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Ann Diagn Pathol ; 45: 151459, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32000075

RESUMO

Aplastic anemia (AA) is a rare disorder characterized by suppression of bone marrow function, which can progress to myelodysplastic syndrome (MDS) or to acute myeloid leukemia (AML). To determine if there are characteristics in bone marrow biopsies in children and adults previously diagnosed with acquired AA, which could predict progression to MDS, we evaluated 118 hypocellular bone marrow biopsies from adults (76 patients) and children (42) diagnosed initially with acquired AA previously to any treatment. Histology was reviewed according to a detailed protocol including Bennett and Orazi criteria for hypocellular myelodysplastic syndrome (h-MDS) and Bauman et al. criteria for refractory cytopenia of childhood (RCC). Twelve patients (10.2%; 6 children and 6 adults) progressed to MDS after a median time of 56 months. Criteria described by Bennett and Orazi suggestive of h-MDS in bone marrow biopsies were detected in 16 cases (13.5%; 8 adults and 8 children), and none in patients that progressed to MDS/AML. Twenty adults' biopsies (26.3%) had the histological criteria used for the diagnosis of pediatric RCC, and none showed MDS/AML evolution. Ten children (23.8%) were reclassified morphologically as RCC, and only one progressed to MDS. In this population with acquired aplastic anemia (AAA), no histological/immunohistochemical (H/IHC) bone marrow findings could discriminate patients with higher risk for myeloid clonal progression, which questions the diagnosis of h-MDS/RCC based only on the finding of dysplasia in the cases without increased blasts and/or the characteristic genetic abnormalities.


Assuntos
Anemia Aplástica/diagnóstico , Anemia Aplástica/patologia , Medula Óssea/patologia , Síndromes Mielodisplásicas/diagnóstico , Adolescente , Adulto , Idoso , Anemia Aplástica/complicações , Anemia Aplástica/metabolismo , Antígenos CD34/metabolismo , Biópsia , Criança , Pré-Escolar , Citogenética/métodos , Diagnóstico Diferencial , Progressão da Doença , Feminino , Humanos , Imuno-Histoquímica/métodos , Lactente , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/etiologia , Leucemia Mieloide Aguda/mortalidade , Leucemia Mieloide Aguda/terapia , Masculino , Megacariócitos/imunologia , Megacariócitos/patologia , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/etiologia , Síndromes Mielodisplásicas/mortalidade , Síndromes Mielodisplásicas/terapia , Valor Preditivo dos Testes , Adulto Jovem
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