Κατεύθυνση Θρόμβωση-Αιμορραγία-Ιατρική των μεταγγίσεων
Library of the School of Health Sciences

2019-04-18

2019

Tsionas Ioannis

Βαλσάμη Σερένα, Επίκουρη Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ
Βασιλακόπουλος Θεόδωρος, Αναπληρωτής Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Σιακαντάρη Μαρίνα, Αναπληρώτρια Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ

Αυτοάνοση Αιμολυτική Αναιμία

Greek

Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. One of the most common causes of autoimmune hemolytic anemia is immunologic destruction of red blood cells (RBCs) mediated by IgG autoantibodies reacting against protein antigens on the RBC surface at body temperature 37ο C, a condition called warm type autoimmune hemolytic anemia .AIHA may occur primarily in disease states characterized by abnormalities in the immune system with no specific etiology or may be caused secondarily by congenital abnormalities of the immune system, other acquired autoimmune disorders, lymphoid malignancies or follow certain infections.
AIHA is diagnosed by demonstrating either IgG or a fragment of the third component of complement (C3d) on the surface of the RBC (a positive direct antiglobulin [Coombs] test). Most patients with AIHA present with an acute onset of severe hemolysis with symptomatic anemia, requiring immediate treatment. In the case of warm type autoimmune hemolytic anemia, the use of corticosteroids is effective. On the contrary, cold agglutinin disease is a type of autoimmune hemolytic anemia that may arise secondary to infection or may reflect the presence of an underlying lymphoproliferative disease due to an IgM autoantibody reacting at 3-4ο C. We recommend rituximab as first-line therapy in this disease, with high possibility of responding.
Finally, paroxysmal cold hemoglobinuria (PCH) is caused by an IgG antibody that fixes complement in the cold and causes intravascular hemolysis and hemoglobinuria upon rewarming. Avoiding the cold and additional use of glucocorticosteroids when necessarily are some measures to deal with this disease.

Health Sciences

Warm type autoimmune hemolytic anemia, Intravascular hemolysis, Extravascular hemolysis, Coombs positive test, Direct antiglobulin test, Fragment of the third component of complement (C3d), IgG autoantibodies, IgM autoantibody, Cold agglutinin disease, Paroxysmal cold hemoglobinuria, Rituximab, Glucocorticosteroids

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https://pergamos.lib.uoa.gr/uoa/dl/object/2867851