rev bras hematol hemoter. 2014;36(5):313–314
Revista
Brasileira
de
Hematologia
e
Hemoterapia
Brazilian
Journal
of
Hematology
and
Hemotherapy
w w w . r b h h . o r g
Scientific
comment
Pregnancy
in
sickle
cell
disease
–
do
we
know
what
to
expect?
夽
Kleber
Yotsumoto
Fertrin
UniversidadeEstadualdeCampinas(UNICAMP),Campinas,SP,Brazil
Pregnancy in patients with sickle cell disease (SCD) has always been a challenge for both hematologists and obstetricians.Althoughincreasingknowledgeonthecomplex pathophysiology of the sickle vaso-occlusive process has enabledbettercharacterizationoftheendothelialdysfunction in SCD and how different genotypes present with varying degreesof severity, physicianscan still not besure ofthe outcomeofapregnancy ina givenpatientbasedsolelyon thebaselineassessmentoutside ofthepregnancysetting– obstetricianshavefrequentlybeensurprisedbyhowseverely illpregnantpatientswithSCDcanget,eventhoughtheyhad neverhadlife-threateningcomplicationspreviously.
SCD predisposespregnantwomen toa largenumber of complications, such as a higher incidence of eclampsia, preterm labor and delivery, deep venous thrombosis, intrauterine growth restriction, urinary tract infections, sepsis,etc.1Whileitisunderstandablethatwidespreadsickle
vasculopathy can contribute to poor pregnancy outcomes, scientific literature on both the pathophysiology of these complications in SCD pregnancies and evidence-based recommendations for the proper management of these patientsarestilllacking.
A clear example is the concept that hydroxyurea (HU) should not be used in pregnant women, and that its use should be interrupted once pregnancy is confirmed.2 This
is easily accepted by physicians, but evidence for such recommendationsislargely based onanimal studiesusing veryhighdosesofHU,andacarefulreviewoftheliterature sofar hasfailedtoproveincreasedriskofbirthdefects in pregnantwomen takingHU.3 Without HU asa therapeutic
option,managementofpregnantpatientswithSCDislimited
DOIoforiginalarticle:http://dx.doi.org/10.1016/j.bjhh.2014.07.002.
夽
SeepaperbySilva-PintoACetal.onpages329–33.
Correspondingauthorat:ClinicalPathologyDepartment,UniversidadeEstadualdeCampinas(Unicamp),RuaCarlosChagas,450,Cidade UniversitáriaProf.ZeferinoVaz,13083-878DistritodeBarãoGeraldo,Campinas,SP,Brazil.
E-mailaddress:[email protected](K.Y.Fertrin).
to blood transfusion. Reportshave been mostly limited to case series, with onlya few published prospective studies addressing transfusion strategies.4,5 In this regard,thereis
neitherconsensusonhowtodecide whichpatientsshould be transfused nor are there studies that investigate how andatwhattimepointduringthepregnancythiswouldbe ideal.Gillietal.havepreviouslyreportedfavorableoutcomes in SCD patients that were systematically subjected to erythrocytapheresisby28weeksofgestation.6Similarly,the
articlebySilva-Pintoetal.inthisissueoftheRevistaBrasileira de Hematologia eHemoterapia (RBHH) joins the voices of several other publications that present data associating transfusion with better fetal and maternal outcomes.7
Statisticalsignificanceishardtocomebybecausepregnancy inSCDisstillarelativelyrare eventinasingleinstitution. While only one randomized, prospective trial showed no benefitinreducingpregnancy-relatedcomplicationsorfetal growth impairment with prophylactic transfusions, there wasasignificantreductionintheincidenceofvaso-occlusive crises.
WiththecurrentefforttowardbetterqualityoflifeforSCD patients,andtheperspectiveofnewtherapiestoincreaselife expectancyinthispopulation,thereisneedformulticentric collaborationtoobtainbetterevidenceonhowtomanagethis specialpopulationofpregnantwomen.Sinceteratogenicity offetalhemoglobininducerswillmostprobablyprecludethe designofclinicaltrialsinvolvingpregnantwomen,prospective studiesshouldfocusontransfusionalmanagementofthese patients,evaluatingbothobstetricandhematologicoutcomes. Untilthen,physicianswillstillnotknowwhattoexpectwhen theirpatientsareexpecting.
http://dx.doi.org/10.1016/j.bjhh.2014.07.017
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revbrashematolhemoter.2014;36(5):313–314Conflicts
of
interest
Theauthordeclaresnoconflictsofinterest.
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1.VillersMS,JamisonMG,DeCastroLM,JamesAH.Morbidity associatedwithsicklecelldiseaseinpregnancy.AmJObstet Gynecol.2008;199(2):125.e1–5.
2.BrawleyOW,CorneliusLJ,EdwardsLR,GambleVN,GreenBL, InturrisiC,etal.NationalInstitutesofHealthConsensus DevelopmentConferencestatement:hydroxyureatreatment forsicklecelldisease.AnnInternMed.2008;148(12):932–8.
3.Diav-CitrinO,HunnisettL,SherGD,KorenG.Hydroxyureause duringpregnancy:acasereportinsicklecelldiseaseand
reviewoftheliterature.AmJHematol.1999;60(2): 148–50.
4.NgôC,KayemG,HabibiA,BenachiA,GoffinetF,GalactérosF, etal.Pregnancyinsicklecelldisease:maternalandfetal outcomesinapopulationreceivingprophylacticpartial exchangetransfusions.EurJObstetGynecolReprodBiol. 2010;152(2):138–42.
5.KoshyM,BurdL,WallaceD,MoawadA,BaronJ.Prophylactic red-celltransfusionsinpregnantpatientswithsicklecell disease.Arandomizedcooperativestudy.NEnglJMed. 1988;319(22):1447–55.
6.GilliSC,DePaulaEV,BiscaroFP,MarquesJF,CostaFF,SaadST. Third-trimestererythrocytapheresisinpregnantpatientswith sicklecelldisease.IntJGynaecolObstet.2007;96(1):8–11.
