ISSN 2997-1036
International Journal of Hematology | Vol. 2, No. 2, February 2011 | pp. 9–16
DOI: 10.46882/2011/IJH/000014
Review Article
Title: Current perspectives on the pathophysiology and management of acute chest syndrome in sickle cell disease
Names of Authors: T. M. Ahmed¹, V. I. Nnamdi²
Authors’ Affiliations: ¹Department of Haematology, Aminu Kano Teaching Hospital, Kano, Nigeria; ²Department of Paediatrics, University of Nigeria Teaching Hospital, Enugu, Nigeria
Abstract: Acute chest syndrome remains a leading cause of mortality and intensive care admission among individuals with sickle cell disease. This review integrates contemporary understanding of its complex, multi-factorial pathophysiology, which involves pulmonary microvascular occlusion, fat embolism from necrotic bone marrow, and secondary atypical infectious pathogens. High-affinity hemoglobin interactions, endothelial selectin upregulation, and free plasma hemoglobin from intravascular hemolysis drive pulmonary inflammatory cascades. Management paradigms emphasize early identification using bedside pulse oximetry and rapid initiation of empiric broad-spectrum antibiotics, including macrolides. Incentive spirometry reduces pulmonary splinting and prevents atelectasis progression. For severe cases characterized by worsening hypoxemia (PaO₂ < 60 mmHg) or multi-lobar infiltrates, automated erythrocytapheresis is preferred over simple transfusion to rapidly reduce sickle hemoglobin levels below 30% without expanding total blood volume. This review outlines targeted operational protocols to reduce critical care length of stay and improve clinical survival.
Keywords: Sickle cell disease, acute chest syndrome, exchange transfusion, pulmonary inflation, endothelial activation
Manuscript Timeline: Received: November 11, 2010; Revised: December 20, 2010; Accepted: January 08, 2011; Published: February 15, 2011