Aplastic Anemia Overview
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Questions and Answers

Which factors are associated with secondary acquired aplastic anemia?

  • Genetics and family history
  • Chemicals, viruses, and certain medications (correct)
  • Nutritional deficiencies
  • Radiation exposure and environmental toxins

Which of the following correctly characterizes the features of Fanconi Anemia?

  • Characterized by an excess of red blood cells
  • Increased risk of heart disease and diabetes
  • Primarily affects the lungs and respiratory system
  • Physical abnormalities, cancer susceptibility, and aplastic anemia (correct)

Which of the following is NOT a common physical abnormality associated with Fanconi Anemia?

  • Microcephaly
  • Amputation of limb (correct)
  • Café-au-lait lesions
  • Scoliosis

What is the most frequently implicated drug in acquired aplastic anemia?

<p>Chloramphenicol (D)</p> Signup and view all the answers

Which of the following tests is used for diagnosing Paroxysmal Nocturnal Hemoglobinuria (PNH)?

<p>Ham's Acidified Serum Test (A)</p> Signup and view all the answers

What is the primary cause of Microcytic, Hypochromic Anemias?

<p>Insufficient iron level for erythropoiesis (B)</p> Signup and view all the answers

What defines the main characteristic of acquired aplastic anemia cases?

<p>About 80-85% of cases are acquired with no known cause (D)</p> Signup and view all the answers

Which of the following statements correctly describes the nature of inherited aplastic anemia?

<p>Includes diseases like Dyskeratosis congenita and Fanconi Anemia (A)</p> Signup and view all the answers

Flashcards

Acquired Aplastic Anemia

A type of aplastic anemia (80-85% of cases) where the bone marrow's ability to make blood cells fails, often due to an unknown cause (idiopathic) or a specific trigger (secondary), such as chemicals, a virus or drugs.

Inherited Aplastic Anemia

A type of aplastic anemia (15-20% of cases) linked to specific genetic conditions, affecting the bone marrow's production of blood cells.

Fanconi Anemia (FA)

The most common inherited aplastic anemia, with chromosome instability leading to bone marrow failure and an increased risk of cancers and certain physical features.

Paroxysmal Nocturnal Hemoglobinuria (PNH)

A blood disorder where red blood cells are destroyed prematurely, often at night (hence the name), due to missing proteins (DAF and MIRL) that protect them.

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Microcytic, Hypochromic Anemia

A form of anemia characterized by small, pale red blood cells, often caused by insufficient iron.

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DAF (decay-accelerating factor)

A protein that protects red blood cells from being destroyed by the immune system.

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MIRL (membrane inhibitor of reactive lysis)

A protein that protects red blood cells from being destroyed by the immune system

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Study Notes

Acquired Aplastic Anemia

  • Accounts for approximately 80-85% of aplastic anemia cases
  • Two categories:
    • Idiopathic: no known cause
    • Secondary: associated with a known cause (e.g., chemicals, viruses, drugs; chloramphenicol is a frequent cause)

Inherited Aplastic Anemia

  • Accounts for approximately 15-20% of aplastic anemia cases
  • Associated diseases:
    • Dyskeratosis congenita
    • Shwachman-Bodian-Diamond syndrome
    • Fanconi Anemia (FA)

Fanconi Anemia (FA)

  • Most common inherited aplastic anemia
  • Chromosome instability disorder
  • Characterized by: aplastic anemia, cancer susceptibility, and physical abnormalities
    • Skeletal abnormalities (e.g., thumb malformations, microcephaly, scoliosis)
    • Skin pigmentation abnormalities (e.g., hyperpigmentation, hypopigmentation, café-au-lait lesions)
    • Short stature
    • Abnormalities in the eyes, kidneys, and genitals

Paroxysmal Nocturnal Hemoglobinuria (PNH)

  • Also known as Marchiafava-Micheli Syndrome
  • Caused by deficiency of:
    • DAF (decay-accelerating factor) or CD55
    • MIRL (membrane inhibitor of reactive lysis) or CD59

Microcytic, Hypochromic Anemias

  • Characterized by small red blood cells with central pallor, and decreased MCV and MCHC
  • Results from insufficient iron levels for erythropoiesis
  • Abnormal iron studies
  • Early stages may show reduced iron stores, but not always a full-blown anemia

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Description

This quiz covers the two primary types of aplastic anemia: acquired and inherited. It delves into the causes, associated syndromes, and specific characteristics of Fanconi Anemia and Paroxysmal Nocturnal Hemoglobinuria. Test your knowledge on these critical hematological conditions.

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