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Editorials
it is a rare condition that often poses a diagnostic dilem- ma with acquired AA potentially confounded by a differ- ential of inherited bone marrow failure syndromes in younger patients and hypoplastic myelodysplasia in older patients. These variations in clinical presentation and potential differences in pathophysiology between pedi- atric and adult patients with AA emphasize the impor- tance of having data sets dedicated to pediatric cases on
which to analysis patterns of presentation, diagnosis and treatment outcome and thereby recommend consensus- driven therapeutic algorithms, particularly in an environ- ment in which there has been substantial historical varia- tions in practice.2
The insights provided in the Rogers et al. analysis pro- vides substantial clarity around several management issues in pediatric AA, but also pose several other ques-
Figure 1. Immune mediated attack on hematopoietic stem cells (HSC) and potential responses to therapy. IST: immunosuppressive therapy; HCT: hematopoietic stem cell transplant.
haematologica | 2019; 104(10)