Hereditary spherocytosis is a relatively common haematological disorder and will be encountered by all haematologists. The abundance of new information, dealing principally with molecular and genetic aspects of pathophysiology, is beginning to have implications for its investigation and management. While these advances have not yet exerted a large influence at therapeutic level, the promise of ...

As a rare complication in chronic anemic states, the extramedullary hematopoiesis may provide diagnostic and therapeutic challenge. Caused by the insufficiency of the bone marrow with reactivation of quiescent erythropoietic sites, this condition may vary its presentation as a simple radiologic finding to a spontaneous massive haemothorax. In this paper, we report the case of a 61-years-old fem...

Resistance and elastic deformability of red cells are due to a protein network (cytoskeleton) that laminates the lipid bilayer and to proteins that span the latter. All proteins are interconnected. Their structure as well as the structure of the corresponding genes are now well known. Hereditary spherocytosis (HS) is the most common hemolytic anemia due to a red cell membrane defect. It derives...

I HE CONTROL of red cell productiomi is undoubtedly geared toward maintaining the red cell mass at a functionally optimal size. Since the primary function of the red cell mass is to transport oxygen to the tissues, it is generally assumed that the control is triggered by the tissue tension of oxygen. Recent studies indicate that the oxygen tension in one target area, the kidney, is inversely pr...

Erythrocyte protein phosphorylation was from immunologically acquired spheroexamined in membrane preparations of cytosis cells or from cells from patients 25 patients with hereditary spherocytosis with hereditary elliptocytosis. Heating (HS). Reduced phosphorylation in submembranes to 45’C caused negligible strafe polypeptides was observed in 22 inhibition of membrane protein phosHS erythrocyte...

Membrane microfilaments are found throughout the animal world in situations suggesting that they fulfill a critical role in providing normal cell shape and plasticity. We have hypothesized that hereditary spherocytosis, a congenital hemolytic anemia associated with intrinsically rigid and mishapen erythrocytes, might result from genetically defective microfilaments in erythrocyte membranes. By ...