rev bras hematol hemoter. 2015;37(6):364–365
w w w . r b h h . o r g
Revista
Brasileira
de
Hematologia
e
Hemoterapia
Brazilian
Journal
of
Hematology
and
Hemotherapy
Scientific
Comment
Comment
on
sickle
cell
disease
and
left
ventricular
hypertrophy
夽
Monica
Pinheiro
de
Almeida
Verissimo
CentroInfantildeInvestigac¸õesHematológicasDr.DomingosA.Boldrini,Campinas,SP,Brazil
Sicklecelldisease(SCD)isamonogenicdiseasethatcausesa significantreductioninlifeexpectancyduetothepresenceof chronicanemia,acutepainfulepisodesandorganicinjuries. Themostcommonchronicinjuriesarecardiopulmonary com-plications,suchaspulmonaryhypertension(HP),chroniclung disease, diastolic dysfunction and congestive heart failure (CHF),whicharemanifestedmostlyinadulthood.
Thesecomplicationsareassociatedwithearly mortality. Withtheimprovementofthehealthcaresystemandgreater access to proper treatment, there is a growing interest in identifyingcomplicationsasearlyaspossiblebyevaluating patientsinchildhoodand adolescenceinorder toimprove morbidityandmortalityrates.1
Patientstendtohaveincreasedleftventricular(LV) stiff-nesswithconsequentleftventricularhypertrophy(LVH)and progression to increased high pulmonary blood pressure. AnothercomplicationduetoLVHisdiastolicdysfunction,an earlymarkerofcardiacimpairmentprecedingCHFthatis fre-quentlyfoundinadultswithSCD.2
ThepathophysiologicmechanismofHPinindividualswith SCDhasnotbeenwelldefinedbutisprobablymultifactorial. Potentialetiologicfactors includehemolysisthat interferes with nitric oxide-mediated vasodilatation, LV dysfunction, pulmonary thromboembolism, airway hyperreactivity, and sleep-disorderedbreathing.3Ahighcardiacoutputandhigh
pulmonarybloodflow,combinedwithintravascularhemolysis promotethesestructuralchanges.4
TheincidenceofHPinthepediatricpopulationrangesfrom 11%to30%.InapaperpublishedbyColombattietal.,there isconfirmationthatHPstartsearlyinchildrenshowingthe
DOIoforiginalarticle:http://dx.doi.org/10.1016/j.bjhh.2015.07.001.
夽
SeepaperbyFaroetal.inRevBrasHematolHemoter.2015;37(5):324–8.
Correspondingauthorat:CentroInfantildeInvestigac¸õesHematológicasDr.DomingosA.Boldrini,DepartamentodeHematologia,Rua GabrielPorto,1270,CidadeUniversitária,13083-210Campinas,SP,Brazil.
E-mailaddress:[email protected]
needforearlyandsystematicassessmentsinthispopulation.5
Thispaperalsoshowedthatdiastolicdysfunctionoccurredin childrenwithSCD.
InthecurrentissueoftheRevistaBrasileirade Hematolo-giaeHemoterapia,thereisanarticleentitled“Leftventricular hypertrophyinchildren,adolescentsandyoungadultswith sickle cellanemia” byFaroet al.Inthisarticlethe authors aimedtoestimatethefrequencyofLVHinchildren, adoles-centsandyoungadultswithsicklecellanemia.6
Ofthe109patientsenrolledinthisstudy,37.6%hadLVH. SCD patients had high rates of LV mass as seen in other populations. The sizeofthe left atrium, another aspectof cardiac complications and a potential marker of diastolic dysfunction, was significantly larger in patients with LVH. Theauthors showedanassociationbetweenthis condition and low hemoglobinand hematocritlevels, a low reticulo-cyteindexandahigheralbumin:creatinineratio.Hemolysis markers (lactate dehydrogenase, indirect bilirubin and fer-ritin) were statistically similar with high values in both groups.6
Unfortunately,this paperdidnotconfirmanyprotective effectofhydroxyurea,buttheauthorshypothesizedthatthis mayberelatedtothesmallnumberofpatientswhowere tak-ing thedrug.Clinicaltrialswithlargernumbers ofpatients mayhelptoclarifytheroleofhydroxyureainthetreatment ofthesecomplications.
Thispaperdemonstratesthatthepopulationofpatients withSCDpresentedcardiaccomplicationsatanearlierage andthattheassociationofclinicalsignsandsymptomsmay guideus.6
http://dx.doi.org/10.1016/j.bjhh.2015.08.005
revbrashematolhemoter.2015;37(6):364–365
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Earlydiagnosisandappropriatetreatmentcouldimpacton themorbidityandmortalityofthesepatientsbychangingthe naturalhistoryofthedisease.
Conflicts
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interest
Theauthordeclaresnoconflictsofinterest.
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1.LiemRI,NevinMA,PrestridgeA,YoungLT,ThompsonAA. Functionalcapacityinchildrenandyoungadultswithsickle celldiseaseundergoingevaluationforcardiopulmonary disease.AmJHematol.2009;84(10):645–9.
2.ZilbermanMV,DuW,DasS,SarnaikSA.Evaluationofleft ventriculardiastolicfunctioninpediatricsicklecelldisease patients.AmJHematol.2007;82(6):433–8.
3.JohnsonMC,KirkhamFJ,RedlineS,RosenCL,YanY,RobertsI, etal.Leftventricularhypertrophyanddiastolicdysfunctionin childrenwithsicklecelldiseasearerelatedtoasleepand wakingoxygendesaturation.Blood.2010;116(1):16–21.
4.MillerAC,GladwinMT.Pulmonarycomplicationsofsicklecell disease.AmJRespirCritCareMed.2012;185(11):1154–65.
5.ColombattiR,MaschiettoN,VarottoE,GrisonA,GrazzinaN, MeneghelloL,etal.Pulmonaryhypertensioninsicklecell diseasechildrenunder10yearsofage.BrJHaematol. 2010;150(5):601–9.
