kobe-consumptive coagulopathy.doc

8/18/2019 kobe-CONSUMPTIVE COAGULOPATHY.doc

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CONSUMPTIVE COAGULOPATHY

Obstetrical syndromes commonly termed consumptive coagulopathy or disseminated

intravascular coagulation (DIC) were described in the 1901 report by DeLee in which

“temporary hemophilia” developed with placental abruption or with a long-dead macerated

fetus !n ensuing decades" similar #but fre$uently less intense#coagulopathic syndromeshave been described for almost all areas of medicine %Levi" &010b' (ontagnana" &010)

Disseminated Intravascular Ca!ulatin in Pre!nanc"

*ecause of many definitions used and its variable severity" citing an accurate incidence for

consumptive coagulopathy is not possible in pregnant women +or e,ample" as will be

discussed" some degree of significant coagulopathy is found with most cases of placental

abruption and amnionic-fluid embolism Other instances in which fre$uently occurring but

insignificant degrees of coagulation activation can be found include sepsis" thrombotic

microangiopathies" acute idney in.ury" and preeclampsia and /LL % hemolysis" elevated

liver en2yme levels" low platelet count) syndromes %3attray" &01&' 4u" &01&) 5lthough

profound consumptive coagulopathy can be associated with fatty liver disease of pregnancy"diminished hepatic synthesis of procoagulants maes a significant contribution %6elson"

&017) 8hen consumptive coagulopathy is severe" the lielihood of maternal and perinatal

morbidity and mortality is increased 3attray and colleagues %&01&) described 9 cases from

6ova 4cotia during a 70-year period 5ntecedent causes included placental abruption"

obstetrical hemorrhage" preeclampsia and /LL syndromes" acute fatty liver" sepsis" and

amnionic-fluid embolism Of these" :9 percent received blood transfusions" 1; percent

underwent hysterectomy" < percent were dialy2ed" and there were three maternal deaths =he

perinatal mortality rate was 70 percent

Pre!nanc"#Induced Ca!ulatin C$an!es

4everal changes in coagulation and fibrinolysis can be documented during normal pregnancy

4ome of these include appreciable increases in the plasma concentrations of factors !

%fibrinogen)" >!!" >!!!" !?" and ? 5 partial list of these normal values can be found in the

5ppendi, % p 1&;;) and in @hapter % p :A) 5t the same time" plasminogen levels are

increased considerably" but levels of plasminogen activator inhibitor-1 and -& %5!-1 and

5!-&) also increase =hus" plasmin activity usually decreases until after delivery %/ale" &01&'

/ui" &01&) =he mean platelet count decreases by 10 percent during pregnancy" and there is

increased platelet activation %Benny" &01) =he net results of these changes include

increased levels of fibrinopeptide 5" C-thromboglobulin" platelet factor " and fibrinogen-

fibrin degradation products" which includes d-dimers 5long with decreased concentrations of

anticoagulant protein 4" hypercoagulability" and decreased fibrinolysis" there is augmented# yet compensated#intravascular coagulation that may function to maintain the uteroplacental

interface

Pat$l!ical Activatin % Ca!ulatin

6ormal coagulation and fibrinolysis can be pathologically activated via two pathways =he

extrinsic pathway is active by thromboplastin from tissue destruction" whereas the intrinsic

pathway is initiated by collagen and other tissue components that become e,posed with loss

of endothelial integrity %&i!' ()#*+) =issue factor !!! is an integral membrane protein !t is

released by endothelial cells to comple, with factor >!!" which in turn activates tenase %factor

!?) and prothrombinase %factor ?) comple,es ncontrolled thrombin generation converts

fibrinogen to fibrin" which polymeri2es to deposit in small vessels of virtually every organsystem =his seldom causes organ failure" because these vessels are protected by enhanced


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fibrinolysis stimulated by fibrin monomers released by coagulation =hese monomers

combine with tissue plasminogen activator and plasminogen to release plasmin" which lyses

fibrinogen and fibrin monomers and polymers =he products form a series of fibrinogen-

fibrin derivatives that are measured by immunoassay =hese are the fibrin degradation

products or fibrin-split products" which include d-dimers %see +ig 1-70) =here may also be

evidence for microangiopathic hemolysis from mechanical trauma to the red cell membrane by fibrin strands in small vessels =his is liely a contributing cause of hemolysis in women

with preeclampsia and /LL syndromes %ritchard" 19A


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5s discussed" any surgical procedure provides the ultimate bioassay and elicits generali2ed

oo2ing from the sin" subcutaneous and fascial tissues" the retroperitoneal space" the

episiotomy" or incisions and dissections for cesarean delivery or hysterectomy

&i,rin!en and De!radatin Prducts' !n late pregnancy" plasma fibrinogen levels

typically have increased to 700 to


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&etal Deat$ and Dela"ed Deliver"

@onsumptive coagulopathy associated with prolonged retention of a dead fetus is unusual

today because fetal death can be easily confirmed and there are highly effective methods for

labor induction @urrently" the syndrome is only occasionally encountered when there is one

dead twin fetus and an ongoing pregnancy 8ith singleton pregnancies" if the dead fetus is

undelivered" most women enter spontaneous labor within & wees Fross disruption of maternal coagulation rarely develops before wees %ritchard" 19:9" 19A7) 5fter 1 month"

however" almost a fourth will develop consumptive coagulopathy =he pathogenesis of

coagulopathy appears to be mediated by thromboplastin released by the dead fetus and the

placenta %Gimene2" 19


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between this disorder and hypertonus from o,ytocin !n obvious cases of amnionic-fluid

embolism" the clinical picture is un$uestionably dramatic =he classic e,ample is that of a

woman in the late stages of labor or immediately postpartum who begins gasping for air and

then rapidly suffers sei2ures or cardiorespiratory arrest complicated by massive hemorrhage

from consumptive coagulopathy !t has become apparent that there is a variation in the

clinical manifestations of this condition +or e,ample" we and others have managed severalwomen in whom otherwise uncomplicated vaginal or cesarean delivery was followed by

severe acute consumptive coagulopathy without overt cardiorespiratory difficulties !n those

women" consumptive coagulopathy appears to be the forme fruste of amnionic-fluid

embolism %Bramer" &01&' orter" 199


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Pstmrtem &indin!s' /istopathological findings may be dramatic in fatal cases of

amnionic-fluid embolism such as the one shown in &i!ure ()#*0 =he detection of such

debris" however" may re$uire special staining" and even then" it may not be seen !n one study"

fetal elements were detected in A: percent of autopsies and in :0 percent of specimens

prepared from concentrated buffy coat aspirates taen antemortem from a pulmonary artery

catheter %@lar" 199:) 4everal other studies" however" have demonstrated that fetal s$uamouscells" trophoblasts" and other debris of fetal origin may commonly be found in the central

circulation of women with conditions other than amnionic-fluid embolism =hus" the

diagnosis is generally made by identifying clinically characteristic signs and symptoms and

e,cluding other causes

Mana!ement and Clinical Outcmes

!mmediate resuscitative actions are necessary to interdict the high mortality rate 5s

discussed" the initial period of systemic and pulmonary hypertension that fre$uently heralds

amnionic-fluid embolism is transient =racheal intubation" cardiopulmonary resuscitation" and

other supportive measures must be instituted without delay =reatment is directed at

o,ygenation and support of the failing myocardium" along with circulatory support thatincludes rapid blood and component replacement =hat said" there are no data indicating that

any type of intervention improves maternal or fetal prognosis !n undelivered women

undergoing cardiopulmonary resuscitation" consideration should be given to emergency

cesarean delivery to perhaps optimi2e these efforts and improve newborn outcome Decision

maing for perimortem cesarean delivery is more comple, in a woman who is

hemodynamically unstable but who has not suffered cardiac arrest %@hap A" p 9:


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Pur.ura &ulminans

=his severe#often lethal#form of consumptive coagulopathy is caused by microthrombi in

small blood vessels leading to sin necrosis and sometimes vasculitis Debridement of large

areas of sin over the e,tremities and buttocs fre$uently re$uires treatment in a burn unit

urpura fulminans usually complicates sepsis in women with hetero2ygous protein @

deficiency and low protein @ serum levels %Levi" &010b) 3ecall that homo2ygous protein @deficiency results in fatal neonatal purpura fulminans %@hap :&" p 1071)

A,rtin

4eptic abortion#especially associated with the organisms discussed above#can incite

coagulation and worsen hemorrhage" especially with midtrimester abortions !ndeed" sepsis

syndrome accompanied by intravascular coagulation accounts for &: percent of abortion-

related deaths %4araiya" 1999) !n the past" especially with illegal abortions" infections with

Clostridium perfringens were a fre$uent cause of intense intravascular hemolysis at arland

/ospital %ritchard" 19A1) (ore recently" however" septic abortions from infection with

Clostridium sordellii have emerged as important causes %@hap 1;" p 7:A) 4econd-trimester

induced abortions can stimulate intravascular coagulation even in the absence of sepsis *en-5mi and associates %&01&) described a 1

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