| ID | 148 |
|---|---|
| Name | HEMOLYTIC ANEMIA |
| Cause | Types & causes of hemolytic anemias: A. Congenital: 1. Abnormalities of the red cell membrane: i. Hereditary spherocytosis ii. Hereditary elliptocytosis 2. Abnormalities of the hemoglobin (Hemoglobinopathies): i. Thalassemia ii. Sickle-cell anemia iii. HbC iv. HbD v. HbE 3. Abnormalities of the red cell protective enzymes (Enzymopathies): i. Glucose-6-phosphate dehydrogenase (G6PD) deficiency ii. Pyruvate kinase deficiency iii. Pyrimidine 5 nucleotidase deficiency B. Acquired: 1. Autoimmune hemolytic anemia: i. Warm antibodies (mostly IgG, bind best at 37°C, account for about 80% of cases), ii. Cold antibodies (usually IgM, bind best at 4°C but can bind upto 37°C, account for about 20% of cases). 2. Alloimmune hemolytic anemia: i. Unmatched transfusion of red cells (Hemolytic transfusion reaction), ii. Maternal sensitisation to paternal antigens of fetal cells (Hemolytic disease of the newborn). 3. Non-immune hemolytic anemia: i. Physical trauma to red cells, e.g- a. Mechanical heart valves. b. March (exercise) hemoglobinuria. c. Thermal injury. d. Microangiopathic hemolytic anemia, ii. Infection, e.g- a. Plasmodium falciparum malaria. b. Clostridium perfringens septicemia. iii. Chemical & drugs: a. Drugs, e.g- dapsone & sulfasalazine b. Chemicals, e.g- arsenic gas, copper, chlorates, nitrites, & nitrobenzine derivatives. 4. Paroxysmal nocturnal hemoglobinuria (rare cause of hemolytic anemia). This occurs as in- i. Hypoplastic bone marrow failure, ii. Aplastic anemia, iii. Myelodysplastic syndrome. |
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| Introduction | Anemia which occurs due to increased breakdown of red blood cells (R.B.Cs) called hemolytic anemia. Usually, when hemolysis occurs (due to any cause), bone marrow produces red cells 6-8 folds more by increasing volume of active marrow and releasing reticulocytes prematurely as a compensatory mechanism. If the rate of destruction (hemolysis) exceeds this increased rate of production then anemia develops. The hemolytic anemia usually accompanies hyperbilirubinemia, hemolytic jaundice, excessive stercobilinogen in the stool, excessive urobilinogen in the urine, high reticulocyte count in the blood and sometimes splenomegaly. Bone marrow shows reactive hyperplasia. |
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| Types | Types & causes of hemolytic anemias: A. Congenital: 1. Abnormalities of the red cell membrane: i. Hereditary spherocytosis ii. Hereditary elliptocytosis 2. Abnormalities of the hemoglobin (Hemoglobinopathies): i. Thalassemia ii. Sickle-cell anemia iii. HbC iv. HbD v. HbE 3. Abnormalities of the red cell protective enzymes (Enzymopathies): i. Glucose-6-phosphate dehydrogenase (G6PD) deficiency ii. Pyruvate kinase deficiency iii. Pyrimidine 5 nucleotidase deficiency B. Acquired: 1. Autoimmune hemolytic anemia: i. Warm antibodies (mostly IgG, bind best at 37°C, account for about 80% of cases), ii. Cold antibodies (usually IgM, bind best at 4°C but can bind upto 37°C, account for about 20% of cases). 2. Alloimmune hemolytic anemia: i. Unmatched transfusion of red cells (Hemolytic transfusion reaction), ii. Maternal sensitisation to paternal antigens of fetal cells (Hemolytic disease of the newborn). 3. Non-immune hemolytic anemia: i. Physical trauma to red cells, e.g- a. Mechanical heart valves. b. March (exercise) hemoglobinuria. c. Thermal injury. d. Microangiopathic hemolytic anemia, ii. Infection, e.g- a. Plasmodium falciparum malaria. b. Clostridium perfringens septicemia. iii. Chemical & drugs: a. Drugs, e.g- dapsone & sulfasalazine b. Chemicals, e.g- arsenic gas, copper, chlorates, nitrites, & nitrobenzine derivatives. 4. Paroxysmal nocturnal hemoglobinuria (rare cause of hemolytic anemia). This occurs as in- i. Hypoplastic bone marrow failure, ii. Aplastic anemia, iii. Myelodysplastic syndrome. |
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