103 Questions
Which of the following is a cause of microcytic anemia?
Thalassemia
Which condition is associated with macrocytic anemia?
Vitamin B12 deficiency
What can cause nonmegaloblastic macrocytic anemia?
Alcohol abuse
Which condition is associated with normocytic anemia?
Anemia of inflammation/chronic disease
What is the gene frequency of HbC in African Americans?
1.5%
What is the median age at death for men with sickle cell anemia?
42
How many haplotypes of sickle cell disease are there?
5
What contributes to progressive vasculopathy and various complications in sickle cell disease?
Ischemia-reperfusion injury and hemolysis
What is associated with increased life expectancy and fewer complications in sickle cell disease?
Higher levels of fetal hemoglobin
What leads to decreased rates of hemolysis and increased hemoglobin levels in sickle cell patients?
Coexistent alpha-thalassemia
What are the clinical manifestations of sickle cell anemia?
Hematologic abnormalities, pulmonary complications, and hypercoagulability
What is a common and serious complication of sickle cell disease?
Acute chest syndrome
What is a contributing factor to the etiology of acute chest syndrome?
Fat embolism
What are the general principles of management for sickle cell anemia?
Addressing complications, preventing vaso-occlusive crises, and managing pain
What may sickle cell anemia patients require to address the complex pathophysiology and clinical manifestations of the disease?
Comprehensive care
What percentage of sickle cell disease patients have reactive airways?
35%
What is the percentage of sickle cell disease patients with pulmonary hypertension?
35%
What percentage of adult males with sickle cell disease experience priapism?
40%
What is the approximate percentage of sickle cell disease patients with renal complications leading to chronic kidney disease?
30%
What percentage of sickle cell disease patients experience vaso-occlusive episodes affecting bones and joints?
30%
What percentage of sickle cell disease patients have retinopathy more commonly in HbSC disease?
30%
What percentage of sickle cell disease patients have elevated transcranial Doppler velocities and are at high stroke risk?
15%
What percentage of sickle cell disease patients experience infection, with 8% due to bacteria?
27%
What percentage of sickle cell disease patients are affected by infarction?
10%
What percentage of sickle cell disease patients have reactive airways, pulmonary hypertension, and renal complications combined?
60%
What percentage of sickle cell trait carriers have an increased risk for venous thromboembolism?
8%
What percentage reduction in stroke risk is achieved by regular transfusions to keep HbS level below 30%?
90%
What is the recommended therapy to reduce the rate of pain crises and development of acute chest syndrome in moderate to severe sickle cell disease?
Hydroxyurea therapy
What is the primary pathophysiological process involved in sickle cell disease?
HbS polymerization
What is the recommended prophylactic treatment for all pediatric patients with sickle cell disease?
Prophylactic penicillin
What is the increased risk for venous thromboembolism in patients with sickle cell trait?
2-fold
What is the recommended vaccination for patients of all ages with sickle cell disease due to functional hyposplenism?
Streptococcus pneumoniae
What is the primary diagnostic method for sickle cell disease in older children and adults?
Electrophoresis and solubility tests
What is the primary management strategy for vaso-occlusive crises in sickle cell disease?
Analgesics, hydration, and oxygen therapy
What is the primary clinical manifestation of acute chest syndrome in adults?
Chest pain
What is the recommended therapy for acute exacerbation of anemia, acute chest syndrome, and acute stroke in sickle cell disease?
Transfusion therapy
What is the primary screening method for identifying patients with sickle cell disease?
Newborn screening
What is the experimental therapy for sickle cell disease?
Stem cell transplant
What is the recommended approach to pain management for sickle cell disease patients?
Multidisciplinary approach involving nurses and social workers
Which serum test is considered the best for diagnosing iron deficiency anemia?
Serum ferritin
What is the classic presentation of iron deficiency anemia?
Fatigue and craving for ice in a young, menstruating woman
What is the optimal dose range of elemental iron for oral iron replacement therapy?
15-150 mg every 1-2 days
Which test should increase 7-10 days after starting iron therapy?
Reticulocytes
In chronic kidney disease, how are iron abnormalities defined?
Using transferrin saturation and ferritin levels
What is the progression of iron deficiency anemia from normal CBC to mild anemia?
Hb of 9-12 g/dL, hypochromia, and microcytosis
What is the best-absorbed oral iron for iron replacement therapy?
Ferrous sulfate
What is the main cause of iron deficiency anemia in menstruating women?
Blood loss
When is IV iron therapy reserved for in the treatment of iron deficiency anemia?
Malabsorption or intolerance to oral iron
What should be continued for 6 months to replete iron stores?
Iron therapy
How are iron abnormalities in chronic illness challenging to interpret?
Due to the increase in ferritin as an acute phase reactant
How can the gastrointestinal side effects of oral iron be reduced?
Taking it with food, adjusting the dose, or switching to IV iron
Which of the following is a cause of macrocytic anemia?
Vitamin B12 deficiency
Which of the following is a cause of microcytic anemia?
Thalassemia
Which condition is associated with normocytic anemia?
Anemia of inflammation/chronic disease
Which of the following is a rare cause of microcytic anemia?
Copper deficiency
What is the optimal dose range of elemental iron for oral iron replacement therapy?
30-60 mg of elemental iron every 1-2 days
What is the best serum test for diagnosing iron deficiency anemia?
Serum ferritin
What is the primary management strategy for vaso-occlusive crises in sickle cell disease?
Pain management and hydration
What should reticulocytes do 7-10 days after starting iron therapy?
Increase
In chronic kidney disease, how are iron abnormalities defined?
Using transferrin saturation and ferritin levels
What is the classic presentation of iron deficiency anemia?
Fatigue and craving for ice in young, menstruating women
What is the cause of microcytic anemia?
Iron deficiency
What test is used to diagnose iron deficiency anemia in the presence of chronic illness?
Red cell protoporphyrin
What is the primary screening method for identifying patients with sickle cell disease?
Hemoglobin electrophoresis
What is the recommended therapy to reduce the rate of pain crises and development of acute chest syndrome in moderate to severe sickle cell disease?
Hydroxyurea
What is the approximate percentage of sickle cell disease patients with reactive airways, pulmonary hypertension, and renal complications combined?
60%
What is the gene frequency of HbC in African Americans?
0.02
Which condition is associated with increased life expectancy and fewer complications in sickle cell disease?
Hydroxyurea therapy
What is the primary pathophysiological process involved in sickle cell disease?
HbS polymerization
What percentage of adult males with sickle cell disease experience priapism?
30%
What is the recommended vaccination for patients of all ages with sickle cell disease due to functional hyposplenism?
Streptococcus pneumoniae vaccine
What percentage of sickle cell disease patients have reactive airways, pulmonary hypertension, and renal complications combined?
80%
What is the progression of iron deficiency anemia from normal CBC to mild anemia?
Microcytosis, hypochromia, anisocytosis
What percentage of sickle cell disease patients have retinopathy more commonly in HbSC disease?
30%
What is the optimal dose range of elemental iron for oral iron replacement therapy?
60-120 mg/day
What is the median age at death for men with sickle cell anemia?
54 years
What is the increased risk for venous thromboembolism in patients with sickle cell trait?
2-fold
What is the recommended therapy to reduce the rate of pain crises and development of acute chest syndrome in moderate to severe sickle cell disease?
Hydroxyurea therapy
What may sickle cell anemia patients require to address the complex pathophysiology and clinical manifestations of the disease?
Multidisciplinary pain management
What is the gene frequency of HbC in African Americans?
1.5%
What is the median age at death for men with sickle cell anemia?
42
What is the incidence of sickle cell anemia in African Americans?
1 in 600
What is the primary cause of vaso-occlusion in sickle cell disease?
Ischemia
What is the most common genotype of sickle cell disease?
Sickle cell anemia
What is the main contributing factor to the etiology of acute chest syndrome?
Infection
What is associated with increased life expectancy and fewer complications in sickle cell disease?
Higher levels of fetal hemoglobin
What leads to decreased rates of hemolysis and increased hemoglobin levels in sickle cell patients?
Coexistent alpha-thalassemia
What is a common and serious complication of sickle cell disease, often leading to hospitalization?
Acute chest syndrome
What is the primary management strategy for sickle cell anemia?
All of the above
What do patients with sickle cell anemia require to address the complex pathophysiology and clinical manifestations of the disease?
Comprehensive care
What is the most common age at death for women with sickle cell anemia?
48
What percentage of sickle cell disease patients have pulmonary hypertension?
40%
What is the percentage reduction in stroke risk achieved by regular transfusions to keep HbS level below 30%?
90%
What is the gene frequency of HbC in African Americans?
20%
What is the median age at death for men with sickle cell anemia?
48 years
What should be continued for 6 months to replete iron stores?
Oral iron supplementation
What is the primary screening method for identifying patients with sickle cell disease?
Hemoglobin electrophoresis
What is the recommended therapy to reduce the rate of pain crises and development of acute chest syndrome in moderate to severe sickle cell disease?
Hydroxyurea
What is the primary management strategy for vaso-occlusive crises in sickle cell disease?
Pain medications
What is the recommended prophylactic treatment for all pediatric patients with sickle cell disease?
Penicillin prophylaxis
What percentage of adult males with sickle cell disease experience priapism?
40%
What is the recommended vaccination for patients of all ages with sickle cell disease due to functional hyposplenism?
Pneumococcal vaccine
What is the primary diagnostic method for sickle cell disease in older children and adults?
Hemoglobin electrophoresis
Study Notes
Iron Deficiency Anemia: Diagnosis and Treatment
- Classic presentation of iron deficiency anemia is a young, menstruating woman experiencing fatigue and craving for ice
- Iron deficiency anemia progresses from normal CBC to mild anemia with Hb of 9-12 g/dL, hypochromia, and microcytosis
- Causes of iron deficiency include blood loss (menstrual or GI), malabsorption, inadequate intake, and increased demand
- Serum ferritin is the best serum test for diagnosing iron deficiency anemia
- Interpretation of ferritin in the presence of chronic illness is challenging due to its increase as an acute phase reactant
- In chronic kidney disease, iron abnormalities are defined using transferrin saturation and ferritin levels
- Other tests for diagnosing iron deficiency anemia include serum iron, MCV, transferrin saturation, red cell protoporphyrin, red cell ferritin, and RDW
- Iron deficiency anemia is generally treated with oral iron replacement, IV iron therapy is reserved for malabsorption or intolerance
- Transfusion is necessary for patients with severe symptoms or very low hemoglobin levels
- The best-absorbed oral iron is ferrous sulfate, with an optimal dose ranging from 15-150 mg of elemental iron every 1-2 days
- GI side effects of oral iron can be reduced by taking it with food, adjusting the dose, or switching to IV iron
- Reticulocytes should increase 7-10 days after starting iron therapy, and Hb and HCT should increase by 14 days; iron therapy should be continued for 6 months to replete iron stores
Sickle Cell Anemia: Key Points
- Infection occurs in 27% of patients, with 8% due to bacteria, 5% mycoplasma, and 9% chlamydia
- Infarction affects about 10% of patients
- Hypoventilation, atelectasis, and fluid overload may play a role in sickle cell complications
- General principles of management include supplemental oxygen, broad-spectrum antibiotics, and bronchodilators
- 35–60% of sickle cell disease patients have reactive airways, and up to 30% have pulmonary hypertension
- Renal complications include inability to concentrate urine, renal tubular acidosis, and chronic kidney disease in 30% of adults
- Priapism affects 30–40% of adult males with sickle cell disease
- Patients with elevated transcranial Doppler velocities are at high stroke risk
- Regular transfusions to keep HbS level below 30% reduce stroke risk by 90%
- Vaso-occlusive episodes often affect bones and joints, leading to avascular necrosis and chronic pain
- Retinopathy is more common in HbSC disease and should be screened every 1–2 years
- Sickle cell trait affects 8% of African Americans and carries a 2-fold increased risk for venous thromboembolism
Test your knowledge of iron deficiency anemia with this quiz on its diagnosis and treatment. Explore the classic presentation, diagnostic tests, and appropriate management options for this common condition, including oral and IV iron therapy.
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