Management of patients with Hematologic disorders
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Questions and Answers

What is the most common type of sickle cell disease?

  • Sickle cell anaemia (correct)
  • Sickle cell crisis
  • Sickle cell disorder
  • Sickle cell trait

    • At what age do problems in sickle cell disease typically begin?

  • 1 to 2 years
  • 15 to 18 years
  • 10 to 12 years
  • 5 to 6 months (correct)

    • What causes an attack in people with sickle cell disease?

  • Regular exercise
  • Healthy diet
  • Temperature changes (correct)
  • Adequate sleep

    • How does a person without symptoms of sickle cell disease is referred to?

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

    What is one of the earliest clinical manifestations of sickle cell anemia?

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

    Which condition can result in symptoms of acute chest syndrome in sickle cell anemia patients?

    <p>Painful crisis</p> Signup and view all the answers

    What is the most commonly used treatment during childhood to reduce the risk of severe bacterial infections in sickle cell anemia patients?

    <p>Daily penicillin prophylaxis</p> Signup and view all the answers

    Which condition can lead to stroke in sickle cell anemia patients?

    <p>Silent stroke</p> Signup and view all the answers

    Which complication is associated with excessive bilirubin production due to prolonged haemolysis in sickle cell anemia patients?

    <p>Cholelithiasis (gallstones)</p> Signup and view all the answers

    What is the most common cause of osteomyelitis in sickle cell anemia patients?

    <p>Salmonella typhimurium</p> Signup and view all the answers

    What may occur as a result of ischaemia in sickle cell anemia patients?

    <p>Leg ulcers</p> Signup and view all the answers

    What does decreased immune reactions due to malfunctioning of the spleen result in for sickle cell anemia patients?

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

    What is the most common complication of sickle cell disease?

    <p>Vaso-occlusive crises</p> Signup and view all the answers

    Which type of crisis is characterized by acute painful enlargements of the spleen?

    <p>Splenic sequestration crises</p> Signup and view all the answers

    Which type of crisis is caused by an acute worsening of a patient's baseline anemia, usually due to parvovirus B19 infection?

    <p>Aplastic crises</p> Signup and view all the answers

    What is the second most common complication of sickle cell disease?

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

    What is the protective effect against malaria discovered in 1954?

    <p>Sickle cell trait</p> Signup and view all the answers

    Who first described sickle cell disease in 1910?

    <p>James B. Herrick</p> Signup and view all the answers

    In which year were new gene therapies approved to cure a small percentage of people with sickle cell disease?

    <p>2023</p> Signup and view all the answers

    Where do the majority of sickle cell disease cases occur?

    <p>Sub-Saharan Africa</p> Signup and view all the answers

    What is the mainstay of management for vaso-occlusive crises?

    <p>Pain medication</p> Signup and view all the answers

    What does acute chest syndrome involve?

    <p>Pulmonary infiltrate and hypoxemia</p> Signup and view all the answers

    What causes haemolytic crises?

    <p>Increased breakdown of red blood cells</p> Signup and view all the answers

    What is the main management approach for aplastic crises?

    <p>Supportive management or blood transfusion</p> Signup and view all the answers

    What is the average life expectancy in the developed world for individuals with sickle cell disease?

    <p>40 to 60 years</p> Signup and view all the answers

    What triggers an attack in individuals with sickle cell disease?

    <p>Temperature changes, stress, dehydration, and high altitude</p> Signup and view all the answers

    How is a person with a single abnormal copy of the β-globin gene (HBB) that makes haemoglobin usually described?

    <p>Sickle cell disease carrier</p> Signup and view all the answers

    At what age do problems in sickle cell disease typically begin?

    <p>5 to 6 months of age</p> Signup and view all the answers

    What is the most commonly used treatment during childhood to reduce the risk of severe bacterial infections in sickle cell anemia patients?

    <p>Daily penicillin prophylaxis</p> Signup and view all the answers

    What is the main management approach for aplastic crises in sickle cell anemia patients?

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

    Which complication is associated with excessive bilirubin production due to prolonged haemolysis in sickle cell anemia patients?

    <p>Cholelithiasis (gallstones) and cholecystitis</p> Signup and view all the answers

    Which type of crisis is caused by an acute worsening of a patient's baseline anemia, usually due to parvovirus B19 infection?

    <p>Aplastic crisis</p> Signup and view all the answers

    What causes haemolytic crises in sickle cell anemia patients?

    <p>Intravascular sickling of the bowel</p> Signup and view all the answers

    What condition can lead to stroke in sickle cell anemia patients?

    <p>Cerebral infarction</p> Signup and view all the answers

    What does decreased immune reactions due to hyposplenism result in for sickle cell anemia patients?

    <p>Increased risk of severe bacterial infections</p> Signup and view all the answers

    What is the second most common complication of sickle cell disease?

    <p>Priapism and infarction of the penis</p> Signup and view all the answers

    What is the main cause of a vaso-occlusive crisis in sickle cell disease?

    <p>Obstructing capillaries with sickle-shaped red blood cells</p> Signup and view all the answers

    What is the characteristic feature of splenic sequestration crises in sickle cell disease?

    <p>Painful enlargements of the spleen</p> Signup and view all the answers

    What is the second most common complication in sickle cell disease?

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

    What causes an aplastic crisis in sickle cell disease?

    <p>Parvovirus B19 infection</p> Signup and view all the answers

    Which crisis in sickle cell disease involves acute painful enlargements of the spleen?

    <p>Splenic sequestration crisis</p> Signup and view all the answers

    What is the main cause of acute chest syndrome in sickle cell disease?

    <p>Obstructing capillaries with sickle-shaped red blood cells</p> Signup and view all the answers

    Which complication in sickle cell disease involves accelerated drops in hemoglobin level?

    <p>Haemolytic crisis</p> Signup and view all the answers

    What is the most common complication in sickle cell disease?

    <p>Vaso-occlusive crisis</p> Signup and view all the answers

    In which type of crisis does a patient experience a precipitous fall in haemoglobin levels if left untreated?

    <p>Splenic sequestration crisis</p> Signup and view all the answers

    Which complication accounts for about 25% of deaths in patients with sickle cell disease?

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

    What is the characteristic feature of haemolytic crises in sickle cell disease?

    <p>Accelerated drops in hemoglobin level</p> Signup and view all the answers

    Where do the majority of sickle cell disease cases occur?

    <p>Sub-Saharan Africa</p> Signup and view all the answers

    Study Notes

    • Sickle cell disease (SCD) is a condition that requires extensive care, including infection prevention through vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication.

    • New gene therapies were approved in 2023 to cure a small percentage of people with sickle cell disease through bone marrow transplant.

    • The disease affects approximately 4.4 million people, with an additional 43 million having the sickle cell trait. The majority of cases occur in Sub-Saharan Africa.

    • The disease was first described by James B. Herrick in 1910, and the genetic transmission was determined by E. A. Beet and J. V. Neel in 1949. The protective effect against malaria of sickle cell trait was discovered in 1954.

    • Sickle cell disease leads to various acute and chronic complications, with sickle cell crises being one of the most common and potentially life-threatening.

    • There are several types of sickle cell crises, including vaso-occlusive, aplastic, splenic sequestration, and haemolytic crises, each with unique characteristics and treatments.

    • The vaso-occlusive crisis is caused by sickle-shaped red blood cells obstructing capillaries and restricting blood flow, leading to pain, ischemia, and potential organ damage. This crisis can be managed with hydration, analgesics, and blood transfusion for milder cases, and intravenous opioids for more severe cases.

    • Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells, which can result in a precipitous fall in haemoglobin levels and hypovolemic shock if left untreated. Management is supportive and may include blood transfusion.

    • Acute chest syndrome is the second most common complication and accounts for about 25% of deaths in patients with SCD. It is characterized by chest pain, fever, pulmonary infiltrate, respiratory symptoms, and hypoxemia. Most cases involve vaso-occlusive crises, but about 80% of people have vaso-occlusive crises during acute chest syndrome.

    • Aplastic crises are instances of an acute worsening of a patient's baseline anaemia, caused by parvovirus B19 infection, which directly affects red blood cell production and results in a precipitous drop in haemoglobin levels. This crisis can be managed supportively, but some patients may require a blood transfusion.

    • Haemolytic crises are acute accelerated drops in haemoglobin level, caused by an increased breakdown of red blood cells, particularly common in those with coexistent G6PD deficiency. The imbalance in oxygen reactive species in the cells leads to more production of red blood cells that are not properly oxygenated or formed. Management is supportive, with the use of antioxidants and blood transfusion if necessary.

    • Sickle cell disease (SCD) is a condition that requires extensive care, including infection prevention through vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication.

    • New gene therapies were approved in 2023 to cure a small percentage of people with sickle cell disease through bone marrow transplant.

    • The disease affects approximately 4.4 million people, with an additional 43 million having the sickle cell trait. The majority of cases occur in Sub-Saharan Africa.

    • The disease was first described by James B. Herrick in 1910, and the genetic transmission was determined by E. A. Beet and J. V. Neel in 1949. The protective effect against malaria of sickle cell trait was discovered in 1954.

    • Sickle cell disease leads to various acute and chronic complications, with sickle cell crises being one of the most common and potentially life-threatening.

    • There are several types of sickle cell crises, including vaso-occlusive, aplastic, splenic sequestration, and haemolytic crises, each with unique characteristics and treatments.

    • The vaso-occlusive crisis is caused by sickle-shaped red blood cells obstructing capillaries and restricting blood flow, leading to pain, ischemia, and potential organ damage. This crisis can be managed with hydration, analgesics, and blood transfusion for milder cases, and intravenous opioids for more severe cases.

    • Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells, which can result in a precipitous fall in haemoglobin levels and hypovolemic shock if left untreated. Management is supportive and may include blood transfusion.

    • Acute chest syndrome is the second most common complication and accounts for about 25% of deaths in patients with SCD. It is characterized by chest pain, fever, pulmonary infiltrate, respiratory symptoms, and hypoxemia. Most cases involve vaso-occlusive crises, but about 80% of people have vaso-occlusive crises during acute chest syndrome.

    • Aplastic crises are instances of an acute worsening of a patient's baseline anaemia, caused by parvovirus B19 infection, which directly affects red blood cell production and results in a precipitous drop in haemoglobin levels. This crisis can be managed supportively, but some patients may require a blood transfusion.

    • Haemolytic crises are acute accelerated drops in haemoglobin level, caused by an increased breakdown of red blood cells, particularly common in those with coexistent G6PD deficiency. The imbalance in oxygen reactive species in the cells leads to more production of red blood cells that are not properly oxygenated or formed. Management is supportive, with the use of antioxidants and blood transfusion if necessary.

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