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Questions and Answers
What can cause a vaso-occlusive thrombotic crisis?
What can cause a vaso-occlusive thrombotic crisis?
Which is a sign of splenic sequestration crisis?
Which is a sign of splenic sequestration crisis?
What primarily triggers an aplastic crisis?
What primarily triggers an aplastic crisis?
What distinguishes Hemophilia A from Hemophilia B?
What distinguishes Hemophilia A from Hemophilia B?
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What is the role of fibrin in the coagulation process?
What is the role of fibrin in the coagulation process?
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What is the primary deficiency in Hemophilia A?
What is the primary deficiency in Hemophilia A?
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Which of the following is a manifestation associated with hemophilia?
Which of the following is a manifestation associated with hemophilia?
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Which statement accurately describes the inheritance pattern of hemophilia?
Which statement accurately describes the inheritance pattern of hemophilia?
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Which factor deficiency is associated with Hemophilia B, also known as Christmas disease?
Which factor deficiency is associated with Hemophilia B, also known as Christmas disease?
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What serious complication can arise from hematomas in the spinal cord due to hemophilia?
What serious complication can arise from hematomas in the spinal cord due to hemophilia?
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What is the primary cause of splenic sequestration crisis in sickle cell disease?
What is the primary cause of splenic sequestration crisis in sickle cell disease?
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During a vaso-occlusive thrombotic crisis, what primary change occurs to the red blood cells?
During a vaso-occlusive thrombotic crisis, what primary change occurs to the red blood cells?
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What can trigger episodes of sickling in sickle cell disease?
What can trigger episodes of sickling in sickle cell disease?
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What is the characteristic feature of sickle cell trait?
What is the characteristic feature of sickle cell trait?
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What is the role of immunoprophylaxis using RhoGAM in the context of acquired congenital hemolytic anemia?
What is the role of immunoprophylaxis using RhoGAM in the context of acquired congenital hemolytic anemia?
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What is the most prominent clinical manifestation of iron deficiency anemia in its severe form?
What is the most prominent clinical manifestation of iron deficiency anemia in its severe form?
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What is a common complication associated with hyperbilirubinemia in hemolytic disease of the fetus and newborn?
What is a common complication associated with hyperbilirubinemia in hemolytic disease of the fetus and newborn?
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Which factor is not associated with increased iron requirements leading to iron deficiency anemia?
Which factor is not associated with increased iron requirements leading to iron deficiency anemia?
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What is a potential outcome if a vaso-occlusive thrombotic crisis is not reversed?
What is a potential outcome if a vaso-occlusive thrombotic crisis is not reversed?
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What are common signs associated with splenic sequestration crisis?
What are common signs associated with splenic sequestration crisis?
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Which of the following best describes the main effect of an aplastic crisis?
Which of the following best describes the main effect of an aplastic crisis?
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In hemophilia, what is primarily affected in the coagulation process?
In hemophilia, what is primarily affected in the coagulation process?
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What can trigger an aplastic crisis?
What can trigger an aplastic crisis?
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Which of the following is NOT a clinical manifestation of hemophilia?
Which of the following is NOT a clinical manifestation of hemophilia?
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In terms of inheritance, which statement about hemophilia is correct?
In terms of inheritance, which statement about hemophilia is correct?
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What type of bleeding is specifically associated with hemarthrosis?
What type of bleeding is specifically associated with hemarthrosis?
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Which factor deficiency is primarily responsible for Hemophilia A?
Which factor deficiency is primarily responsible for Hemophilia A?
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What is a potential outcome for children born to a female carrier of hemophilia?
What is a potential outcome for children born to a female carrier of hemophilia?
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What is a common clinical manifestation of severe iron deficiency anemia?
What is a common clinical manifestation of severe iron deficiency anemia?
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Which factor is commonly associated with iron deficiency anemia in children?
Which factor is commonly associated with iron deficiency anemia in children?
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In sickle cell disease, which of the following is true regarding the RBCs during a sickling episode?
In sickle cell disease, which of the following is true regarding the RBCs during a sickling episode?
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What preventive measure is crucial for Rh-negative mothers to protect future pregnancies?
What preventive measure is crucial for Rh-negative mothers to protect future pregnancies?
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Which statement best describes the occurrence of jaundice in acquired congenital hemolytic anemia?
Which statement best describes the occurrence of jaundice in acquired congenital hemolytic anemia?
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Which type of anemia involves the replacement of normal hemoglobin with abnormal hemoglobin S?
Which type of anemia involves the replacement of normal hemoglobin with abnormal hemoglobin S?
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What might be an effect of chronic hemolytic anemia in sickle cell disease?
What might be an effect of chronic hemolytic anemia in sickle cell disease?
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What does the presence of sickle cell trait indicate?
What does the presence of sickle cell trait indicate?
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Study Notes
Aplastic Crisis
- Diminished production and increased destruction of red blood cells (RBCs).
- Triggered by viral infection or depletion of folic acid.
- Virus causes a temporary shutdown of RBC production in the bone marrow, but hemolysis continues, leading to a sudden drop in hemoglobin level.
- Signs include profound anemia, pallor, and fatigue.
Vaso-occlusive Thrombotic Crisis
- Precipitated by dehydration, exposure to cold, acidosis, or localized hypoxemia.
- Caused by stasis of blood with clumping of cells in the microcirculation, leading to ischemia and infarction.
- Thrombosis and infarction of local tissue may occur if the crisis is not reversed.
- Cerebral occlusion can result in a stroke!
- Symptoms include fever, pain, tissue engorgement, and redness.
Splenic Sequestration/Sequestration Crisis
- Life-threatening: death can occur within hours.
- Caused by pooling of blood in the spleen.
- Signs include profound anemia, hypovolemia, and shock.
- Clinical manifestations of hypovolemia include low blood pressure and high heart rate.
- Complications of splenomegaly can be life-threatening.
Coagulation Review
- After injury, clotting factors are activated by products of tissue destruction and inflammation, including collagen, thrombin, and platelet-activating factor.
- A blood clot is a meshwork of protein (fibrin) strands that stabilizes the platelet plug and traps other cells, such as erythrocytes, phagocytes, and microorganisms.
- In hemophilia, there is a problem with the intrinsic and extrinsic pathways and/or the fibrinolytic system.
Hemophilia
- It is a hereditary bleeding disorder or spontaneous mutation of the factor gene.
- Treatment is determined based on the deficient factor and involves replacement therapy.
- It's not a cure, but symptoms can be controlled.
Hemophilia Clinical Manifestations
- Prolonged bleeding
- Excessive bruising
- Subcutaneous and intramuscular hemorrhages
- Oral bleeding from dental work
- Hemarthrosis (bleeding into a joint space)
- Signs of swelling, warmth, redness, pain, and loss of movement of limbs.
- Bleeding in the neck, mouth, or thorax
- Anemia
- Hemorrhage from any trauma (cuts, injections, circumcision)
- Hematomas in the spinal cord → paralysis
Hemophilia A & B (Pathophysiology)
- X-linked recessive trait.
- Males are mostly affected.
- Females may be carriers and may exhibit manifestations.
- Female carrier (4 possible outcomes with each pregnancy):
- A girl who is not a carrier
- A girl who is a carrier
- A boy without hemophilia
- A boy with hemophilia
X-linked Recessive Inheritance
- Unaffected Father (XY) + Carrier Mother (XX) = Unaffected Son (XY) + Carrier Daughter (XX)
- Unaffected Father (XY) + Carrier Mother (XX) = Affected Son (XY) + Carrier Daughter (XX)
- Affected Father (XY) + Carrier Mother (XX) = Unaffected Son (XY) + Carrier Daughter (XX)
- Affected Father (XY) + Carrier Mother (XX) = Affected Son (XY) + Carrier Daughter (XX)
- Note: a few carriers may be mildly affected due to skewed X-inactivation.*
Hemophilia A
- Known as "classic hemophilia"
- Deficiency of factor VIII (antihemophilic factor)
- AHF is produced in the liver and is necessary for the formation of thromboplastin.
- Accounts for 80-85% of cases of hemophilia.
Hemophilia B
- Also known as "Christmas disease"
- Caused by deficiency of factor IX (Christmas factor)
Iron Deficiency Anemia
- Most common blood disorder of infancy and childhood.
- Infants and young children who drink excessive amounts of cow's milk may develop IDA because of chronic intestinal blood loss.
- Protein in cow's milk causes inflammation in the GI tract, damage to the mucosa, and leads to microhemorrhage.
Can Result From
- Dietary lack of iron
- Problems with iron absorption
- Blood loss
- Increased requirement for iron
Clinical Manifestations
- Irritability, decreased activity tolerance, pica (MILD)
- Pallor, tachycardia, murmurs (SEVERE)
- Developmental delays; changes in the child's attention span, alertness, and learning ability
Acquired Congenital Hemolytic Anemia
- Hemolytic disease of fetus and newborn (HDFN)
- Alloimmune disease (fighting against an invader)
- The first incompatible fetus causes the mothers immune system to produce antibodies that will affect the fetuses of subsequent incompatible pregnancies.
- ABO incompatibility or Rh incompatibility.
Manifestations
- Anemia
- Hyperbilirubinemia --> Jaundice
- Kernicterus (brain damage from high levels of bilirubin)
Prevention is KEY!
- Immunoprophylaxis using Rh immune globulin (RhoGAM) to prevent Rh-negative woman from producing antibodies against the next pregnancy.
Sickle Cell Disease
- Hereditary condition where there is a replacement of normal hemoglobin with abnormal hemoglobin S.
Sickle Cell Trait
- Most common form of sickle cell disease.
- Child is a CARRIER of sickle cell anemia and rarely has symptoms.
Sickle Cell Anemia
- Child is subject to sickle cell crises.
- Chronic hemolytic anemia.
Pathophysiology
- During episodes of sickling, RBCs become abnormally crescent-shaped.
- Sickling depends on the degree of oxygenation, pH, and dehydration.
- Sickled cells can resume a normal shape when rehydrated and re-oxygenated.
- May occlude small blood vessels.
- RBCs become weak and are hemolyzed and removed.
Types of Sickle Cell Crises
- Vaso-occlusive Thrombotic Crisis
- Splenic Sequestration/Sequestration Crisis
- Aplastic Crisis
Iron Deficiency Anemia (Acquired Disorders of Erythrocytes)
- Most common blood disorder in children
- Infants & young children who drink excessive amounts of cow's milk may develop IDA due to chronic intestinal blood loss
- Protein causing inflammation in GI tract leads to damage in the mucosa and microhemorraging
- Can result from dietary lack of iron, problems with iron absorption, blood loss, and increased iron requirement
- Clinical Manifestations:
- Mild: Irritability, decreased activity tolerance, pica
- Severe: Pallor, tachycardia, murmurs
- Developmental delays
Acquired Congenital Hemolytic Anemia (Acquired Disorders of Erythrocytes)
- Includes Hemolytic disease of fetus and newborn (HDFN)
- Alloimmune disease (immune system fighting against foreign invaders)
- First incompatible fetus causes mother's immune system to produce antibodies that will affect subsequent incompatible pregnancies
- ABO or Rh incompatibility
- Manifestations:
- Anemia
- Hyperbilirubinemia (jaundice)
- Kernicterus (brain damage from high levels of bilirubin)
- Prevention is key: Immunoprophylaxis using Rh immune globulin (RhoGAM) to prevent Rh-negative women from producing antibodies
Sickle Cell Disease (Inherited Disorder of Erythrocytes)
- Hereditary condition where normal Hgb is replaced with abnormal Hgb S
- Sickle Cell Trait (Heterozygous, Ss):
- Most common form
- Child is a carrier and rarely experiences symptoms
- Sickle Cell Anemia (Homozygous, ss):
- Child is susceptible to sickle cell crises
- Chronic hemolytic anemia
- Pathophysiology:
- During sickling episodes, RBCs become crescent shaped due to oxygenation, pH and dehydration levels
- Sickled cells can return to normal shape when rehydrated and re-oxygenated
- May occlude small blood vessels
- RBCs become weak and are hemolyzed and removed
Types of Sickle Cell Crises
- Vaso-occlusive Thrombotic Crisis
- Splenic Sequestration/Sequestration Crisis
- Aplastic Crisis
Sickle Cell Crises
Vaso-occlusive Thrombotic Crisis
- Precipitated by dehydration, exposure to cold, acidosis, or localized hypoxemia
- Caused by stasis of blood with clumping of cells in microcirculation → ischemia → infarction
- Thrombosis and infarction of local tissue may occur if the crisis is not reversed
- Cerebral occlusion can result in a stroke!
- Symptoms: fever, pain, tissue engorgement & redness
Splenic Sequestration/Sequestration Crisis
- Life-threatening, death can occur within hours
- Caused by pooling of blood in the spleen
- Signs include profound anemia, hypovolemia, and shock
- CM of hypovolemia (low BP, high HR!)
- Complications of Splenomegaly:
- Autosplenectomy
- Increased risk of infection
Aplastic Crisis
- Diminished production and increased destruction of RBCs
- Triggered by viral infection or depletion of folic acid
- Virus causes a temporary shutdown of RBC production in the bone marrow but hemolysis continues → leads to a sudden drop in hemoglobin level
- Signs: profound anemia, pallor, and fatigue
Hemophilias (Inherited Coagulation and Platelet Disorders)
- It is a hereditary bleeding disorder or spontaneous mutation of the factor gene
- Treatment is determined based on the deficient factor by replacement therapy, it is not a cure but symptoms can be controlled. Hemophilia A and Hemophilia B are most common
Coagulation Review
- After injury, clotting factors are activated by products of tissue destruction and inflammation, including collagen, thrombin, and platelet-activating factor.
- A blood clot is a meshwork of protein (fibrin) strands that stabilizes the platelet plug and traps other cells. - In hemophilia, there is a problem with the intrinsic & extrinsic pathway and/or the fibrinolytic system.
Hemophilia Clinical Manifestations
- Prolonged bleeding
- Excessive bruising
- Subcutaneous and intramuscular hemorrhages
- Oral bleeding from dental work
- Hemarthrosis (bleeding into a joint space)
- Signs of swelling, warmth, redness, pain and loss of movement of limbs
- Bleeding in the neck, mouth, or thorax
- Anemia
- Hemorrhage from any trauma (cuts, injections, circumcision)
- Hematomas in the spinal cord → paralysis
Hemophilia A & B
- X-linked recessive trait
- Males are mostly affected
- Females may be carriers & may exhibit manifestations
- Female carrier (4 possible outcomes with each pregnancy):
- A girl who is not a carrier
- A girl who is a carrier
- A boy without hemophilia
- A boy with hemophilia
X-linked recessive
- Unaffected Father (XY) and Carrier Mother (XX) can have:
- Unaffected son (XY)
- Carrier daughter (XX)
- Affected daughter (XX)
- Unaffected son (XY)
- Affected Father (XY) and Carrier Mother (XX) can have:
- Unaffected son (XY)
- Carrier daughter (XX)
- Affected daughter (XX)
- Affected son (XY)
- Note: a few carriers may be mildly affected due to skewed X-inactivation.*
Hemophilia A & B
Hemophilia A
- Known as "classic hemophilia"
- Deficiency of factor VIII (antihemophilic factor)
- AHF is produced in the liver & is necessary for the formation of thromboplastin
- Accounts for 80-85% of cases of hemophilia
Hemophilia B
- Also known as "Christmas disease"
- Caused by deficiency of factor IX (Christmas factor)
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Description
This quiz covers three critical hemolytic crises: Aplastic Crisis, Vaso-occlusive Thrombotic Crisis, and Splenic Sequestration Crisis. You'll learn about the causes, symptoms, and significant health risks associated with each type of crisis. Test your knowledge and understanding of these urgent medical conditions.
