The finding of a lupus anticoagulant or anticardiolipin antibodies together with thrombosis is termed the antiphospholipid thrombosis syndrome. The thrombosis seen in patients with anticardiolipin antibodies tends to follow a more predictable pattern than that seen in patients with lupus anticoagulants.
The thrombotic syndromes associated with anticardiolipin antibodies can be divided into one of six subgroups based on the pattern of thrombotic findings. The subgroup seen in a patient can help guide appropriate antithrombotic therapy.
|
Syndrome |
Clinical Findings |
|
Type I |
deep venous thrombosis with or without pulmonary embolus |
|
Type II |
coronary artery thrombosis peripheral artery thrombosis aortic thrombosis carotid artery thrombosis |
|
Type III |
retinal artery thrombosis retinal vein thrombosis cerebrovascular thrombosis transient cerebral ischemic attacks |
|
Type IV |
mixtures of types I, II and III rare |
|
Type V |
fetal wastage placental vascular thrombosis maternal thrombocytopenia (uncommon) |
|
Type VI |
laboratory evidence of antiphospholipid antibody no apparent clinical manifestations |
|
Syndrome |
Antithrombotic Regimens |
|
Type I |
intravenous or subcutaneous heparin followed by long-term self-administration of subcutaneous porcine or LMW heparin |
|
Type II |
intravenous or subcutaneous heparin followed by long-term self-administration of subcutaneous porcine or LMW heparin |
|
Type III |
retinal: pentoxiphylline (400 mg tid) cerebrovascular: long term low dose warfarin plus low dose ASA , or long term self administration of subcutaneous porcine or LMW heparin |
|
Type IV |
according to Type I, II and/or III (above) |
|
Type V |
low dose ASA (81 mg/day) pre-conception; addition of fixed low dose porcine heparin (5,000 U every 12 hours) immediately after conception |
|
Type VI |
no clear indications for antithrombotic therapy |
Long-term antithrombotic therapy should not be stopped unless the anticardiolipin antibody has been absent for the preceding 4-6 months.
Specialty: Hematology Oncology, Clinical Laboratory, Immunology/Rheumatology
