Approach to hemolytic anemia

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Questions and Answers

Which investigation is the preferred method for diagnosing Paroxysmal Nocturnal Hemoglobinuria (PNH)?

  • Acidified glycerol test
  • Flow cytometry (correct)
  • Ham test
  • Acidified serum test

What condition is suggested by the following factors: presence of anemia, and schistocytes?

  • Iron-deficiency anaemia
  • Aplastic anaemia
  • Megaloblastic anaemia
  • Microangiopathic haemolytic anaemia (correct)

Which of the following conditions does not typically promote sickling in sickle cell anemia?

  • Increased MCHC
  • Infections
  • Dehydration
  • Oxygenation (correct)

In which condition is autosplenectomy typically observed?

<p>Sickle cell anemia (B)</p> Signup and view all the answers

What is the most probable diagnosis for a patient presenting with severe anemia, hepatosplenomegaly, or abnormal facies despite repeated blood transfusions?

<p>Thalassemia major (A)</p> Signup and view all the answers

Which protein is most commonly affected by mutations in Hereditary Spherocytosis?

<p>Ankyrin (B)</p> Signup and view all the answers

In which of the following conditions is intravascular hemolysis the predominant mechanism?

<p>Hemolytic Uremic Syndrome (D)</p> Signup and view all the answers

Which of the following is typically NOT a feature of hemolytic anemia?

<p>Increased Haptoglobin (B)</p> Signup and view all the answers

During hemoglobin electrophoresis, how many bands are typically observed in individuals with sickle cell trait?

<p>2 (C)</p> Signup and view all the answers

A patient presents with hemolytic anemia. Lab results show elevated levels of bilirubin and reticulocytes. Which of the following would you expect to find?

<p>Increased Lactate Dehydrogenase (LDH) (D)</p> Signup and view all the answers

Investigation of choice for thalassemia

<p>Hemoglobin electrophoresis (A)</p> Signup and view all the answers

Hb Barts is composed of

<p>Four gamma chains (C)</p> Signup and view all the answers

Flashcards

PNH Investigation

Flow cytometry is the investigation of choice.

Thalassemia major

A condition primarily affecting individuals of Mediterranean, African, and Southeast Asian descent, resulting in abnormal hemoglobin production and reduced red blood cell survival.

Sickling Precipitation

Sickling is a process that is not precipitated by oxygenation.

Prosthetic Valve Anemia

RBC fragments due to mechanical damage from the valve.

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Hb Barts Composition

Four gamma (γ) chains form Hemoglobin Barts

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Sickle Cell Anemia

A genetic blood disorder causing red blood cells to become rigid and sickle-shaped, leading to various complications.

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Beta Thalassemia Minor

A mild form of beta thalassemia where individuals carry one normal and one abnormal beta-globin gene.

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Microangiopathic Hemolytic Anemia

Hemolytic anemia caused by damage to red blood cells as they pass through small blood vessels, often due to clots or other obstructions.

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HbH Disease

A type of alpha thalassemia where there are deletions of three of the four alpha-globin genes, resulting in the formation of HbH (beta-4) hemoglobin.

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Autosplenectomy

The gradual loss of spleen function due to chronic damage, often seen in conditions like sickle cell anemia.

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Donath-Landsteiner antibody

An antibody specifically associated with Paroxysmal Cold Hemoglobinuria (PCH).

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Intravascular hemolysis

Red blood cell destruction primarily occurs within blood vessels.

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Hemolytic anemia features

Decreased haptoglobin is seen as haptoglobin binds free hemoglobin.

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Sickle cell trait electrophoresis bands

Hemoglobin electrophoresis typically shows two bands: HbA and HbS.

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Hemolytic Uremic Syndrome (HUS) and hemolysis

Red blood cell destruction primarily occurs in Hemolytic Uremic Syndrome (HUS).

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Study Notes

  • Flow cytometry is the investigation of choice for Paroxysmal Nocturnal Hemoglobinuria.
  • Thalassemia major is the most probable diagnosis shown in the image.
  • Oxygenation does not precipitate sickling.
  • Microangiopathic haemolytic anaemia is the probable diagnosis for a patient with a prosthetic heart valve who develops anaemia with schistocytes on peripheral smear.
  • Hb Barts is composed of four y chains.
  • Microangiopathic hemolytic anemia is identified in the peripheral smear picture.
  • Autosplenectomy is seen in sickle cell anemia.
  • Thalassemia is screened using the test shown in the image.
  • Anemia, Hyperkalemia and Renal microthrombi are features of Hemolytic uremic syndrome.
  • Direct Coombs test is positive for hemolytic anemia due to Paroxysmal cold hemoglobinuria.
  • Ankyrin is the most common mutation in Hereditary spherocytosis.
  • Donath Landsteiner antibody is seen in Paroxysmal Cold Hemoglobinuria.
  • Intravascular hemolysis is predominant in Hemolytic uremic syndrome.
  • Increased bilirubin, Increased reticulocytes and Increased urobilinogen are features of hemolytic anemia.
  • In sickle cell trait, the number of bands seen in hemoglobin electrophoresis is 2.

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