The Pathophysiology of Acquired Aplastic Anemia: Current Concepts Revisited

Overview

Journal Hematol Oncol Clin North Am

Specialties Hematology
Oncology

Date 2018 Jul 27

PMID 30047412

Citations 38

Authors

Michelle L Schoettler

David G Nathan

Affiliations

Soon will be listed here.

Abstract

Idiopathic acquired aplastic anemia is a rare, life-threatening bone marrow failure syndrome characterized by cytopenias and hypocellular bone marrow. The pathophysiology is unknown; the most favored model is of a dysregulated immune system leading to autoreactive T-cell destruction of hematopoietic stem and progenitor cells in a genetically susceptible host. The authors review the literature and propose that the major driver of acquired aplastic anemia is a combination of hematopoietic stem and progenitor cells intrinsic defects and an inappropriately activated immune response in the setting of a viral infection. Alterations in bone marrow microenvironment may also contribute to the disease process.

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