EHA Library - The official digital education library of European Hematology Association (EHA)

Splenectomy and emerging novel treatments in rare inherited hemolytic anemias
Joanne Joanne Yacobovich
Hematology Oncology Division, Hematology Unit, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel
EHA Library. Tamary H. 06/14/19; 273705
Hannah Tamary
Hannah Tamary
Learning Objectives

Achille Iolascon - Chair introduction

The red blood cell membrane is one of the major determinants of the ability of erythrocytes to cross repeatedly through the microcirculation. The skeleton that laminates the inner side of the red cell membrane plays an essential role in determining the shape and deformability of the red cell. Defects and deficiencies in the erythrocyte membrane protein structure have been described in several hereditary and acquired anemias, where they give rise to mechanically and/or thermally unstable erythrocytes with shortened life spans.
Lesley Bruce will illustrate how the human red blood cell membrane is packed with a regular array of proteins and protein complexes attached at intervals to the underlying cytoskeleton. She will demonstrate how proteomic analysis and whole genome sequencing are beginning to provide a better understanding of the structural and functional changes in the membrane through erythropoiesis.
After this part Roberta Russo will show how these proteins are involved in the pathogenesis of a large series of hemolytic anemias. Although the workflow to diagnose RBC membrane disorders is a standard clinical practice, differential diagnosis, classification, and patient stratification among these diseases are often very difficult. She will illustrate how new next-generation sequencing techniques and new proteomic/molecular approach could help in better defining these conditions.
The last part will be on treatments for these inherited anemias. Since the spleen have a major role in removing damaged red cells, Hannah Tamary will discuss on the role of splenectomy as therapeutic option. Moreover, linked to this, she will present associated complications, such as infections and thrombotic events.

Learning goals of the article
• To know the structure and function of the red cell membrane components: mainly proteins.
• To know how to suspect these conditions and how to use new molecular diagnosis tools.
• To know how to manage these conditions, mainly when to suggest the use of splenectomy and what to do before and after.

Learning goals of the presentation
After attending this lecture, the participant will be able to
• gain knowledge on current splenectomy complications,
• appreciate splenectomy indications and contraindications in two rare congenital hemolytic anemias (Pyruvate Kinase Deficiency and Hereditary Stomatocytosis), and
• gain knowledge on possible emerging alternatives to splenectomy in Pyruvated Kinase Deficiency and Hereditary Stomatocytosi.

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