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Acquired aplastic anemia: bone marrow histology complemented by immunohistochemistry in identifyng unfavorable prognosis, defined by progression to MDS/AML- a comparison between children and adults

Grant number: 14/01149-0
Support type:Regular Research Grants
Duration: October 01, 2014 - March 31, 2017
Field of knowledge:Health Sciences - Medicine
Principal Investigator:Maria Cláudia Nogueira Zerbini
Grantee:Maria Cláudia Nogueira Zerbini
Home Institution: Faculdade de Medicina (FM). Universidade de São Paulo (USP). São Paulo , SP, Brazil
Assoc. researchers: Elvira Deolinda Rodrigues Pereira Velloso ; Marlene Pereira Garanito ; Raquel Ferrari Marchesi ; Raymundo Soares de Azevedo Neto ; Sheila Aparecida Coelho Siqueira

Abstract

Aplastic anemia (AA) or bone marrow failure, classified as congenital or acquired, is a bone marrow condition characterized by reduced production of red cells, granulocytes and platelets, resulting in pancytopenia in the peripheral blood. A anemia aplástica adquirida (AAA) is more often observed in children and its differential diagnosis includes congenital bone marrow failures and Myelodysplastic syndromes (MDS), which, in this age group, presents frequently hypocellular and is called Refractory cytopenia of childhood (RCC). The relationship between the AAA and RCC, and consequently the differential diagnosis between them, has been the subject of controversy and diagnostic difficulties clinically and morphologically. The importance of this differential diagnosis is due to the difference with respect to prognosis and treatment, since MDS have an increased risk of developing acute myeloid leukemia. The common lack of representative material in BM smears determined by their hypocellularity, makes the biopsy a valuable material for the diagnostic approach in these conditions. This project is retrospective and aims to analyze 52 children's and 129 adults's bone marrow biopsies diagnosed with AAA, referred to Hospital das Clinicas, University of Sao Paulo School of Medicine. All biopsies will undergo immunohistochemistry (IHC) examination in order to identify, among a set of cases of AAA, morphological / IHC criteria to assist in identifying the patients with unfavorable clinical evolution in the two groups, defined as progression to MDS and/or AML . (AU)