Recent

  • Show all results for ""
Sickle Cell Anemia Overview
40 Questions
0 Views

Sickle Cell Anemia Overview

Created by
@InnocuousWashington

Questions and Answers

What is the main cause of sickle cell anemia?

  • A vitamin deficiency in erythropoiesis
  • Excessive iron deposition in the kidneys
  • An autoimmune attack on red blood cells
  • A single nucleotide base change in DNA (correct)

    • What complication is characterized by painful swelling of the hands and feet in sickle cell anemia?

  • Foot-hand syndrome (correct)
  • Acute chest syndrome
  • Acute sequestration crisis
  • Vaso-occlusive crisis

    • What percentage of patients with sickle cell anemia typically experiences a vaso-occlusive crisis?

  • 50% (correct)
  • 15%
  • 25%
  • 75%

    • Which of the following is NOT a common trigger for sickling of red blood cells?

    <p>High plasma volume</p> Signup and view all the answers

    Which life-threatening condition can develop from acute chest syndrome if not treated immediately?

    <p>Acute respiratory distress syndrome</p> Signup and view all the answers

    What is a serious complication of sickle cell anemia in males that can occur repeatedly?

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

    What is the typical life expectancy reduction for individuals with sickle cell anemia?

    <p>Significantly reduced with significant mortality rate</p> Signup and view all the answers

    What occurs in an aplastic crisis related to sickle cell anemia?

    <p>Profound anemia due to reduced erythropoiesis</p> Signup and view all the answers

    What percentage of total hemoglobin is HbS in a carrier of the sickle cell gene?

    <p>35-40%</p> Signup and view all the answers

    Which imaging technique is indicated for assessing neurologic crises in sickle cell disease?

    <p>Head CT or MRI</p> Signup and view all the answers

    What is the typical appearance of vertebrae seen on radiographs of patients with increased erythropoiesis?

    <p>Fish mouth appearance</p> Signup and view all the answers

    What is the mortality rate of septicemia and meningitis in children with sickle cell anemia during the first 5 years of life?

    <p>25%</p> Signup and view all the answers

    What was the average age of death for female patients with sickle cell anemia?

    <p>48 years</p> Signup and view all the answers

    How has the average life expectancy of people with sickle cell anemia changed in recent years?

    <p>It has started to increase.</p> Signup and view all the answers

    Which finding is observed in radiographs of pediatric patients within 5 weeks of hand-foot syndrome onset?

    <p>Thinning of cortical bone</p> Signup and view all the answers

    What has been a major obstacle in the progress toward successful gene therapy for sickle cell disease?

    <p>Slow development progress</p> Signup and view all the answers

    What is the primary cause of irreversible sickling in red blood cells?

    <p>Injury to the plasma membrane</p> Signup and view all the answers

    Which hematocrit range is typically seen in patients with Sickle Cell Anemia (SCA)?

    <p>18% to 30%</p> Signup and view all the answers

    Which laboratory feature is characteristically elevated in patients with SCA?

    <p>White blood cell count</p> Signup and view all the answers

    What characteristic feature is observed in the peripheral blood smear of a patient with SCA?

    <p>Presence of nucleated RBCs</p> Signup and view all the answers

    What is a typical finding in patients with reduced splenic function related to SCA?

    <p>Presence of target cells</p> Signup and view all the answers

    What percentage of circulating erythrocytes do reticulocytes normally account for?

    <p>2%</p> Signup and view all the answers

    What leads to the influx of calcium ions into red blood cells in the context of irreversible sickling?

    <p>Loss of active membrane transport</p> Signup and view all the answers

    What abnormal characteristic do target cells exhibit?

    <p>Dense center with a pale ring</p> Signup and view all the answers

    What is the primary goal of a blood transfusion in the management of acute chest syndrome?

    <p>To decrease the serum concentration of HbS to 30% or less of total hemoglobin</p> Signup and view all the answers

    Which treatment options are appropriate for managing priapism?

    <p>Analgesics and hydration, with possible surgical drainage if unresolved after 6 hours</p> Signup and view all the answers

    What may be necessary to treat chronic leg ulcers that are not healing properly?

    <p>Use of antibiotics and elevation along with possible skin grafting</p> Signup and view all the answers

    What is the description of an exchange transfusion?

    <p>Introducing whole blood in place of 75-85% of the patient’s total blood volume that is withdrawn in small amounts</p> Signup and view all the answers

    Bone marrow and stem cell transplantation are primarily limited to a small minority of patients due to which of the following reasons?

    <p>The high risk of adverse effects and difficulty in finding suitable donors</p> Signup and view all the answers

    What is a potential complication of bone marrow and stem cell transplantation?

    <p>Graft-versus-host disease</p> Signup and view all the answers

    Which treatment is not commonly indicated for pain crises that do not respond to standard treatments?

    <p>Increased oral hydration</p> Signup and view all the answers

    Which of the following is a key component in the management of an aplastic crisis in patients?

    <p>Blood transfusion</p> Signup and view all the answers

    What is primarily responsible for the sickle shape of red blood cells in sickle cell anemia?

    <p>Presence of hemoglobin S</p> Signup and view all the answers

    Which of the following complications is most directly associated with sickle cell anemia?

    <p>Organ failure due to blood vessel obstruction</p> Signup and view all the answers

    What is a common result of the hemolytic anemia caused by sickle cell disease?

    <p>Accumulation of bilirubin in the body</p> Signup and view all the answers

    Which was the primary symptom described by the physician in the first account of sickle cell anemia?

    <p>Shortness of breath</p> Signup and view all the answers

    Why is sickle cell anemia categorized as an autosomal recessive disease?

    <p>It requires both parents to carry the trait for the disease to manifest.</p> Signup and view all the answers

    What triggers the pathophysiological changes in red blood cells in sickle cell anemia?

    <p>Dehydration and deoxygenation</p> Signup and view all the answers

    What is a significant consequence of the altered shape of red blood cells in individuals with sickle cell anemia?

    <p>Sluggish blood flow</p> Signup and view all the answers

    What is a defining characteristic of hemolytic anemias like sickle cell anemia?

    <p>Premature destruction of red blood cells</p> Signup and view all the answers

    Study Notes

    Sickle Cell Anemia Overview

    • Sickle cell anemia (SCA) is a severe autosomal recessive blood disorder characterized by abnormal hemoglobin (HbS) causing red blood cells (RBCs) to form a sickle shape.
    • Hemolytic anemia occurs due to the premature destruction of RBCs, leading to increased erythropoiesis, iron retention, and bilirubin buildup.
    • SCA is one of three inherited blood disorders within sickle cell disease (SCD).

    Clinical Manifestations

    • Symptoms include severe pain, organ failure, hemolytic anemia, shortness of breath, anemia, palpitations, and icterus.
    • Acute sequestration crisis can lead to painful enlargement of the liver and spleen in infants.
    • Foot-hand syndrome occurs between 6 months and 3 years, characterized by painful swelling in hands and feet.
    • Strokes are common in children and can recur, affecting approximately two-thirds of patients.

    Complications of SCA

    • Vaso-occlusive crisis is a frequent complication in adults, causing severe pain and accounting for 90% of hospital admissions.
    • Acute chest syndrome is a medical emergency, presenting with chest pain, difficulty breathing, and can lead to respiratory distress.
    • Aplastic and hemolytic crises result in profound anemia and high-output heart failure.
    • Priapism, an abnormal prolonged erection, occurs in up to 40% of males with SCA.

    Genetic Basis

    • SCA is caused by a point mutation in the beta-globin chain of hemoglobin, leading to valine substitution for glutamic acid.
    • HbS leads to a significant loss of RBC lifespan (10-20 days) compared to the normal 120 days.

    Pathophysiology

    • Pathophysiological triggers for sickling include low oxygen tension, high hydrogen ion concentration, cold temperature, and dehydration.
    • Sickling is often reversible but can become permanent due to repeated episodes damaging RBC membranes.

    Laboratory Features

    • Hallmark laboratory findings in SCA include a hematocrit of 18%-30%, elevated white blood cell count, and presence of irreversibly sickled cells.
    • Serum bilirubin levels are elevated due to hemolysis, leading to jaundice and potential pigment gallstones.

    Diagnostic Tools

    • Imaging techniques such as CT, MRI, and X-rays can identify complications and monitor organ health.
    • Common X-ray findings in acute chest syndrome show changes in vertebrae and lung structure.

    Management Strategies

    • Treatment includes analgesics, oxygen, antibiotics, and blood transfusions to reduce HbS levels.
    • Aplastic crisis and priapism require appropriate medical interventions, including potential surgical drainage.
    • Pain crises may be managed through exchange transfusions, while bone marrow and stem cell transplants can potentially cure SCA in children, although they carry significant risks.

    Prognosis

    • Life expectancy for SCA patients has improved; average ages of death are 42 for males and 48 for females, though reports of patients living into their 70s exist.
    • Individuals with sickle cell trait usually have a normal life expectancy but may experience complications in extreme conditions.

    Economic and Social Impact

    • SCA contributes to substantial economic loss and disability, with a notable mortality rate before reaching adulthood.
    • Early medical intervention and advances in care continue to improve patient outcomes and quality of life.

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Description

    This quiz covers the characteristics and classifications of sickle cell anemia, a group of inherited blood disorders. Explore its definition, related blood cell destruction, and iron retention issues. Get ready to deepen your understanding of this critical health topic.

    More Quizzes Like This

    Sickle Cell Anemia and Its Traits
    22 questions

    Sickle Cell Anemia and Its Traits

    FascinatingJuxtaposition avatar
    FascinatingJuxtaposition
    Sickle Cell Anemia Quiz
    10 questions

    Sickle Cell Anemia Quiz

    CalmFairy avatar
    CalmFairy
    Medical Quiz on Burger's Disease and Sickle Cell Anemia
    8 questions

    Medical Quiz on Burger's Disease and Sickle Cell Anemia

    DexterousImagery avatar
    DexterousImagery
    Final Exam Practice for Anatomy and Physiology II
    97 questions

    Final Exam Practice for Anatomy and Physiology II

    LowCostHarpy avatar
    LowCostHarpy
    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser