA, 13.5 APL. Antiphosphatidylglycerol: IgG, 2.8 GPL; IgM, 11.2 MPL; IgA, 0.8 APL. Lupus anticoagulant: positive 
4. 12/2016 Recurrent Left common femoral and iliac, popliteal vein thrombosis Thrombolysis and stent placement Apixaban (5 mg twice daily) Apixaban (5 mg twice daily) + ASA (81 mg daily) + rituximab (889 mg = 375 mg/m2 × 2.37 m2), weekly × 4 Per #3 
5. 2/2018 Left common femoral to saphenous vein thrombosis None Apixaban (5 mg twice daily) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) + ASA (81 mg daily) Anticardiolipin: IgG, 110.7 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 46.0 APL-U/mL.† Anti–β-2 GPI: IgG, 90.5GPL-U/ML; IgM, >112.0 MPL-U/mL; IgA, 38.2 APL-U/mL. Lupus anticoagulant: positive 
6. 3/2018 Left common femoral vein thrombosis (new thrombus burden) Thrombolysis and venoplasty Apixaban (5 mg twice daily) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg daily) Per #5 
7. 4/2018 Left leg extensive venous thrombus burden Thrombolysis + stent Apixaban (5 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg daily) Enoxaparin (1 mg/kg twice daily) × 1 moHydroxychloroquine (200 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily). Enoxaparin transitioned to fondaparinux 10 mg daily after approximately 1 mo outpatient Per #5 
8. 4/2019 Hepatic infarction Plasma exchange × 6 sessions for 50-75% titer reduction goal + methylprednisolone 1000 mg × 3 days, followed by prednisone 60 mg daily Fondaparinux (10 mg daily + ASA (81 mg daily) + clopidogrel (75 mg daily) Fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) + prednisone (60 mg daily) PREplasma exchange: Anticardiolipin: IgG, 103.5 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 59.3 APL-U/mL; Anti–β-2 GPI: IgG, 82.2 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 42.6 APL-U/mL.† Lupus anticoagulant: positivePOSTplasma exchange: Anticardiolipin: IgG, 111.5 GPL-U/mL; IgM, 60.7 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, 102.1 MPL-U/mL; IgA, >65.0 APL-U/mL.† Lupus anticoagulant: positiveRepeated 5/2019: Anticardiolipin: IgG, 89.6 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >80.3 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL.† Lupus anticoagulant: positive 
9. 6/2019 Evolution of hepatic infarction None Fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) + prednisone (60 mg daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Anticardiolipin: IgG, 110.1 GPL-U/mL; IgM, 97.7 MPL-U/mL; IgA, 61.2 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 52.1 APL-U/mL.† Lupus anticoagulant: positive 
10. 10/2019 Segmental and subsegmental pulmonary emboli-left lower lobe of lung None Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Per #9 
11. 6/2020 Outpatient follow-up: no new thrombotic event None Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Anti–β-2 GPI: IgG, 142.4 SGU; IgM, 136 SMU; IgA, >150 SAU.* Antiphosphatidylserine: IgG, 35.2 GPL; IgM, 115.5 MPL; IgA, 5.1 APL. Antiphosphatidylinositol: IgG, 5.0 GPL; IgM, 13.9 MPL; IgA, 2.1 APL. Antiphosphatidylethanolamide: IgG, 2.4 GPL; IgM, 5.0 MPL; IgA, 2.0 APL. Antiphosphatidic acid: IgG, 85.2 GPL; IgM, 82.1 MPL; IgA, 5.8 APL. Antiphosphatidylglycerol: IgG, 43.3 GPL; IgM, 79.9 MPL; IgA, 6.9 APL. Anticardiolipin: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >30.9 APL-U/mL.† Lupus anticoagulant: positive 
EventDatePresentationInterventionPrior anticoagulationCurrent anticoagulationAntiphospholipid antibody titers (U)
1. 7/2016 Right subclavian, axillary, internal jugular, brachiocephalic vein thrombosis Thrombolysis,cervical rib resection None Enoxaparin (1 mg/kg twice daily) bridged to warfarin (INR 2-3) Anti–β-2 GPI: IgG, 84.9 SGU; IgM, 76.5 SMU; IgA, 66.7 SAU.* Antiphosphatidylserine: IgG, 3.9 GPL; IgM, 26.2 MPL; IgA, 2.4 APL. Antiphosphatidylinositol: IgG, 4.5 GPL; IgM, 45.8 MPL; IgA, 0.6 APL. Antiphosphatidylcholine: IgG, 2.4 GPL; IgM, 5.1 MPL; IgA, 0.4 APL. Antiphosphatidylethanolamide: IgG, 6.1 GPL; IgM, 112.6 MPL; IgA, 0.9 APL. Antiphosphatidic acid: IgG, 10.1 GPL; IgM, 211.7 MPL; IgA, 14.1 APL. Antiphosphatidylglycerol: IgG, 16.2 GPL; IgM, 56.5 MPL; IgA, 0.5 APL. Lupus anticoagulant: positive 
2. 11/2016 Left subclavian vein thrombosis Thrombolysis Warfarin (INR 1.1) Warfarin (INR 2-3) + ASA (81 mg daily) Anti–β-2 GPI: IgG, 124.9 SGU; IgM, 112.6 SMU; IgA, 66.0 SAU.* Antiphosphatidylserine: IgG, 21.5 GPL; IgM, 39.7 MPL; IgA, 1.8 APL. Antiphosphatidylinositol: IgG, 2.1 GPL; IgM, 12.3 MPL; IgA, 1.8 APL. Antiphosphatidylcholine: IgG, 4.5 GPL; IgM, 8.9 MPL; IgA, 2.2 APL. Antiphosphatidylethanolamide: IgG, 0.6 GPL; IgM, 2.9 MPL; IgA, 0.4 APL. Antiphosphatidic acid: IgG, >120.0 GPL; IgM, 98.2 MPL; IgA, 4.8 APL. Antiphosphatidylglycerol: IgG, 2.0 GPL; IgM, 11.7 MPL; IgA, 1.7 APL. Lupus anticoagulant: positive 
3. 12/2016 Left common femoral and iliac vein thrombosis Thrombolysis Warfarin (INR 1.7) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) Anti–β-2 GPI: IgG, 111.5 GPL-U/mL; IgM, >112 MPL-U/mL; IgA, 34.8 APL-U/mL.* Antiphosphatidylserine: IgG, 18.7 GPL; IgM, 34.4 MPL; IgA 1.6 APL. Antiphosphatidylinositol: IgG, 2.1 GPL; IgM, 7.5 MPL; IgA, 0.8 APL. Antiphosphatidylcholine: IgG, 0.8 GPL; IgM, 11.5 MPL; IgA, 3.8 APL. Antiphosphatidylethanolamide: IgG, 1.4 GPL; IgM, 2.0 MPL; IgA, 1.3 APL. Antiphosphatidic acid: IgG, >120.0 GPL; IgM, 98.9 MPL; IgA, 13.5 APL. Antiphosphatidylglycerol: IgG, 2.8 GPL; IgM, 11.2 MPL; IgA, 0.8 APL. Lupus anticoagulant: positive 
4. 12/2016 Recurrent Left common femoral and iliac, popliteal vein thrombosis Thrombolysis and stent placement Apixaban (5 mg twice daily) Apixaban (5 mg twice daily) + ASA (81 mg daily) + rituximab (889 mg = 375 mg/m2 × 2.37 m2), weekly × 4 Per #3 
5. 2/2018 Left common femoral to saphenous vein thrombosis None Apixaban (5 mg twice daily) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) + ASA (81 mg daily) Anticardiolipin: IgG, 110.7 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 46.0 APL-U/mL.† Anti–β-2 GPI: IgG, 90.5GPL-U/ML; IgM, >112.0 MPL-U/mL; IgA, 38.2 APL-U/mL. Lupus anticoagulant: positive 
6. 3/2018 Left common femoral vein thrombosis (new thrombus burden) Thrombolysis and venoplasty Apixaban (5 mg twice daily) + ASA (81 mg daily) Apixaban (10 mg twice daily × 7 days, 5 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg daily) Per #5 
7. 4/2018 Left leg extensive venous thrombus burden Thrombolysis + stent Apixaban (5 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg daily) Enoxaparin (1 mg/kg twice daily) × 1 moHydroxychloroquine (200 mg twice daily) + ASA (81 mg daily) + clopidogrel (75 mg daily). Enoxaparin transitioned to fondaparinux 10 mg daily after approximately 1 mo outpatient Per #5 
8. 4/2019 Hepatic infarction Plasma exchange × 6 sessions for 50-75% titer reduction goal + methylprednisolone 1000 mg × 3 days, followed by prednisone 60 mg daily Fondaparinux (10 mg daily + ASA (81 mg daily) + clopidogrel (75 mg daily) Fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) + prednisone (60 mg daily) PREplasma exchange: Anticardiolipin: IgG, 103.5 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 59.3 APL-U/mL; Anti–β-2 GPI: IgG, 82.2 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 42.6 APL-U/mL.† Lupus anticoagulant: positivePOSTplasma exchange: Anticardiolipin: IgG, 111.5 GPL-U/mL; IgM, 60.7 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, 102.1 MPL-U/mL; IgA, >65.0 APL-U/mL.† Lupus anticoagulant: positiveRepeated 5/2019: Anticardiolipin: IgG, 89.6 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >80.3 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL.† Lupus anticoagulant: positive 
9. 6/2019 Evolution of hepatic infarction None Fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) + prednisone (60 mg daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Anticardiolipin: IgG, 110.1 GPL-U/mL; IgM, 97.7 MPL-U/mL; IgA, 61.2 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, 52.1 APL-U/mL.† Lupus anticoagulant: positive 
10. 10/2019 Segmental and subsegmental pulmonary emboli-left lower lobe of lung None Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Per #9 
11. 6/2020 Outpatient follow-up: no new thrombotic event None Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Eculizumab (900 mg every alternate week infusion) + fondaparinux (10 mg daily) + ASA (81 mg daily) + clopidogrel (75 mg daily) + hydroxychloroquine (200 mg twice daily) Anti–β-2 GPI: IgG, 142.4 SGU; IgM, 136 SMU; IgA, >150 SAU.* Antiphosphatidylserine: IgG, 35.2 GPL; IgM, 115.5 MPL; IgA, 5.1 APL. Antiphosphatidylinositol: IgG, 5.0 GPL; IgM, 13.9 MPL; IgA, 2.1 APL. Antiphosphatidylethanolamide: IgG, 2.4 GPL; IgM, 5.0 MPL; IgA, 2.0 APL. Antiphosphatidic acid: IgG, 85.2 GPL; IgM, 82.1 MPL; IgA, 5.8 APL. Antiphosphatidylglycerol: IgG, 43.3 GPL; IgM, 79.9 MPL; IgA, 6.9 APL. Anticardiolipin: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >65.0 APL-U/mL; Anti–β-2 GPI: IgG, >112.0 GPL-U/mL; IgM, >112.0 MPL-U/mL; IgA, >30.9 APL-U/mL.† Lupus anticoagulant: positive 

No evidence of venous or arterial thrombosis since 10/2019 to 01/05/2022.

U, units.

*

ELISA assay.

†

Multiplex flow immunoassay.

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