Sickle Cell Pathophysiology Research Articles

The not-so-simple process of sickle cell vasoocclusion.

Traditional concepts of sickle cell disease as a monogenically inherited disorder that is understood completely on the basis of polymerization based pathophysiology are more simple that what clinical observations allow. Detailed explications of the determinants of polymerization can be counted, but these do not account for all aspects of sickle cell disease. Neither can all perturbations that count in the course of sickle cell disease be counted as determinants of polymerization. The polymerization based theory that has been extrapolated to describe clinical disease often is not identical to clinical reality. Although contemporary understandings of sickle cell pathophysiology have been described as crazy by those bound to traditional polymerization based understandings, increasingly iconoclastic, seemingly crazy notions are regularly providing important new understandings of sickle cell disease. One of the major challenges to contemporary investigators is to describe new scientific insights in a way that can be understood by others, particularly those reluctant to afford polymerization independent discoveries validity among the interdependent processes that account for sickle cell disease.

Platelet activation in patients with sickle cell disease.

Vascular occlusion and vasculopathy underlie much of the morbidity in patients with sickle cell anaemia. Platelets may play a role in this vasculopathy. Samples from 43 patients with sickle cell disease (SCD) were examined for evidence of platelet activation using fluorescent-labelled monoclonal antibodies and flow cytometry. There was increased expression of activation-dependent antigens on the platelets from patients with SCD compared to those from both Caucasian and African-American controls. In addition, SCD patients had increased levels of platelet microparticles. Platelets are activated in patients with sickle cell disease. The contribution of platelet activation to sickle cell pathophysiology is under active investigation in our laboratories.

Sickle Cell Anemia 1910-1973

It seems fitting that in 1973, the Archives should undertake to report to American physicians an evaluation of the current status of knowledge concerning sickle cell anemia. Sixty-three years ago in 1910, Dr. Herrick, a Chicago cardiologist, used the sixth volume of the Archives to place into the literature the first reported case of the disease; he described a West Indian student with "peculiar elongated and sickle cell shaped red blood corpuscles," a cardiac murmur, and severe anemia.¹During the six decades that have ensued since this first report, a large body of knowledge has amassed concerning the clinical description of sickle cell anemia, the pathology and pathophysiology of the disease, the intriguing morphologic features and behavior of the sickle erythrocyte, and more recently, the molecular characteristics and behavior of sickle hemoglobin. Many of the facts contained within this body of knowledge are chronicled and summarized in the articles