Page 585 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 28 ■ Disorders of Hemostasis and Thrombosis: Blood Coagulation Factors, Hypercoagulable State, and Anticoagulant Therapy 569

history o recurrent venous or arteria thro boe bo is

BOX 28.4 or a history o iscarriages. APS is an i ortant cause o

acquire thro bo hi ia. APS can occur a one or in associa-

tion with other autoi une con itions.
Criteria for the Laboratory Diagnosis of T e core c inica ani estation is thro bosis. In wo en,

Lupus Anticoagulant it can be associate with recurrent eta oss. Feta orbi -

■ Pro ongation o a hos ho i i - e en ent c otting assay ity an orta ity ay be ue to actors such as acenta

■ Evi ence o an inhibitor e onstrate by a ixing thro bosis an acenta inf a ation ue to co e ent
stu y activation. Secon ary risk actors have been suggeste . T ese

■ Evi ence o a hos ho i i - e en ent inhibitor base are age, hy ertension, iabetes, obesity, s oking, regnancy,
on neutra ization o the inhibitor e ect with a e surgery, an other genetic hy ercoagu ab e states.

hos ho i i s Anti hos ho i i (aPL) antibo ies inc u e

■ Lack o s eci c inhibition o any one coagu ation actor ■ Lu us anticoagu ant

■ Anticar io i in antibo ies

■ Anti–β2-g yco rotein 1 antibo ies

ay have other acquire inhibitors as we . LA occurs in the In the aboratory, e evate eve s o antibo y are require

resence o isease states other than SLE, such as acquire to estab ish a iagnosis. Laboratory iagnosis o anti ho ho-

i uno e ciency syn ro e (AIDS) an a ignancy, an i i (aPL) antibo ies e en s on the etection o an LA,

in rocaina i e, hy ra azine, or ch or ro azine thera y. which ro ongs hos ho i i - e en ent anticoagu ation

A though LA exhibits an anticoagu ant e ect, it is rare y an /or anticar io i in an anti–β-g yco rotein 1 antibo ies.

associate with b ee ing. T e re o inant antigenic targets in anti ho ho i i (aPL)

LA, an IgM, IgG, or IgA i unog obu in, inter eres with

hos ho i i - e en ent coagu ation reactions in aboratory antibo ies are β 2-GP I an rothro bin. Co e ent acti-
vation is sus ecte because increase co e ent activation
assays but oes not inhibit the activity o any s eci c coagu- ro ucts have been oun in APS atients who have su ere

ation actor. LA is an inhibitor that ro ongs hos ho i i - ro a cerebra ische ic event.

e en ent c otting tests in vitro. LA is the ost co on cause Dysregu ate ate et activation ay contribute to thro -

o ro onge activate artia thro bo astin ti e (AP ). botic ani estations. E evate eve s o ate et- erive

In 1995, the Subco ittee on Lu us Anticoagu ant thro boxane etabo ic break own ro ucts have been

Stan ar ization Co ittee ub ishe criteria (Box 28.4) or e onstrate in the urine o APS atients.

the iagnosis o LA. T is gui e ine reco en s at east two Anti hos ho i i (aPL) syn ro e is c inica y e ne

screening tests base on i erent assay rinci es. In a ition, by the resence o one or ore anti hos ho i i antibo -

a ixing stu y or the veri cation o the resence o a coagu a- ies ( u us anticoagu ant, anticyto as ic antibo ies an /

tion inhibitor an a con r ation test or the ocu entation or a bio ogic a se- ositive test or sy hi is acco anie by

o hos ho i i e en ency shou a so be er or e . A the si u taneous or subsequent eve o ent o any one or

assays shou be er or e on citrate anticoagu ate s eci- ore o a nu ber o a iate c inica ani estations. T ese

ens that are ate et oor an ree o un er ying e ects. inc u e venous thro bosis, arteria thro bosis, obstetrica

In co arison, anticar io i in antibo ies (ACAs), IgM, co ications, thro bocyto enia, b ee ing, neuro ogica

IgG, or IgA i unog obu ins, bin to the hos ho i i s isease (transient ische ic attacks [ IA’s] an stroke, ear y-

car io i in in the resence o beta 2-GP 1-car io i in co - onset e entia, a aurosis ugax an retina venous or arte-

ex. It ay be etecte in hea thy atients an in those with ria thro bosis, etc.), skin esions, car iac va ve vegetations

a variety o con itions (e.g., SLE). an itra regurgitation, yocar ia ys unction, ri ary

LA an ACA are risk actors or thro bosis, but the u onary hy ertension, an a rena insu ciency.

echanis o action is unc ear.
Pri ary anti hos ho i i syn ro e is thro bosis an /

or obstetrica co ications in association with anti hos-
Antiphospholipid Syndrom e (APS) ho i i antibo ies, but without signs o connective tis-

Antiphospholipid syndrome (APS) is e ne by c inica sue isease. In co arison, secon ary anti hos ho i i

ani estations that inc u e thro bosis an /or eta oss or syn ro e re ers to those atients with syste ic u us.

regnancy orbi ity in atients with anti ho ho i i (aPL) Overa , autoi une isease is resent or subsequent y

antibo ies. Mu ti e ter s or APS exist. Un ortunate y, so e i enti e .

synony s can be con using. LA syn ro e, or exa e, is is- T e ki neys are a ajor target organ in anti hos ho i i

ea ing because atients with APS ay not necessari y have syn ro e (APS). Ne hro athy in APS is characterize by

SLE an LA is associate with thro botic rather than he or- s a -vesse vasoocc usive esions associate with brous

rhagic co ications. In an atte t to avoi urther con usion, inti a hy er asia o inter obu ar arteries, recana izing

APS is current y the re erre ter or the c inica syn ro e. thro bi in arteries an arterio es, an oca atro hy.

Anti hos ho i i Syn ro e (APS) is a rothro botic T e ha ark resu t ro aboratory tests that e nes

isor er with various ani estations in atients with a anti hos ho i i syn ro e (APS) is the resence o