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![Mechanism [Pathophysiology]
Intravascular and Extravascular
Intravascular hemolysis is the destruction of red blood cells in the circulation with
the release of cell contents into the plasma.
Mechanical trauma from a damaged endothelium, complement fixation and activation on the cell
surface, and infectious agents may cause direct membrane degradation and cell destruction.
The more common extravascular hemolysis is the removal and destruction of red blood
cells with membrane alterations by the macrophages of the spleen and liver.
A normal 8-micron red blood cell can deform itself and pass through the 3-micron openings in the
splenic cords. Red blood cells with structural alterations of the membrane surface (including
antibodies) are unable to traverse this network and are phagocytosed and destroyed by
macrophages.](https://image.slidesharecdn.com/hemolyticanemiatt-200904160251/75/Hemolytic-Anemia-8-2048.jpg)
![Work up
CBC [anemia]
Reticulocytosis
Indirect
Hyperbilirubinemia
LDH
Haptoglobin
Blood picture
Normochromic normocytic
Hypochromic | target cells
Microcytic |
Spherocytosis
Schistocytosis
Sickle cells
anisocytosis, | Polychromasia
RBC indices
RDW
Differential counts
Platelet count
?
Heinz bodies | Bite cells
History + Examination](https://image.slidesharecdn.com/hemolyticanemiatt-200904160251/75/Hemolytic-Anemia-11-2048.jpg)
![LDH
Lactate dehydrogenase (LDH) is rich in RBC
Serum LDH elevation is a criterion for hemolysis.
LDH elevation is sensitive for hemolysis, but is not specific since LDH
ubiquitous and can be released from neoplastic
cells, the liver, or from other damaged organs.
Increase in LDH isozymes 1 and 2 is more specific
[DD -myocardial infarction]](https://image.slidesharecdn.com/hemolyticanemiatt-200904160251/75/Hemolytic-Anemia-13-2048.jpg)
Uploaded byTheekshana Wijesinghe
Hemolytic Anemia
Hemolytic anemia is characterized by the premature destruction of erythrocytes, leading to a compensatory type of anemia where the bone marrow cannot maintain red blood cell levels. It can be hereditary or acquired, with causes including immune-mediated, mechanical disruptions, infections, and various underlying diseases. Clinical manifestations include anemia, jaundice, splenomegaly, and potential renal injury, with diagnostic workup focusing on blood tests indicating hemolysis and the underlying cause.
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Introduction to hemolytic anemia, its general definition and epidemiology. Includes classification of anemia types.
Categorization of hemolytic anemias into hereditary and acquired causes, detailing specific etiologies.
Describes intravascular and extravascular hemolysis mechanisms, as well as recent updates on causes.
Common clinical manifestations of hemolytic anemia, including anemia symptoms, jaundice, and related conditions.
Essential tests and indicators for diagnosing hemolytic anemia, including CBC, indirect bilirubin, LDH, and haptoglobin.
Detailed blood picture findings, autoimmune hemolytic anemia characteristics, and tests for further evaluation.
Discussion focused on anisocytosis, sickle cells, and G6PD deficiency, detailing specific disorders.Sources and references used in the presentation, as well as a thank you note to the audience.
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Hemolytic Anemia
- 1. HEMOLYTIC ANEMIA CAUSES, CONSEQUENCESAND WORK UP Dr.GT Wijesinghe, MBBS
- 2. What is HEMOLYTICANEMIA ? • Hemolysis is the premature destruction of erythrocytes • A hemolytic anemia will develop if bone marrow activity cannot compensate for the erythrocyte loss Normal RBC 120 days Defective RBC < 120 days
- 3. Epidemiology • Hemolytic anemiarepresents approximately 5% of all anemias • Hemolytic anemias are not specific to any race Except ; Sickle cell disorders are found primarily in Africans, African Americans, some Arabic peoples, and Aborigines in southern India • AGE : hemolytic anemia can occur in persons of any age hereditary disorders are usually evident early in life. AIHA is more likely to occur in middle-aged and older individuals • SEX : Most cases of hemolytic anemia are not sex specific. Except ; AIHA is slightly more likely to occur in females than in males. G6PD deficiency is an X-linked recessive disorder. Therefore, males are usually affected, and females are carriers.
- 4. Causes Classification ? Hereditary vsAcquired Intravascular vs Extravascular The etiologies of hemolysis often are categorized as Hereditary or Acquired The mechanisms of hemolysis are explained as Intravascular and Extravascular
- 5. Hereditary Causes Enzymopathies Membranopathies Hemoglobinopathies G6PD deficiency HereditarySpherocytosis Thalassemia | Sickle cell diseases
- 6. Acquired Causes Immune mediatedAntibodies to RBC membrane surface antigens Microangiopathic Mechanical disruption of RBC in circulation Infection Idiopathic | Malignancy | Drugs| Autoimmune disorders | Transfusion | Infections Prosthetic valves TTP | DIC | HUS Pre-eclampsia | Eclampsia Malignant Hypertension Malaria | Clostridium Sp.
- 7. Updates • Recent articleshave noted that ; - intravenous immunoglobulin G (IVIG) therapy given during pregnancy - the contrast medium iomeprol - mitral valve replacement can cause hemolysis. • AIHA is rare in children and has a range of causes. Autoimmune hemolysis can be primary or secondary to conditions such as infections (viral, bacterial, and atypical), systemic lupus erythematosus (SLE), autoimmune hepatitis (AIH), and H1N1- influenza.
- 8. Mechanism [Pathophysiology] Intravascular andExtravascular Intravascular hemolysis is the destruction of red blood cells in the circulation with the release of cell contents into the plasma. Mechanical trauma from a damaged endothelium, complement fixation and activation on the cell surface, and infectious agents may cause direct membrane degradation and cell destruction. The more common extravascular hemolysis is the removal and destruction of red blood cells with membrane alterations by the macrophages of the spleen and liver. A normal 8-micron red blood cell can deform itself and pass through the 3-micron openings in the splenic cords. Red blood cells with structural alterations of the membrane surface (including antibodies) are unable to traverse this network and are phagocytosed and destroyed by macrophages.
- 9. Consequences • Anemia Most commonclinical presentation Tachycardia, dyspnea, angina, and weakness • Jaundice Bilirubin gallstone leading to abdominal pain • Splenomegaly | Hepatomegaly • Iron deficiency In intravascular hemolysis, iron deficiency due to chronic hemoglobinuria can exacerbate anemia and weakness • Hematosiderosis Bronze skin color and diabetes occur in Iron overload
- 10. Continued • Hemoglobinuria |Hemosiderinuria • Leg ulcers • Folate deficiency In patients with chronic hemolytic anemia, increased folate consumption may lead to folate deficiency. Clinical manifestations may include patchy hyperpigmentation, sore tongue, and gastrointestinal symptoms • Renal injury patients with thrombotic thrombocytopenic purpura (TTP) may experience fever, neurologic signs, renal failure, and thrombocytopenia
- 11. Work up CBC [anemia] Reticulocytosis Indirect Hyperbilirubinemia LDH Haptoglobin Bloodpicture Normochromic normocytic Hypochromic | target cells Microcytic | Spherocytosis Schistocytosis Sickle cells anisocytosis, | Polychromasia RBC indices RDW Differential counts Platelet count ? Heinz bodies | Bite cells History + Examination
- 12. Indirect Hyperbilirubinemia Unconjugatedbilirubin is a criterion for hemolysis, but it is not specific elevated indirect bilirubin level also occurs in Gilbert disease With hemolysis, the level of indirect bilirubin usually is less than 3 mg/dL Higher levels of indirect bilirubin indicate compromised hepatic function or cholelithiasis
- 13. LDH Lactate dehydrogenase(LDH) is rich in RBC Serum LDH elevation is a criterion for hemolysis. LDH elevation is sensitive for hemolysis, but is not specific since LDH ubiquitous and can be released from neoplastic cells, the liver, or from other damaged organs. Increase in LDH isozymes 1 and 2 is more specific [DD -myocardial infarction]
- 14. Haptoglobin A lowserum haptoglobin level is a criterion for moderate-to-severe hemolysis Haptoglobin binds free hemoglobin released from RBC with high affinity and thereby inhibits its oxidative activity. The haptoglobin-hemoglobin complex will then be removed by the reticulo endothelial system. Useful in differentiation of intravascular over extravascular hemolysis. However, it is an acute phase reactant. Therefore, haptoglobin levels can be normal or elevated despite significant hemolysis in patients with infections and in other reactive states Severe intravascular hemolysis may lead to hemoglbinuria as haptoglobin can not compensate enough
- 15. Blood picture • Anisocytosis- Red blood cells are of unequal size • Polychromasia - Abnormally high number of immature red blood cells in peripheral smear • Spherocytes DAT positive Direct comb’s test negative Immune disorders Transfusions Hereditary Spherocytosis • Schistocytosis - Microangiopathic hemolytic anemias • Heinz bodies |Bite cells - G6PD deficiency
- 17. Other helpful labtests… 1. Urine free hemoglobin test 2. Urine hemosiderin test 3. Red blood cell survival test 4. Cold agglutinin titer 5. Glucose-6-phosphate dehydrogenase (G6PD) screen 6. Sickle cell screen 7. Hemoglobin electrophoresis 8. Micro RNA analysis (for CLL) 9. Immuno-radiometric assay (IRMA)
- 18. Auto Immune HemolyticAnemia Immune hemolytic anemias are mediated by antibodies directed against antigens on the red blood cell surface Microspherocytes on a peripheral smear and a positive direct antiglobulin test are the characteristic findings Immune hemolytic anemia Auto-immune Allo-immune Drug induced Warm antibodies cold antibodies transfusion
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