Hemolytic anemia

Introduction Hemolytic anemia is a benign disease that refers to an anemia that occurs when red blood cell destruction is accelerated and bone marrow hematopoietic function is insufficiently compensated. The bone marrow has 6-8 times of compensatory capacity for normal hematopoiesis. If the bone marrow increases erythropoiesis and is sufficient to compensate for the shortened survival of red blood cells, anemia will not occur. This state is called "hemolysis" or compensatory hemolytic disease. Hemolytic anemia is often accompanied by jaundice, called "hemolytic jaundice"

Cause

1. Erythrocyte membrane abnormality

(1) hereditary erythrocyte membrane defects, such as hereditary spherocytosis, hereditary ellipsoidosis, hereditary atrophy, hereditary orthostatic syndrome.

(2) Acquired blood cell membrane glycosylated inositol phospholipid (GPI) anchor membrane protein abnormalities, such as paroxysmal nocturnal hemoglobinuria (PNH).

2. Hereditary erythrocyte enzyme deficiency

(1) Defects in the pentose phosphate pathway enzyme, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency.

(2) anaerobic glycolysis pathway enzyme defects, such as pyruvate kinase deficiency, in addition, nucleoside metabolic enzymes, oxidoreductase and other defects can also lead to hemolytic anemia.

3. Hereditary globin production barrier

(1) The globin peptide chain structure is abnormally unstable hemoglobin disease, hemoglobin disease S, D, E and the like.

(2) The number of globin peptide chains is abnormal for thalassemia.

an examination

Related inspection

Erythropoietin D-dimer glucose-6-phosphate dehydrogenase anti-human globulin test deferoxamine test

1. Detailed medical history to understand whether there are external factors such as physical, mechanical, chemical, infection and blood transfusion that cause hemolytic anemia. If there is a history of family anemia, it suggests the possibility of hereditary hemolytic anemia.

2. There are clinical manifestations of acute or chronic hemolytic anemia. Laboratory tests have the basis of laboratory tests for increased red blood cell destruction or hemoglobin degradation, erythroid compensatory hyperplasia and shortening of red blood cell defect life, and there is anemia. Diagnose hemolytic anemia.

3. Hemolysis mainly occurs in the blood vessels, suggesting that heterotypic transfusion, PNH, paroxysmal cold hemoglobinuria and other hemolytic anemia may be larger, hemolysis mainly occurs outside the blood vessels, suggesting autoimmune hemolytic anemia, erythrocyte membrane, enzyme There is a greater chance of hemolytic anemia caused by abnormal hemoglobin.

4. Anti-human globulin test (Coombs test) positive patients consider warm antibody type autoimmune hemolytic anemia, and further determine the cause. The negative ones considered the warm-antibody type autoimmune hemolytic anemia that was negative for the 1Coombs test, and the other non-autoimmune other hemolytic anemia.

Differential diagnosis

The following clinical manifestations are easily confused with hemolytic anemia:

1 anemia and reticulocyte increase: such as hemorrhage, iron deficiency or early recovery of megaloblastic anemia.

2 non-bilirubin urinary jaundice: such as familial non-hemolytic jaundice (Gilbert syndrome, etc.).

3 young erythrocytic anemia with mild reticulocyte enlargement: such as bone marrow metastasis. Although the above situation is similar to hemolytic anemia, the essence is not hemolysis, lack of evidence for three aspects of laboratory diagnosis of hemolysis, so it is easy to identify. Ineffective red blood cell formation with both anemia and non-bilirubin urinary jaundice, is a special extravascular hemolysis, should be noted.

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