Hemolytic anemias present interesting diagnostic and therapeutic challenges due to their rarity and multiplicity of causes. This session will focus on diagnosis, pathophysiology and treatment of a variety of hemolytic anemias not caused by hemoglobinopathies.
Dr. Robert Brodsky will discuss a variety of complement-driven hemolytic anemias including atypical hemolytic uremic syndrome, HELLP syndrome, and paroxysmal nocturnal hemoglobinuria. He will also discuss indications for therapy, need for continuation of therapy, and the next generation of exciting complement inhibitors in clinical development.
Dr. Mohandas Narla will summarize recent advances in the diagnosis and clinical management of inherited red cell membrane disorders. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), while altered membrane transport function accounts for hereditary overhydrated stomatocytosis and hereditary xerocytosis. The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Splenectomy is not beneficial in these latter two membrane transport disorders and in fact contraindicated due to severe post-splenectomy thrombotic complications.
Dr. Anita Hill will discuss both warm autoimmune hemolytic anemias and cold agglutinin disease. Complications, such as thrombosis, are underestimated and contribute to morbidity as well as mortality. Guidelines are now available for the first time and a pathway for diagnosis and management will be discussed. The future for patients with primary cold agglutinin disease is promising with the trial results using a complement inhibitor in this disease.
Robert Brodsky, MD
Mohandas Narla, DSc
Anita Hill, MD, PhS
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