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Hematology 2 - Week 1 - Chapters 26 & 27
Terms in this set (52)
The qualitative hemoglobinopathies cause disease by producing hemoglobin chains that are:
-produced in inadequate amounts
sickle cell diseases
Which of the following hemoglobinopathies is most common?
-sickle cell diseases
-Hgb E disorders
-Hgb C disorders
What clinical feature of sickle cell disease accounts for the highest number of hospital admissions?
Sickle cell disease (SCD) is found in individuals descended from ancestors from all of the following places except:
What percentage of African Americans have heterozygous sickle cell trait?
Which of the following would be an unusual finding in the blood smear of an adult with sickle cell disease (SCD) crisis?
Sickle gene offers some protection against malaria
What is the probable etiologic association between malaria and sickle cell trait?
-Sickle gene makes people more prone to malaria infection
-sickle gene prevents Plasmodium ovalae infection
-They are both found in Asia
-Sickle gene offers some protection against malaria
Which of the following would be unexpected in homozygous hemoglobin S?
-high reticulocyte count
-elevated Hgb F
-hemoglobin that is less soluble
has either sickle cell anemia or trait
A patient has target cells and a positive tube solubility test. This patient probably:
-has sickle cell anemia
-has sickle trait
-has either sickle cell anemia or trait
Select the best test to confirm the diagnosis of a patient with target cells and a positive tube solubility test:
-mean cell volume (MCV)
-presence of sickle cells
confirm sickle gene with tube solubility test
A patient has two bands on alkaline hemoglobin electrophoresis. One lines up ith hemoglobin A and the other is in the S position. Select the best course of action.
-report as normal
-report as sickle trait
-report as sickle disease
-confirm sickle gene with tube solubility test
keeping the oxygen saturation low
All of the following improve the clinical outcome of sickle cell anemia (hemoglobin SS) except which of the following?
-aggressive prevention and treatment of infection
-presence of fetal hemoglobin
-transfusions during crises
-keeping the oxygen saturation low
normal blood smear and no disease
A patient has 45% hemoglobin S, 55% hemoglobin A, and a positive tube solubility. Which of the following would likely be found?
-normal blood smear and no disease
-target cells on the blood smear and no disease
-normal blood smear and severe disease
-sickle cells on the blood smear and severe disease
Moderate to marked target cells are found on a blood smear. Which of the following can most likely be eliminated?
-hemoglobin C disease
-hemoglobin E disease
normal tube solubility test
Which of the following would be an unexpected finding for patients with hemoglobin SC disease or s/B^0- thalassemia?
-shortened red cell survival
-normal tube solubility test
-mild to moderate clinical features
Which of the following is a typical finding in patients with hemoglobin M?
-severe anemia requiring repeated blood transfusions
-elevated levels of ferrous iron (Fe^2+) in the red cells
-presence of Heinz bodies on Wright-stained blood smear
Heinz bodies are found when hemoglobin:
-has higher oxygen affinity
-concentration is decreased
When affinity is high, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells
What is the correct relationship between red cells affinity for oxygen and red cell production?
-when affinity is low, more oxygen is released and the body compensates by making red cells larger
-when affinity is low, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells
-when affinity is high, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells
-when affinity is high, more oxygen is released and fewer red cells are needed
replacement of one nucleotide in the normal gene with a different nucleotide
What is a point mutation?
-mutation of the stop codon
-replacement of one nucleotide in the normal gene with a different nucleotide
-addition of one nucleotide in the normal gene
-deletion of one nucleotide in the normal gene
S, A, F, and A2
Cellulose acetate is performed on a patient with known homozygous hemoglobin S who has received red cell transfusions in the past week. What hemoglobins would be expected to be found?
-S and F
-A and F
-S, A, and F
-S, A, F, and A2
hemoglobins A and E
An adult female patient from Southeast Asia has no anemia, but her CBC shows an MCV of 68 fL and marked target cells are observed on her blood film. Cellulose acetate alkaline electrophoresis shows two large bands: approximately 55% migrating with hemoglobin A and 40% migrating with hemoglobin C. What is the patient's most likely genotype?
-hemoglobins A and C
-hemoglobins A and E
-hemoglobin C and E
-homozygous hemoglobin E
A structurally normal globin chain is absent or produced at lower levels
What is the basic hemoglobin defect in the thalassemias?
-one of the globin chains has an amino acid substitution
-a structurally normal globin chain is absent or produced at lower levels
-Heme is produced at a lower concentration
-Iron is not incorporated into the protoporphyrin ring to form heme
96% 2% 2%
Which of the following is the normal approximate percentage of hemoglobins in adults?
a2b2 a2d2 a2y2
-2% 2% 96%
-2% 96% 2%
-96% 2% 2%
-40% 20% 40%
Infants are protected by their high concentration of fetal hemoglobin
Why are infants with beta-thalassemia major not ill until approximately 6 months of age?
-Infants are protected by their high concentration of fetal hemoglobin
-Infants have less need for hemoglobin because of their small body size
-Infants have less need for hemoglobin because of their small lung capacity
-Infants have a high red count and thus higher hemoglobin from a higher than normal concentration of erythropoietin
an imbalance in the rate of globin chain synthesis
Which of the following is the primary mechanism for anemia in the thalassemias?
-an imbalance in the rate of globin chain synthesis
-impaired iron incorporation into the protoporphyrin ring
-erythroid hypoplasia in the bone marrow
-abnormal amino acid structure of a globin chain
vary from benign forms to those incompatible with life
Which of the following is true regarding the clinical features of thalassemias?
-are mild conditions rarely requiring treatment
-are serious conditions requiring frequent blood transfusions
-have abnormal red cells but little clinical implications for patients
-vary from benign forms to those incompatible with life
Which of the following is/are expected findings in beta-thalassemia minor?
-normal levels of hemoglobins F and A2
-normocytic normochromic red cells
-hemoglobin levels in the 4 to 6 g/dL range
presence of microcytosis, hypochromasia, and target cells
All of the following can distinguish beta-thalassemia minor from major except:
-presence of microcytosis, hypochromasia, and target cells
-presence of hemoglobin A
normal fetal hemoglobin
Which of the following would be an unexpected finding in homozygous beta^0-thalassemia?
-decreased reticulocyte production index
-normal fetal hemoglobin
-bone marrow erythroid hyperplasia
four missing genes for alpha chain production
A patient has 30% hemoglobin F. Which of the following can be eliminated?
-four missing genes for alpha chain production
-heterozygous hereditary persistence of fetal hemoglobin
-the patient is a normal 1-month-old infant
Barts hemoglobin present at birth
What finding is present in all types of alpha-thalassemias?
-hemoglobin H inclusions seen with Brilliant cresyl blue
-microcytic hypochromic red cells
-Barts hemoglobin present at birth
have three missing genes for alpha chain production
Which of the following is true of patients with hemoglobin H disease?
-have a severe anemia
-have three missing genes for alpha chain production
-have excess unpaired beta chains present
-are of northern European ethnicity
incompatible with life
Homozygous alpha-thalassemia (--/--) has what clinical outcome?
-incompatible with life
Which of the following can help distinguish the combination Hgb S-b^0-thalassemia from sickle cell anemia?
Which of the following is least useful in diagnosing and differentiating the thalassemias from each other?
-complete blood count (CBC) results
Which of the following is useful in helping to distinguish mild asymptomatic iron deficiency from thalassemia trait?
-low mean cell volume (MCV) with relatively high red blood count
-increased osmotic fragility
Hemoglobin Barts is composed of:
variable but decreased beta globin chain synthesis
A patient with beta^+-thalassemia has, from that gene locus:
-variable but decreased beta globin chain synthesis
-no beta globin chain synthesis
-variable but decreased gamma chain synthesis
-no gamma chain synthesis
accumulation of iron from massive RBC transfusion therapy
Iron overload in severe beta-thalassemia (beta-thalassemia major) patients is primarily a consequence of:
-an increased rate of absorption of iron because of the severe anemia
-a decreased need for iron because fewer RBCs are made
-accumulation of iron from massive RBC transfusion therapy
-decreased rate of utilization of iron because small RBCs are made
similar to that of beta-thalassemia minor
Patients who are heterozygous for hemoglobin Lepore have a clinical course:
-that is completely benign
-similar to that of beta-thalassemia minor
-similar to that of alpha-thalassemia minor (one gene deletion)
-similar to that of beta-thalassemia major
Hemoglobin E is primarily found in individuals of which ethnic origin?
performing high-performance liquid chromatography (HPLC)
Hemoglobins A2 and F can be quantified by:
-performing a Brilliant cresyl blue (BCB) stain and counting the number of positive cells
-performing high-performance liquid chromatography (HPLC)
-performing cellulose acetate hemoglobin electrophoresis
-performing citrate agar hemoglobin electrophoresis
In hemoglobin SC disease, what would the result of the hemoglobin solubility test be?
lysine for glutamic acid
What is the amino acid substitution in Hemoglobin C?
-lysine for glutamic acid
-valine for glutamic acid
-aspartic acid for asparagine
-lysine for aspartic acid
Hydrops Barts fetalis
Of the four clinical states of alpha thalassemia, which one is incompatible with life?
-Alpha thalassemia silent carrier
-Alpha thalassemia trait
-Hemoglobin H disease
-Hydrops Barts fetalis
Decreased rate of globin synthesis
Thalassemias are characterizes by:
-structural abnormalities in the hemoglobin molecule
-absence of iron in hemoglobin
-decreased rate of heme synthesis
-decreased rate of globin synthesis
Hemoglobin Barts is composed of:
Absence of 3 of 4 alpha genes
Hemoglobin H disease results from:
-Absence of 3 of 4 alpha genes
-absence of 2 of 4 alpha genes
-absence of 1 of 4 alpha genes
-absence of all 4 alpha genes
What is seen most often in thalassemia?
-chronic blood loss
A quantitative deficiency in RNA resulting in decreased globin chain production
Evidence indicates that the genetic defect in thalassemia usually results in:
-the production of abnormal globin chains
-a quantitative deficiency in RNA resulting in decreased globin chain production
-a structural change in the heme portion of the hemoglobin
-an abnormality in the alpha or beta chain binding or affinity
A cellulose acetate electrophoresis revealed a large band of hemoglobin in the hemoglobin S position. This band quantified as 95%. The peripheral smear revealed 70% target cells and the solubility test was negative. Based on this information, what is the hemoglobin?
A 15-month-old was seen in the ER with a femur fracture. History revealed that the break had occurred from a fall down the stairs. The physician noted hepatosplenomegaly, extreme pallor, and a slight arrhythmia. A CBC revealed the following: WBC 10.2 x 10^9/L: RBC 3.05: Hgb 6.1: Hct 17%: MCV 55.7: MCH 20: MCHC 35.9: RDW 21%: Hemoglobin electrophoresis results were obtained as follows: Hgb F 97%: Hgb A2 3%: Hgb A 0%: What condition is most likely causing the hematologic abnormalities?
-Bart's Hydrops fetalis
-sickle cell trait
THIS SET IS OFTEN IN FOLDERS WITH...
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