Anemia Pathologies Overview
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Questions and Answers

What is the medical term for a reduction in the total circulating red cell mass?

Anemia

What is the pathology of Acute Blood Loss?

Loss of intravascular volume

What causes shortened red cell lifespan?

Hemolytic anemias

What is the pathology of Hereditary Spherocytosis?

<p>Defect in RBC cell membrane</p> Signup and view all the answers

What can cause reduced ability of RBCs to protect themselves against oxidative injuries?

<p>Glucose-6-Phosphate Dehydrogenase Deficiency</p> Signup and view all the answers

What is the pathology of Sickle Cell Disease?

<p>Deoxygenated hemoglobin, red cell distortion, vascular occlusion</p> Signup and view all the answers

What is the pathology of Thalassemia?

<p>Defect in globin synthesis cause decreased red cell production and life span</p> Signup and view all the answers

What is the pathology of Thalassemia trait?

<p>Defect in globin synthesis cause decreased red cell production and life span</p> Signup and view all the answers

What is the pathology of Hemoglobin H?

<p>Moderate to severe anemia</p> Signup and view all the answers

What is the pathology of Hydrops Fetalis?

<p>Most severe form of a-thalassemia</p> Signup and view all the answers

What is the pathology of Paroxysmal Cold Hemaglobulinuria?

<p>Presents often in children following infection to measles, mumps, CMV, EBV, Varicella, influenza.</p> Signup and view all the answers

What is the pathology of Paroxysmal Nocturnal Hemoglobinuria?

<p>Hemolysis due to RBC membrane defect increased susceptibility to complement mediated lysis</p> Signup and view all the answers

What is the pathology of Immunohemolytic Anemia?

<p>Antibodies bound to RBC lead to destruction</p> Signup and view all the answers

What is the pathology of Pernicous Anemia?

<p>Megaloblastic anemia</p> Signup and view all the answers

What is the pathology of Aplastic Anemia?

<p>Pancytopenia</p> Signup and view all the answers

What is the pathology of Pure Red Cell Aplasia?

<p>Red cell progenitors are completely absent from marrow or suppressed</p> Signup and view all the answers

What is the pathology of Polycythemia vera?

<p>Myeloproliferative disorder</p> Signup and view all the answers

What is the main effect of blood loss, hemolysis, hereditary, immunohemolytic?

<p>Low oxygen transport capacity of blood</p> Signup and view all the answers

What are the diagnostic tests for blood loss, hemolysis, hereditary, immunohemolytic?

<p>RBC count, Hgb, Hct, RBC indices</p> Signup and view all the answers

What are the diagnostic tests for internal or external bleeding?

<p>RBC count, Hgb, Hct, RBC indices</p> Signup and view all the answers

Which diagnostic tests are used for iron deficiency?

<p>RBC count, Hgb, Hct, RBC indices</p> Signup and view all the answers

What are the diagnostic tests for intravascular hemolysis?

<p>RBC count, Hgb, Hct, RBC indices</p> Signup and view all the answers

Flashcards

Anemia

Reduction of total circulating red cell mass.

Acute blood loss

Loss of intravascular volume due to bleeding.

Chronic blood loss

Ongoing loss of blood over time, leading to anemia.

Hemolytic anemias

Shortened life span of red blood cells leading to anemia.

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Hereditary Spherocytosis

Defect in RBC cell membrane causing spherical red cells.

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G6PD Deficiency

Impaired enzyme function in RBCs leading to oxidative damage.

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Sickle Cell Disease

Deoxygenated hemoglobin distorts RBCs causing vaso-occlusion.

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Thalassemia

Defect in globin synthesis causing decreased red cell production.

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Pernicious Anemia

Megaloblastic anemia due to vitamin B12 deficiency.

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Aplastic Anemia

Pancytopenia due to insufficient production of RBCs, WBCs, and platelets.

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Polycythemia Vera

Myeloproliferative disorder leading to increased red blood cell production.

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Internal bleeding

Bleeding within the body leading to decreased blood pressure.

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External bleeding

Visible bleeding leading to reduced blood volume.

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Intravascular hemolysis

Destruction of red blood cells within blood vessels.

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Hemoglobin H

Moderate to severe anemia due to abnormal hemoglobin synthesis.

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Hydrops Fetalis

Most severe form of alpha-thalassemia in fetuses.

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Paroxysmal Nocturnal Hemoglobinuria

Hemolysis due to RBC membrane defect and complement susceptibility.

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Immunohemolytic anemia

Destruction of RBCs due to antibodies binding to them.

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Sickle-shaped RBCs

RBCs shaped like sickles leading to various complications.

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Hemoglobin electrophoresis

Test used to identify different types of hemoglobin in blood.

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Reticulocyte count

Measure of young red blood cells in blood; indicates production rate.

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Direct Coombs test

Test for antibodies bound to RBCs in hemolytic anemia.

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Vitamin B12 deficiency causes

Megaloblastic anemia from poor intake or absorption of B12.

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Folic acid deficiency

Anemia due to lack of folate, crucial for RBC production.

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Blood transfusion

Replacement of lost blood to restore blood volume and components.

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Iron supplement

Supplement to combat iron deficiency anemia.

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Splenectomy

Surgical removal of the spleen; may help in certain blood disorders.

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Therapeutic phlebotomy

Removal of blood to treat conditions like polycythemia vera.

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Bone marrow transplantation

Replacement of dysfunctional bone marrow to treat blood disorders.

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Study Notes

Anemia Pathologies

  • Anemia is a reduction in the total circulating red blood cell mass.
  • Acute blood loss leads to a loss of intravascular volume.
  • Chronic blood loss results in a shortened red blood cell lifespan.
  • Hemolytic anemias are characterized by a shortened red blood cell lifespan.
  • Hereditary spherocytosis involves defects in the red blood cell (RBC) membrane.
  • Glucose-6-Phosphate Dehydrogenase Deficiency results in deficient or impaired enzyme function, reducing RBC's ability to protect themselves against oxidative injury.
  • Sickle Cell Disease is caused by deoxygenated hemoglobin, leading to red blood cell distortion and vascular occlusion.

Other Anemias

  • Thalassemia is caused by a defect in globin synthesis, decreasing red blood cell production and lifespan.
  • Thalassemia trait also involves a defect in globin synthesis, decreasing red blood cell production and lifespan.
  • Hemoglobin H leads to moderate to severe anemia.
  • Hydrops Fetalis is the most severe form of alpha-thalassemia. It often presents in children following infections like measles, mumps, CMV, EBV, and varicella.
  • Paroxysmal Cold Hemoglobinuria involves hemolysis due to RBC membrane defects, increased susceptibility to complement-mediated lysis.
  • Immunohemolytic anemia is caused by antibodies bound to RBCs, leading to their destruction.

Other Blood Disorders

  • Pernicious anemia (Aplastic anemia) is a megaloblastic anemia.
  • Pernicious anemia (Aplastic anemia) is characterized by pancytopenia, where red cell progenitors are completely absent or suppressed from the bone marrow.
  • Pure Red Cell Aplasia is characterized by the complete absence of red cell progenitors from the bone marrow.
  • Polycythemia Vera is a myeloproliferative disorder.

Causes and Effects of Red Blood Cell Disorders

  • Blood loss, hemolysis, hereditary, immunohemolytic causes a reduction in the oxygen-carrying capacity of blood.
  • Internal or external bleeding can lead to decreased blood pressure, and potentially cardiovascular collapse, shock, and/or death.
  • Rate of blood loss exceeding production results in iron deficiency.
  • Destruction of red blood cells can cause intravascular hemolysis.
  • Inherited disorders, like Parovirus infection can lead to a decreased lifespan of affected RBCs, ranging from 10 to 20 days compared to a normal 100-120 days. They often result in moderate splenomegaly.
  • Abnormalities in the hexose monophosphate shunt or glutathione metabolism cause intravascular and extravascular hemolysis, increasing susceptibility to complement-mediated lysis.
  • Point mutations/defects in globin genes can cause hemoglobin defects, natural protections against malaria, and chronic hemolysis or splenomegaly.

Diagnostic Tests

  • RBC count, Hgb, Hct, RBC indices: are used to diagnose red blood cell disorders.
  • Hemoglobin electrophoresis: measures the different forms of hemoglobin.
  • Direct Coombs test: assesses antibodies bound to RBCs.
  • Urinalysis: aids in evaluating red blood cell conditions.

Treatments

  • Blood transfusion: is used to replace lost blood.
  • Iron supplementation: helps address issues with iron deficiency.
  • Splenectomy: removal of the spleen
  • Intrauterine transfusion: used in cases of severe fetal disease.
  • Hematopoietic stem cell transplantation: a treatment option for certain conditions.
  • Therapeutic phlebotomy: used to remove excess blood.

Additional Notes

  • Inherited deletions of alpha-globin chains can lead to inadequate hemoglobin production.
  • Severity of alpha-thalassemia often depends on the number of genes affected.
  • Mutations in the phosphatidylinositol glycan group A gene (PIGA) can cause X-linked genetic defects.
  • Specific laboratory tests, such as hemoglobin electrophoresis or direct Coombs tests, can offer further diagnostic insights into different types of anemia.
  • Certain conditions, like Vitamin B12 deficiency, can affect RBC production, potentially resulting in anemia.
  • The presence of warm or cold antibodies in the blood can lead to red blood cell destruction.
  • Red blood cell abnormalities, such as microcytosis or hypochromia, may be apparent in blood smears in certain conditions.
  • Pathologies associated with RBCs can be prevalent in specific populations.

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Description

This quiz covers various types of anemia, including their causes, effects, and related pathologies. You will learn about acute and chronic blood loss, hemolytic anemias, and hereditary conditions such as sickle cell disease and thalassemia. Test your understanding of these important topics in hematology.

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