Antiphosholipid syndrome p-What is that ??

Antiphospholipids( APS ) DISORDERS

1)   APL antibodiesà not  only cause thrombosis but also other events (autoimmune vascular thrombosis) both thrombogenic and non thrombogenic effects

1)   Primary APL syndrome :- means threes   no clinical /serological  evidence of SLE

2)   Secondary  APL with c/o SLE

3)   APL abs acts on platelate/endothermic –thrombosis

4)   Inhibition of prosenn c/s anticoagularts –Antithromnin III

APL ab affect inplantation by suppressing uraknase PG & Inter Leukin III

Protein C is vitamin K & Needs a cofactor proteins and it degrades foctors va & VII I a and this causes prothmbotic process. Protein s is natural anticoagulet.

Diag clinical a) At least one missed ab after 10 weeks of gest.

b) 3/more consecutive abortion below 10wks but no hormonal /chpomo /anatomic

c) Preterm birth <34 wks due to severe P/H/pl insuff.

A) APL ab

Test for both the antibodies cofactors are essential for execlution of ill effects of antibodies.

Elisa

1)   IgG is more common

2)   Exerts c/f if highly (+) ab

3)   Expressed as

I)                  Low+ -<20 UNITS

II)               Med + -<20-80

III)            Highly + - >80 units

IgG is expressed as GPL UNITS IgM is expressed is MPL units

B) Lupus antibodies

(Coagulation based ab )

More specific for APS

(Platelate poor plasma has to be prepared)

Screening Procedure

a)   APTT

b)  d RVVT

c)    KCT or divilute P. Time use at least 2 screening procedure then final confirmation by other two steps for demonstration of inhibition.

d)  B2 GP9 (Anti Br glycoprsein/ antibodies)

e)   Anti-annexin antibodies

Classification of APS+ RS

Classic APS (definite APS)	LA(+) or Med/high IgG/ IgM ACL	Rec premby omic or Rec embyomic or IUFD or NND after (7 distance severe PIH) (Rec thembyomic Rec embryonic IUFD	Tr by Hep+ ASA
	Low Levels of IgG /IgM ACL		NO HEP
Syndrome of APL other than LA & ACL	LA-Ve ACL-ve AntiBglycoprotein Iab+		?

LDA Low dose Aspirin 75 Tab

LMWH (Enoxaparin)20-40 / mg does sces upto =6 wk to 34 wk

Minimal alt of PT & aPTT LB rated 85 .95 %

I/v immaglobl only if HEP & ASO do not help to continue the pregnancy

If Heparin (cheaper) is used at all dose will be 5000 subcent BD ( proplylactic dose) some use 10000-20000 BD weekly platelate count 2 wk then meonthly Add (a) wt D Help more posent anticouple than LMWH.

Both Hep & LMWH do not cross pl LB rate less than LMWH 80%

Anti thrombin III  deficiency protein C deficiency protein S deficiency

The antiphospholipid syndrome (APS)

Lupus anticoagulant (LAC) and/ or anti cardiolipin antibodies (acl)

Women who miscarry 10 weeks or later in their first intended pregnancy should be screened for inherited throbophilia.

Table :10.3: Antiphospholipid syndrome – clinical manifestations

.Arterial /venous thrombosis

.Recurrent fetal loss

.Thrombocy topenia

.Hemolytic anaemia

.Mitral valve disruption

.Livedo reticularis

.Systemic hypertension

.Pulmonary hypertension

Women with RPL should be tested for the two most common causes factor V Leiden (FVL) and prothrombin gene (G20210A)

Overall 21 percent of the women with thrombophilia who did not receive thrombophylaxix during pregnancy suffered miscarriage , compared with just 8 percent of those who did receive treatment mainly with heparin.

Heritable thrombophilic defects and fetal loss.