Abstract
Aplastic anemia and agranulocytosis are rare but life-threatening disorders, often caused by drugs and other environmental exposures. Reported incidence of these diseases seems to vary between different geographic regions, and few data on their incidence are available for Latin American countries. The aim of this work is to determine the incidence of agranulocytosis and aplastic anemia in Brazil. Incidence study. Seven centers took part in the pilot phase, so as to represent all Brazilian regions. Each center conducted an active search for new cases in a defined region by means of regular contacts with all hematologists, main clinical laboratories and clinicians in hospitals of the region. 74 patients with aplastic anemia and 16 with agranulocytosis were identified. Patients with agranulocytosis had a median age of 31 years (interquartile range, IQR: 12.5-48.2); 32.2% were male and 81.2% were white. The median age of aplastic anemia patients was 21 years (IQR 15.0-35.2); 62.2% were male, 50.0% were white and 39.2% mulatto. The incidence of agranulocytosis was estimated to be 0.5 cases per million individuals per year, ranging from 0.0 to 1.1 cases per million per year between regions. The incidence of aplastic anemia was 2.7 cases per million per year, ranging from 1.1 to 7.1 cases per million per year between regions. Aplastic anemia and agranulocytosis are rare diseases in Brazil. However, there is considerable variability in their incidences between different regions.
Highlights
)N ORDER TO ESTIMATE THE INCIDENCE OF AGRANULOCYTOSISANDAPLASTICANEMIA EACH CENTERCONDUCTEDANACTIVESEARCHFORNEW CASESTHROUGHOUTITS3TATE WITHTHEEXCEP TIONOF2IBEIRáO0RETO3TATEOF3áO0AULO WHERETHEACTIVESEARCHREGIONWASRESTRICT ED TO ADJACENT CITIES &OR THE PURPOSE OF CARRYINGOUTTHEACTIVESEARCH EACHCENTER RELIED UPON ONE OR MORE STUDY COORDINA TORS WHODREWUPCONTACTLISTSINCLUDINGALL THEACTIVEHEMATOLOGISTSWITHINTHEREGION COVERED THEMAINCLINICALLABORATORIESAND OTHERCLINICIANSINHOSPITALSINTHEREGION )NITIALLY ADOCUMENTWASSENTTOTHESEPRO FESSIONALSTHATINCLUDEDASUMMARYOFTHE STUDYANDAREQUESTFORTHEIRCOLLABORATION THROUGH REPORTING POSSIBLE NEW CASES TO THESTUDYCOORDINATORS4HELATTERKEPTUP WEEKLYCONTACTWITHTHEPROFESSIONALSINTHE VARIOUS INSTITUTIONS 7HENEVER NEW SUS PECTEDCASESWEREIDENTIFIED THESTUDYCO ORDINATORSWOULDHEADFORTHECENTERSAND CONDUCTTHEINTERVIEWS/NLYTHEPATIENTS WHOHADBEENLIVINGINTHEREGIONCOVERED BYTHERESEARCHCENTERFORMORETHANTHREE MONTHSWERECONSIDEREDASINCIDENTCASES FORINCIDENCECALCULATION.
ÂVÓÂÂ 4HEANALYSISOFTHEDATAGENERATEDDURING THEPILOTPHASEOFTHE,!4).STUDYENABLED THE OBTAINING OF PRELIMINARY VALUES FOR THE INCIDENCEOFAPLASTICANEMIAANDAGRANULOCY TOSIS DERIVEDFROMSEVENCENTERSINlVE"RAZIL IAN REGIONS 7E OBSERVED MARKED VARIABILITY IN INCIDENCE BETWEEN THE REGIONS AND THIS PATTERNRESEMBLEDTHEVARIABILITYININCIDENCE BETWEENCOUNTRIESTHATHASBEENREPORTEDFROM PREVIOUSSTUDIES 4HEINCIDENCEOFAPLASTICANEMIAOBSERVED IN THE PRESENT STUDY CASESMILLION INDI VIDUALSYEAR ISSIMILARTOWHATWASPREVIOUSLY REPORTEDFROMASTUDYINSOUTHERN"RAZIL CASESMILLION INDIVIDUALSYEAR AND IN THE )NTERNATIONAL !GRANULOCYTOSIS AND !PLASTIC !NEMIA 3TUDY )!!!3 CASESMILLION INDIVIDUALSYEAR 4HEINCIDENCEOFAPLASTIC ANEMIAREPORTEDFROMSTUDIESIN-EXICOAND
&OLLOWINGTHEPILOTPHASE WEHAVECON CLUDED THAT A LARGER STUDY IS FEASIBLE AND WE HAVEMADETWOMAJORCHANGESTOTHEPROTOCOL &IRSTLY WEHAVERESTRICTEDTHEAREACOVEREDBY EACH CENTERS ACTIVE SEARCH SO AS TO INCLUDE ONLY THE CITIES WITH BETTER MEDICAL SYSTEMS 3ECONDLY WE HAVE REVISED THE CASE REPORT FORMS WHICH ARE SIMPLER AND MORE OBJECTIVE &INALLY AS PLANNED INITIALLY OTHER CENTERSIN!RGENTINA #OLOMBIAAND-EXICO HAVEJOINEDTHESTUDY
Summary
)N ORDER TO ESTIMATE THE INCIDENCE OF AGRANULOCYTOSISANDAPLASTICANEMIA EACH CENTERCONDUCTEDANACTIVESEARCHFORNEW CASESTHROUGHOUTITS3TATE WITHTHEEXCEP TIONOF2IBEIRáO0RETO3TATEOF3áO0AULO WHERETHEACTIVESEARCHREGIONWASRESTRICT ED TO ADJACENT CITIES &OR THE PURPOSE OF CARRYINGOUTTHEACTIVESEARCH EACHCENTER RELIED UPON ONE OR MORE STUDY COORDINA TORS WHODREWUPCONTACTLISTSINCLUDINGALL THEACTIVEHEMATOLOGISTSWITHINTHEREGION COVERED THEMAINCLINICALLABORATORIESAND OTHERCLINICIANSINHOSPITALSINTHEREGION )NITIALLY ADOCUMENTWASSENTTOTHESEPRO FESSIONALSTHATINCLUDEDASUMMARYOFTHE STUDYANDAREQUESTFORTHEIRCOLLABORATION THROUGH REPORTING POSSIBLE NEW CASES TO THESTUDYCOORDINATORS4HELATTERKEPTUP WEEKLYCONTACTWITHTHEPROFESSIONALSINTHE VARIOUS INSTITUTIONS 7HENEVER NEW SUS PECTEDCASESWEREIDENTIFIED THESTUDYCO ORDINATORSWOULDHEADFORTHECENTERSAND CONDUCTTHEINTERVIEWS/NLYTHEPATIENTS WHOHADBEENLIVINGINTHEREGIONCOVERED BYTHERESEARCHCENTERFORMORETHANTHREE MONTHSWERECONSIDEREDASINCIDENTCASES FORINCIDENCECALCULATION.
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