LABORATORY EVALUATION OF ANTIPHOSPHOLIPID SYNDROME

Antiphospholipid syndrome (APS)

Lupus anticoagulant syndrome Hughes syndrome Sneddon syndrome DEFINITION: Disorder characterized by recurrent venous or arterial thrombosis and/ or fetal losses associated with characteristic laboratory abnormalities such as evidence of a circulating anticoagulant or persistently elevated levels of anticardiolipin antibodies.

Primary APS : APS occur in patients without evidence of any definable associated disease

Secondary APS : APS occurs in association with SLE or another Rheumatic or autoimmune disorder
Catastrophic APS : Acute, severe, multiple organ APS illness

Clinical manifestations

Arterial & Venous thromboembolic disease: Most common Extremity Deep vein thrombosis Axillary, retinal or hepatic veins & cerebral venous sinus thrombosis Cerebral thrombosis or mesentric artery occlusion, GI ischemia or ulceration

Obstetric manifestations: Criteria : 1 or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation or 1 or more premature births of a morphologically normal neonate before the 34th week of gestation or 3 or more unexplained consecutive spontaneous abortions before the 10th week of pregnancy excluding anatomic , hormonal & chromosomal abnormalities

Hematologic : Bleeding : coexistent thrombocytopenia, platelet dysfunction, prothrombin deficiency or other underlying coagulopathies Neurologic Syndromes: acute ischemia (CVA, TIA, encephalopathy), severe migraine, multiple infarct dementia, cognitive dysfunction, seizures

Dermatologic disorders: Ischemic dermatologic syndromes include livedo reticularis, acrocyanosis, widespread cutaneous necrosis, pyoderma gangrenosum like skin lesions Cardio pulmonary disorders: MI, intracardiac thrombotic mass, peripheral arterial disease, thromboembolic & non thrombotic pulmonary hypertension

Catastrophic Antiphopholipid Antibody Syndrome: diffuse small vessel ischemia & occlusion with extensive tissue damage including MI, limb ischemia, DIC & a high mortality rate.

SAPPORO Criteria

CLINICAL EVENT: Venous thrombosis Arterial thrombosis Small vessel thrombosis Complications of pregnancy
LABORATORY ABNORMALITY: Positive Lupus anticoagulant test Positive Anticardiolipin antibodies Positive 2 glycoprotein1 antibodies

Laboratory abnormality should persist for 2 or more occasions at least 12 weeks apart
The diagnosis of APS requires the presence of at least one clinical event & at least one laboratory abnormality

ANTIPHOSPHOLIPID ANTIBODIES
Family of antibodies reactive with proteins which are themselves complexed with negatively charged phospholipids. Lupus anticoagulant & Anticardiolipin antibodies

Lupus Anticoagulant
Heterogenous class of immunoglobulins that may develop spontaneously or as a consequence of autoimmune diseases Bind to proteins such as 2 glycoprotein1, prothrombin or others in complex with negatively charged phospholipids, thus prolong phospholipid dependent coagulation tests

 Lupus

Anticoagulant are stronger risk factor for thrombosis than Anticardiolipin antibodies
is a double misnomer for it is neither a test of lupus nor an in vivo anticoagulant

LA

Anticardiolipin antibodies
APLA

where 2 glycoprotein1 serves as a mandatory protein cofactor by Solid- Phase immunoassay

Detected