Antiphospholipid syndrome - Clinicals, Diagnosis, and Management

Hematology

Clinicals - History

Fact Explanation
Body paralysis Antiphospholipid syndrome is an autoimmune thrombophilia characterized by venous or artery thrombosis caused by antiphospholipid antibodies which provokes thrombosis
Ischemic stroke due to thrombus formation can cause body paralysis.
Body paralysis
Antiphospholipid syndrome is an autoimmune thrombophilia characterized by venous or artery thrombosis caused by antiphospholipid antibodies which provokes thrombosis
Ischemic stroke due to thrombus formation can cause body paralysis.
Pregnancy related complications High thrombophilic risk leads to pregnancy related complications such as miscarriages, premature birth Pregnancy related complications
High thrombophilic risk leads to pregnancy related complications such as miscarriages, premature birth
Purpura, skin ulceration Thrombotic events on cutaneous vessels Purpura, skin ulceration
Thrombotic events on cutaneous vessels
Chest pain Ischemia due to blocked coronary vessels Chest pain
Ischemia due to blocked coronary vessels
Seizures Non thrombotic neurological features Seizures
Non thrombotic neurological features
Visual defects Due to thrombosis in retinal artery and vein. Visual defects
Due to thrombosis in retinal artery and vein.
hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle These comorbidities may act on inflammatory mechanisms and lipid metabolism contributing to the process of vascular injury, which therefore will trigger and propagate the formation of atherosclerotic plaques. It is suspected that this process may be implicated in vascular involvement in patients with antiphospholipid syndrome hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle
These comorbidities may act on inflammatory mechanisms and lipid metabolism contributing to the process of vascular injury, which therefore will trigger and propagate the formation of atherosclerotic plaques. It is suspected that this process may be implicated in vascular involvement in patients with antiphospholipid syndrome

Clinicals - Examination

Fact Explanation
Heart murmurs Patients with anti phospholipid syndrome have a higher chance of valvular lesions Heart murmurs
Patients with anti phospholipid syndrome have a higher chance of valvular lesions
Livedo reticularis Thrombotic events in cutaneous vessels (arterial thrombosis) Livedo reticularis
Thrombotic events in cutaneous vessels (arterial thrombosis)
Tachypnea Pulmonary embolism- high risk due to thrombophilic state Tachypnea
Pulmonary embolism- high risk due to thrombophilic state

Investigations - Diagnosis

Fact Explanation
Lupus anticoagulant antibody Together with the history,diagnosed if present or if medium to high levels,in plasma Lupus anticoagulant antibody
Together with the history,diagnosed if present or if medium to high levels,in plasma
Anticardiolipin antibodies Together with the history,presence of moderate to high levels of anticardiolipin antibiodies in serum or plasma Anticardiolipin antibodies
Together with the history,presence of moderate to high levels of anticardiolipin antibiodies in serum or plasma

Investigations - Management

Fact Explanation
transthoracic echocardiogram To identify heart valve lesions as patients with Antiphospholipid syndrome are more prone to have valve lesions transthoracic echocardiogram
To identify heart valve lesions as patients with Antiphospholipid syndrome are more prone to have valve lesions
Full blood count To assess the general fitness and for the correcion of other blood abnormalities (eg: hemoglobin levels) Full blood count
To assess the general fitness and for the correcion of other blood abnormalities (eg: hemoglobin levels)
Clotting profile To detect thrombotic abnormalities Clotting profile
To detect thrombotic abnormalities
Fasting blood sugar,lipid profile For the correction of conventional risk factors for thrombosis (diabetes, high blood pressure, high cholesterol, obesity, and smoking) Fasting blood sugar,lipid profile
For the correction of conventional risk factors for thrombosis (diabetes, high blood pressure, high cholesterol, obesity, and smoking)
Anti- beta2GPI antibodies High prevalence of IgA-β2GPI antibodies in patients with antiphospholipid syndrome Anti- beta2GPI antibodies
High prevalence of IgA-β2GPI antibodies in patients with antiphospholipid syndrome
aPTT Detection of thrombotic risk
frequently used as the initial screening test
aPTT
Detection of thrombotic risk
frequently used as the initial screening test
Anti cardiolipin antibody Correlation between higher antibody titers and antiphospholipid syndrome Anti cardiolipin antibody
Correlation between higher antibody titers and antiphospholipid syndrome
Doppler studies To identify deep venous thrombosis, as these patients have a higher risk due to thrombophilic state Doppler studies
To identify deep venous thrombosis, as these patients have a higher risk due to thrombophilic state
Noncontrast head computed tomography (CT) To identify ischemic changes caused by thrombi Noncontrast head computed tomography (CT)
To identify ischemic changes caused by thrombi

Management - Supportive

Fact Explanation
Avoid smoking,take regular physical exercise, maintain a healthy diet and avoid overweight/obesity,avoid excessive alcohol intake. Healthy lifestyle in line with prevention of cardiovascular disease Avoid smoking,take regular physical exercise, maintain a healthy diet and avoid overweight/obesity,avoid excessive alcohol intake.
Healthy lifestyle in line with prevention of cardiovascular disease
Adequate management of cardiovascular risk factors, including diabetes, hypertension and hyperlipidaemia To control the other co morbidities Adequate management of cardiovascular risk factors, including diabetes, hypertension and hyperlipidaemia
To control the other co morbidities
Avoid other factors which contribute to increase the thrombophilic risk Eg:oral contraceptives increase the thrombophilic risk Avoid other factors which contribute to increase the thrombophilic risk
Eg:oral contraceptives increase the thrombophilic risk

Management - Specific

Fact Explanation
Medical therapy for vascular events Acute thrombotic events are treated with anticoagulants (blood thinners), initially with intravenous heparin and then followed by oral warfarin
oral anticoagulants (producing an international normalized ratio of >3) with or without low-dose aspirin are an effective prophylaxis against both venous and arterial thrombosis in most patients. cessation of warfarin therapy in patients with antiphospholipid-antibody–associated thromboses carries a high risk of recurrent thrombosis.
Aspirin is widely used as an antiplatelet agent.
Medical therapy for vascular events
Acute thrombotic events are treated with anticoagulants (blood thinners), initially with intravenous heparin and then followed by oral warfarin
oral anticoagulants (producing an international normalized ratio of >3) with or without low-dose aspirin are an effective prophylaxis against both venous and arterial thrombosis in most patients. cessation of warfarin therapy in patients with antiphospholipid-antibody–associated thromboses carries a high risk of recurrent thrombosis.
Aspirin is widely used as an antiplatelet agent.
Medical therapy for obstetrical manifestations Subcutaneous injections of heparin and low-dose aspirin are the standard therapy for preventing miscarriages. The therapy is started at the beginning of the pregnancy and continued in the period immediately after the delivery.
Treatment of antiphospholipid syndrome during pregnancy reduces the risks of pregnancy loss, preeclampsia, placental insufficiency, preterm birth, and thrombosis.
Low molecular weight heparin can be used as early as six weeks’ gestation until 34–36 weeks’ gestation
Medical therapy for obstetrical manifestations
Subcutaneous injections of heparin and low-dose aspirin are the standard therapy for preventing miscarriages. The therapy is started at the beginning of the pregnancy and continued in the period immediately after the delivery.
Treatment of antiphospholipid syndrome during pregnancy reduces the risks of pregnancy loss, preeclampsia, placental insufficiency, preterm birth, and thrombosis.
Low molecular weight heparin can be used as early as six weeks’ gestation until 34–36 weeks’ gestation

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