TY  - JOUR
A1  - Solimando, Antonio G.
A1  - Palumbo, Carmen
A1  - Pragnell, Mary Victoria
A1  - Bittrich, Max
A1  - Argentiero, Antonella
A1  - Krebs, Markus
T1  - Aplastic anemia as a roadmap for bone marrow failure: an overview and a clinical workflow
JF  - International Journal of Molecular Sciences
N2  - In recent years, it has become increasingly apparent that bone marrow (BM) failures and myeloid malignancy predisposition syndromes are characterized by a wide phenotypic spectrum and that these diseases must be considered in the differential diagnosis of children and adults with unexplained hematopoiesis defects. Clinically, hypocellular BM failure still represents a challenge in pathobiology-guided treatment. There are three fundamental topics that emerged from our review of the existing data. An exogenous stressor, an immune defect, and a constitutional genetic defect fuel a vicious cycle of hematopoietic stem cells, immune niches, and stroma compartments. A wide phenotypic spectrum exists for inherited and acquired BM failures and predispositions to myeloid malignancies. In order to effectively manage patients, it is crucial to establish the right diagnosis. New theragnostic windows can be revealed by exploring BM failure pathomechanisms.
KW  - hematopoietic stem cells
KW  - bone marrow immune-microenvironment
KW  - bone marrow failure
KW  - cytopenia
KW  - aplastic anemia
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-290440
SN  - 1422-0067
VL  - 23
IS  - 19
ER  -