The University of Alabama
Capstone College of Nursing
NUR 529 Exam 2 Blueprint
Pages referred to below are from Porth’s Pathophysiology: Concepts of Altered States, 11th ed.,
and the current e-book on coursepoint. Older versions are not included, this course does not
utilize older textbook versions. Format for page numbers below is Porth 10th ed “hard copy”
book/ Course Point Porth E-Book. Example: p. 967/975. If the pages correlate between editions
then only one will be listed.
---------------------------------------------------------------------------------------------------------------------
Unit 7. Disorders of the Hematopoietic System. Chapters 22, 23, and 24. There are 6
questions from this unit.
1. Chapter 22. Mechanisms of Hemostasis. See page 639, Understanding hemostasis,
three stages.
vascular spasm, formation of a temporary “platelet plug,” activation of the coagulation cascade,
and formation of a “fibrin plug” or the final clot.
2. Chapter 22. Bleeding Disorders: Bleeding Associated with Platelet Disorders.
Some drugs, such as aspirin, atorvastatin, and some antibiotics, may cause drug-induced immune
thrombocytopenia
Heparin-induced thrombocytopenia (HIT) is associated with the anticoagulant drug heparin.
Immune thrombocytopenia purpura (ITP) (previously known as idiopathic thrombocytopenia
purpura) is an isolated thrombocytopenia with a platelet count of less than 100,000 mL. A
purpuric rash along with normal white blood cells and a normal hemoglobin are found. ITP
results in platelet antibody formation and excess destruction of platelets. Primary ITP is an
autoimmune disease in which platelets are directly destroyed or their formation inhibited by the
immune system. Primary ITP affects both children and adults, though the incidence is highest in
people older than 60 years (Pietras & Pearson-Shaver, 2023). Primary ITP is classified as newly
diagnosed (from diagnosis until 3 months), persistent (from diagnosis until 3 to 12 months), or
chronic (from diagnosis until >12 months).
The familial form of thrombotic thrombocytopenic purpura (TTP) is an autosomal recessive
inheritance. The acquired form is not inherited but is more common, occurring in late childhood
or into adulthood. Acquired TTP may be a single occurrence or many occurrences over time. The
familial form is rare, appearing in infancy and early childhood with symptoms occurring
regularly and exacerbating with stress
Impaired platelet function (also called thrombocytopathia ) may result from inherited disorders
of adhesion (e.g., von Willebrand disease) or acquired defects resulting from drugs, disease, or
,surgery. Defective platelet function is also common in uremia, presumably because of
unexcreted waste products. Chart 22.2 lists drugs that impair platelet function.
Chapter 23. Anemia: Red blood cell characteristics seen in different types anemias.
There are four primary causes of anemia:
(1) excessive loss of red blood cells from bleeding,
(2) destruction (hemolysis) of red blood cells,
Hereditary spherocytosis is mostly transmitted as an autosomal dominant trait and is the most
common inherited disorder of the red cell membrane (Mentzer, 2021). The disorder is caused by
abnormalities of the spectrin, ankyrin, protein 4.2, or band 3 membrane proteins that lead to a
gradual loss of the membrane surface. The loss of membrane relative to cytoplasm causes the
cell to lose its lipid bilayer from the red blood cell cytoskeleton. The red cell forms a spherical
shape and cannot easily traverse the spleen (Mentzer, 2021). Gradually, most of these cells lose
more of their surface membrane and die
Clinical signs are variable but typically include mild hemolytic anemia, jaundice, splenomegaly,
and bilirubin gallstones. A life-threatening aplastic crisis may occur when a sudden disruption of
red cell production (often from a parvovirus B19) causes a rapid drop in hematocrit and the
hemoglobin level (Mentzer, 2021). The disorder usually is treated with splenectomy to reduce
red cell destruction. Blood transfusions may be required in a crisis
Sickle cell disease is an inherited disorder in which an abnormal hemoglobin (hemoglobin S
[HbS]) leads to chronic hemolytic anemia, pain, and organ failure. The HbS gene is transmitted
by recessive inheritance and can manifest as sickle cell trait (i.e., heterozygote with one HbS
gene) or sickle cell disease (i.e., homozygote with two HbS genes). Sickle cell disease affects
approximately 100,000 Americans. It can affect people of any race or ethnicity; however, it
disproportionally affects people with African heritage. Approximately 1 in 365 African-
Americans have sickle cell disease (Noisette, 2019) and 1 in 13 have sickle cell trait
The thalassemias are a group of inherited disorders of hemoglobin synthesis leading to decreased
synthesis of either the α- or β-globin chains of HbA. β-Thalassemias are caused by deficient
synthesis of the β chain and α-thalassemias by deficient synthesis of the α chain (Bhargava et al.,
2020). The defect is inherited as a mendelian trait, and a person may be heterozygous for the trait
and have a mild form of the disease or be homozygous and have the severe form of the disease.
Like sickle cell disease, the thalassemias occur with high degree of frequency in certain
populations. The β-thalassemias, sometimes called Cooley anemia or Mediterranean anemia, are
most common in the Mediterranean populations of southern Italy and Greece, and the α-
thalassemias are most common among Asians. Both β- and α-thalassemias are common in
Africans and Americans of African descent.
The most severe form of α-thalassemia occurs in infants in whom all four α-globin genes are
deleted and is called hydrops fetalis syndrome. Such a defect results in a hemoglobin molecule
, (Hb Bart) that is formed exclusively from the chains of HbF. Hb Bart, which has an extremely
high oxygen affinity, cannot release oxygen in the tissues (Bhargava et al., 2020). Hydrops
fetalis syndrome usually results in death in utero or shortly after birth.
(3) defective red blood cell production,
Iron deficiency is a common worldwide cause of anemia affecting people of all ages. The anemia
results from dietary iron deficiency, loss of iron through bleeding, or insufficient iron to meet
increased demands as occurs with growth spurts during childhood
Megaloblastic anemias are caused by impaired DNA synthesis that results in enlarged red cells
(MCV > 100 fL) because of impaired maturation and division (Bhargava et al., 2020). Vitamin
B12 and folate deficiencies are the most common conditions associated with megaloblastic
anemias.
Vitamin B 12 , also known as cobalamin, serves as a cofactor for two important reactions in
humans. It is essential for DNA synthesis and nuclear maturation, which in turn leads to normal
red cell maturation and division. Vitamin B12 is also involved in a reaction that prevents
abnormal fatty acids from being incorporated into neuronal lipids. This abnormality may
predispose to myelin breakdown and produce some of the neurologic complications of vitamin
B12 deficiency (Ciesla, 2019).
Clinical Manifestations The loss of red cells results in a moderate to severe anemia and mild
jaundice. The MCV is elevated because the cells are larger than normal, and the MCHC is
normal. Neurologic changes that accompany the disorder are caused by deranged methylation of
myelin protein. Demyelination of the dorsal and lateral columns of the spinal cord causes
symmetric paresthesias of the feet and fingers, loss of vibratory and position sense, and eventual
spastic ataxia. In more advanced cases, cerebral function may be altered. In some cases,
confusion and dementia and other neuropsychiatric changes may precede hematologic changes.
Diagnosis and Treatment Diagnosis of vitamin B12 deficiency is made by finding an abnormally
low vitamin B12 serum level. The diagnosis of pernicious anemia, as the cause of the deficiency,
is usually made by the detection of parietal cell and intrinsic factor antibodies (Ciesla, 2019).
Lifelong treatment is often necessary for malabsorption. Because adequate absorption does not
occur with standard doses of vitamin B12, treatment consists of intramuscular injections,
intranasal sprays, or high oral doses of vitamin B12 to reverse the anemia and improve the
neurologic changes (Ciesla, 2019).
Chronic Disease Anemias
Anemia often occurs as a complication of chronic infections, inflammation, and cancer. Of the
anemias that occur secondary to chronic conditions, anemia of renal disease is especially
common and frequently severe. The kidneys are the primary site for the synthesis of
erythropoietin, the colony-stimulating factor that stimulates stem cells to differentiate into
Capstone College of Nursing
NUR 529 Exam 2 Blueprint
Pages referred to below are from Porth’s Pathophysiology: Concepts of Altered States, 11th ed.,
and the current e-book on coursepoint. Older versions are not included, this course does not
utilize older textbook versions. Format for page numbers below is Porth 10th ed “hard copy”
book/ Course Point Porth E-Book. Example: p. 967/975. If the pages correlate between editions
then only one will be listed.
---------------------------------------------------------------------------------------------------------------------
Unit 7. Disorders of the Hematopoietic System. Chapters 22, 23, and 24. There are 6
questions from this unit.
1. Chapter 22. Mechanisms of Hemostasis. See page 639, Understanding hemostasis,
three stages.
vascular spasm, formation of a temporary “platelet plug,” activation of the coagulation cascade,
and formation of a “fibrin plug” or the final clot.
2. Chapter 22. Bleeding Disorders: Bleeding Associated with Platelet Disorders.
Some drugs, such as aspirin, atorvastatin, and some antibiotics, may cause drug-induced immune
thrombocytopenia
Heparin-induced thrombocytopenia (HIT) is associated with the anticoagulant drug heparin.
Immune thrombocytopenia purpura (ITP) (previously known as idiopathic thrombocytopenia
purpura) is an isolated thrombocytopenia with a platelet count of less than 100,000 mL. A
purpuric rash along with normal white blood cells and a normal hemoglobin are found. ITP
results in platelet antibody formation and excess destruction of platelets. Primary ITP is an
autoimmune disease in which platelets are directly destroyed or their formation inhibited by the
immune system. Primary ITP affects both children and adults, though the incidence is highest in
people older than 60 years (Pietras & Pearson-Shaver, 2023). Primary ITP is classified as newly
diagnosed (from diagnosis until 3 months), persistent (from diagnosis until 3 to 12 months), or
chronic (from diagnosis until >12 months).
The familial form of thrombotic thrombocytopenic purpura (TTP) is an autosomal recessive
inheritance. The acquired form is not inherited but is more common, occurring in late childhood
or into adulthood. Acquired TTP may be a single occurrence or many occurrences over time. The
familial form is rare, appearing in infancy and early childhood with symptoms occurring
regularly and exacerbating with stress
Impaired platelet function (also called thrombocytopathia ) may result from inherited disorders
of adhesion (e.g., von Willebrand disease) or acquired defects resulting from drugs, disease, or
,surgery. Defective platelet function is also common in uremia, presumably because of
unexcreted waste products. Chart 22.2 lists drugs that impair platelet function.
Chapter 23. Anemia: Red blood cell characteristics seen in different types anemias.
There are four primary causes of anemia:
(1) excessive loss of red blood cells from bleeding,
(2) destruction (hemolysis) of red blood cells,
Hereditary spherocytosis is mostly transmitted as an autosomal dominant trait and is the most
common inherited disorder of the red cell membrane (Mentzer, 2021). The disorder is caused by
abnormalities of the spectrin, ankyrin, protein 4.2, or band 3 membrane proteins that lead to a
gradual loss of the membrane surface. The loss of membrane relative to cytoplasm causes the
cell to lose its lipid bilayer from the red blood cell cytoskeleton. The red cell forms a spherical
shape and cannot easily traverse the spleen (Mentzer, 2021). Gradually, most of these cells lose
more of their surface membrane and die
Clinical signs are variable but typically include mild hemolytic anemia, jaundice, splenomegaly,
and bilirubin gallstones. A life-threatening aplastic crisis may occur when a sudden disruption of
red cell production (often from a parvovirus B19) causes a rapid drop in hematocrit and the
hemoglobin level (Mentzer, 2021). The disorder usually is treated with splenectomy to reduce
red cell destruction. Blood transfusions may be required in a crisis
Sickle cell disease is an inherited disorder in which an abnormal hemoglobin (hemoglobin S
[HbS]) leads to chronic hemolytic anemia, pain, and organ failure. The HbS gene is transmitted
by recessive inheritance and can manifest as sickle cell trait (i.e., heterozygote with one HbS
gene) or sickle cell disease (i.e., homozygote with two HbS genes). Sickle cell disease affects
approximately 100,000 Americans. It can affect people of any race or ethnicity; however, it
disproportionally affects people with African heritage. Approximately 1 in 365 African-
Americans have sickle cell disease (Noisette, 2019) and 1 in 13 have sickle cell trait
The thalassemias are a group of inherited disorders of hemoglobin synthesis leading to decreased
synthesis of either the α- or β-globin chains of HbA. β-Thalassemias are caused by deficient
synthesis of the β chain and α-thalassemias by deficient synthesis of the α chain (Bhargava et al.,
2020). The defect is inherited as a mendelian trait, and a person may be heterozygous for the trait
and have a mild form of the disease or be homozygous and have the severe form of the disease.
Like sickle cell disease, the thalassemias occur with high degree of frequency in certain
populations. The β-thalassemias, sometimes called Cooley anemia or Mediterranean anemia, are
most common in the Mediterranean populations of southern Italy and Greece, and the α-
thalassemias are most common among Asians. Both β- and α-thalassemias are common in
Africans and Americans of African descent.
The most severe form of α-thalassemia occurs in infants in whom all four α-globin genes are
deleted and is called hydrops fetalis syndrome. Such a defect results in a hemoglobin molecule
, (Hb Bart) that is formed exclusively from the chains of HbF. Hb Bart, which has an extremely
high oxygen affinity, cannot release oxygen in the tissues (Bhargava et al., 2020). Hydrops
fetalis syndrome usually results in death in utero or shortly after birth.
(3) defective red blood cell production,
Iron deficiency is a common worldwide cause of anemia affecting people of all ages. The anemia
results from dietary iron deficiency, loss of iron through bleeding, or insufficient iron to meet
increased demands as occurs with growth spurts during childhood
Megaloblastic anemias are caused by impaired DNA synthesis that results in enlarged red cells
(MCV > 100 fL) because of impaired maturation and division (Bhargava et al., 2020). Vitamin
B12 and folate deficiencies are the most common conditions associated with megaloblastic
anemias.
Vitamin B 12 , also known as cobalamin, serves as a cofactor for two important reactions in
humans. It is essential for DNA synthesis and nuclear maturation, which in turn leads to normal
red cell maturation and division. Vitamin B12 is also involved in a reaction that prevents
abnormal fatty acids from being incorporated into neuronal lipids. This abnormality may
predispose to myelin breakdown and produce some of the neurologic complications of vitamin
B12 deficiency (Ciesla, 2019).
Clinical Manifestations The loss of red cells results in a moderate to severe anemia and mild
jaundice. The MCV is elevated because the cells are larger than normal, and the MCHC is
normal. Neurologic changes that accompany the disorder are caused by deranged methylation of
myelin protein. Demyelination of the dorsal and lateral columns of the spinal cord causes
symmetric paresthesias of the feet and fingers, loss of vibratory and position sense, and eventual
spastic ataxia. In more advanced cases, cerebral function may be altered. In some cases,
confusion and dementia and other neuropsychiatric changes may precede hematologic changes.
Diagnosis and Treatment Diagnosis of vitamin B12 deficiency is made by finding an abnormally
low vitamin B12 serum level. The diagnosis of pernicious anemia, as the cause of the deficiency,
is usually made by the detection of parietal cell and intrinsic factor antibodies (Ciesla, 2019).
Lifelong treatment is often necessary for malabsorption. Because adequate absorption does not
occur with standard doses of vitamin B12, treatment consists of intramuscular injections,
intranasal sprays, or high oral doses of vitamin B12 to reverse the anemia and improve the
neurologic changes (Ciesla, 2019).
Chronic Disease Anemias
Anemia often occurs as a complication of chronic infections, inflammation, and cancer. Of the
anemias that occur secondary to chronic conditions, anemia of renal disease is especially
common and frequently severe. The kidneys are the primary site for the synthesis of
erythropoietin, the colony-stimulating factor that stimulates stem cells to differentiate into
