APLS

Question 1

APS definition

Answer 1

Presenting with vascular thrombosis or recurrent fetal losses associated with the presence of APLA
ie. LA, ACL, or anti B2 glycoprotein 1 antibodies IgG and/or IgM detected on two or more occasions at least 12 weeks apart

Question 2

Pediatric APS

Answer 2

onset before age 18

presents primarily as vascular thromboses and less frequently as isolated neurological or hematological disease

Question 3

Probable APS

Answer 3

patients with APLA who have clinical features other than thromboses, like unexplained thrombocytopenia, livedo reticularis, heart valve disease, renal thrombotic microangiopathy and neurological manifestations

Question 4

Definite CAPS

Answer 4

1. Evidence of involvement of 3 or more organs, systems and/or tissues
2. Development of manifestations simultaneous or in less than a week
3. Confirmation by histopathology of small vessel occlusion in at least one organ or tissue
4. Lab confirmation of the presence of antiphospholipid antibodies

Question 5

Primary APS

Answer 5

Accounts for 25-50% pf pediatric patients with APS

• Typically younger
• Higher frequency of arterial thrombotic events , esp cerebrovascular

Question 6

Secondary APS

Answer 6

Higher frequency of venous thrombotic events and hematologic or skin manifestations

Question 7

Viruses associated with APS

Answer 7

Varicella
Parvo
HIV
Strep 
Staph
Gram negative bacteria 
Mycoplasma pneumonae 

“molecular mimicry”

Question 8

Mechanism of APLA damage

Answer 8

• Induces platelets to aggregate and release thromboxane
• Activates monocytes to release inflammatory cytokines and increase TF expression
• Activate Endothelium to increased TX expression and secretion –> leads to increasing vWF and adhesion molecule expression
• Impede fibrinolysis via impairment of activated protein C
• Acts on complement, endothelial and neutrophils to activate and promote tissue accumulation of neutrophils –> enhancing local inflammation

Question 9

LA hypoprothrombinemia syndrome

Answer 9

Rare complication consistent of severe bleeding diathesis associated with LA

Question 10

Which APLs can cross the placenta?

Answer 10

Anti cardiolipn and beta 2 GP

Question 11

What is used to monitor heparin activity in the setting of APLs?

Answer 11

Anti Factor Xa because pTT is altered by LAC