Exam 4 Hematology quizes
A defect in ankyrin is described as: a. A vertical interaction defect b. A horizontal interaction defect c. Both vertical and horizontal interaction defects d. Cannot be determined
a. A vertical interaction defect
Macrocytes are often seen in hospitalized alcoholics because of: a. Alcohol toxicity on erythroid precursors b. Stimulated erythropoiesis c. Reticulocytosis d. Iron deficiency
a. Alcohol toxicity on erythroid precursors
Hereditary stomatocytosis is a condition resulting from defects in: a. Cation permeability b. Spectrin c. Protein 4.1 d. Spectrin dimer-dimer association
a. Cation permeability
Laboratory findings include a blood urea nitrogen >30 mg/dL, serum ferritin levels higher than normal, and a normocytic, normochromic morphology. What cause can be attributed to the anemia? a. Chronic renal disease b. Iron deficiency c. Diamond-Blackfan anemia d. Fanconi's syndrome
a. Chronic renal disease
Fanconi's syndrome is an example of: a. Constitutional aplastic anemia b. Acquired aplastic anemia c. Constitutional anemia from external stimuli d. Acquired aplastic anemia from external exposure
a. Constitutional aplastic anemia
The presence of what poikilocyte would lead to a suspicion of myelophthisic anemia rather than pure red cell aplasia? a. Dacryocyte b. Drepanocyte c. Schistocyte d. Stomatocyte
a. Dacryocyte
Which of the following contributes to the production of hypocellular bone marrow? a. Damage to the stem or progenitor cells in the bone marrow b. Iron storage disease c. Gene mutations d. Hemoglobinopathy
a. Damage to the stem or progenitor cells in the bone marrow
Which of the following is found in megaloblastic macrocytosis but not in nonmegaloblastic macrocytosis? a. Decreased WBC count and vitamin B12 levels b. Decreased hemoglobin and hematocrit c. Normal-to-decreased reticulocyte count d. Increased serum bilirubin
a. Decreased WBC count and vitamin B12 levels
The cytopenias associated with MDS are caused by: a. Dyshematopoiesis b. Bone marrow replacement c. Hypoplasia of the bone marrow d. Decreased cytokine production
a. Dyshematopoiesis
What are the two main metabolic pathways that erythrocytes use to break down glucose? a. Glycolytic and hexose monophosphate shunt b. Glycolytic and Luebering-Rapoport pathway c. Krebs cycle and electron transport system d. Hexokinase and Krebs cycle
a. Glycolytic and hexose monophosphate shunt
Which of the following disorders is characterized by both a deficiency in spectrin and the presence of a mutant spectrin protein? a. HPP b. HE c. HS d. Acanthocytosis
a. HPP
What can falsely elevate the serum folate level? a. Hemolysis of the serum sample b. Recent low dietary intake c. Vitamin B12 deficiency d. Recent alcohol consumption
a. Hemolysis of the serum sample
Laboratory findings on a stained blood smear exhibit striking erythrocyte morphologic abnormalities. The MCV is decreased, osmotic fragility is abnormal, and the thermal sensitivity test demonstrates an increase in erythrocyte fragmentation. Autohemolysis in increased and is not corrected with glucose. What disorder is associated with these findings? a. Hereditary pyropoikilocytosis b. Hereditary stomatocytosis c. PNH d. Hereditary spherocytosis
a. Hereditary pyropoikilocytosis
Megaloblastic anemia is characterized by: a. Hypersegmentation of PMNs b. Presence of Heinz bodies c. hypochromia d. Microcytosis
a. Hypersegmentation of PMNs
PNH is currently detected in the lab by which of the following? a. Immunophenotyping b. Ham and sucrose lysis tests c. Donath-Landsteiner test d. Osmotic fragility
a. Immunophenotyping
Intrinsic erythrocyte enzyme deficiencies lead to: a. Increased erythrocyte oxidative susceptibility b. Exacerbated immune response against erythrocytes c. Increased opsonization of erythrocytes d. Increased extravascular hemolysis
a. Increased erythrocyte oxidative susceptibility
A patient is suspected of having HS. However, there appear to be <1% spherocytes on the peripheral blood smear, and the osmotic fragility test is normal. What test could be performed to increase the sensitivity of testing for HS? a. Incubated osmotic fragility test b. Immunophenotyping for CD55 and CD59 c. Sucrose hemolysis test d. Ham test
a. Incubated osmotic fragility test
The reason that lack of vitamin B12 causes megaloblastic changes is that vitamin B12: a. Is needed for the formation of nucleotide bases b. Is needed for the conversion of methionine to SAM c. Initiates mitosis d. Is needed for synthesis of intrinsic factor
a. Is needed for the formation of nucleotide bases
Deficiencies in folic acid lead to which of the following? a. Nuclear cytoplasmic asynchrony b. Absence of nuclear division c. Excessive proliferation of myeloid precursors d. Decreased proliferation of erythroid precursors
a. Nuclear cytoplasmic asynchrony
The nuclear cytoplasmic asynchronous morphology in megaloblastosis is best characterized by: a. Nuclear immaturity with cytoplasmic maturity b. Nuclear maturity with cytoplasmic immaturity c. Nuclear maturity with abundant cytoplasm d. Normal nuclear and cytoplasmic maturity
a. Nuclear immaturity with cytoplasmic maturity
Anemia in hypersplenism differs from anemia found in true hypoproliferative anemias because in hypersplenism, there is: a. Reticulocytosis b. Ineffective hematopoiesis c. Dyshematopoiesis d. Hypoplastic bone marrow
a. Reticulocytosis
The laboratory professional notes about 20% spherocytes on a peripheral blood smear of a 4-year-old boy. An osmotic fragility test is performed. The control shows initial hemolysis at 0.50% NaCl and complete hemolysis at 0.35% NaCl. The patient sample has initial hemolysis 60% NaCl and complete hemolysis at 0.45% NaCl. What does this indicate? a. The patient is exhibiting increased osmotic fragility. b. The patient has AIHA. c. The patient is not exhibiting decreased osmotic fragility. d. The control is erroneous, so patient results are invalid.
a. The patient is exhibiting increased osmotic fragility.
A 5-year-old boy is admitted to the pediatric unit with a fractured tibia. Routine blood work is ordered. His PB smear shows moderate spherocytosis. An osmotic fragility is also ordered, and the test result indicates an increased osmotic fragility. Based on these findings, what is the most likely defect? a. Hereditary elliptocytosis b. Hereditary spherocytosis c. Microangiopathic hemolytic anemia from trauma d. Cold autoimmune hemolytic anemia
b. Hereditary spherocytosis
Which of the following is found in megaloblastic anemia but not in nonmegaloblastic macrocytic anemia? a. Macrocytic erythrocytes b. Howell-Jolly bodies c. Heinz bodies d. Hypochromia
b. Howell-Jolly bodies
Which of the following describes the bone marrow in a patient with Fanconi's syndrome? a. Hypercellular with erythroid hyperplasia b. Hypocellular with normal M:E ratio c. Normocellular with decreased M:E ratio d. Hypocellular with elevated M:E ratio
b. Hypocellular with normal M:E ratio
Select the best statement from the following choices to describe the pathogenesis of PNH. a. Increased hemolytic episodes are produced as a result of complement binding to erythrocytes via IgG antibodies in cold temperatures. b. Increased hemolytic episodes are produced as a result of missing cell membrane proteins that regulate complement activation. c. Increased hemolytic episodes are produced as a result of missing enzymes necessary for ATP production in erythrocytes. d. Increased hemolytic episodes are produced as a result of initiation of an immune response against erythrocytes.
b. Increased hemolytic episodes are produced as a result of missing cell membrane proteins that regulate complement activation.
Pernicious anemia is a malabsorption of vitamin B12 from what component deficiency? a. Gastric juice b. Intrinsic factor c. Histamine d. Folate
b. Intrinsic factor
Pernicious anemia is a disorder characterized by: a. Antivitamin B12 antibodies b. Lack of IF c. Improper dietary intake of vitamin B12 d. Increased absorption of vitamin B12
b. Lack of IF
What conditions listed can result in macrocytic anemia without megaloblastosis? a. Diabetes b. Liver disease c. Myelodysplastic syndrome d. Pernicious anemia
b. Liver disease
What diagnosis is suggested if part II of the Schilling test is more than 7.5% when part I was 1%? a. Sprue b. Pernicious anemia c. Renal disease d. Celiac disease
b. Pernicious anemia
Which of the following is among the diagnostic criteria for aplastic anemia? a. Granulocyte count > 0.5 x 109/L b. Platelet count < 20 x 109/L c. Anemia d. Anemia with corrected reticulocyte count >1%
b. Platelet count < 20 x 109/L
Which of the following statements best describes the function of DAF in the body? a. Facilitates the amplification of the complement cascade by activating C3 convertase b. Prevents the amplification of C3/C5 convertase activity c. Facilitates complement binding on erythrocytes d. Prevents apoptosis of erythrocyte precursors
b. Prevents the amplification of C3/C5 convertase activity
Which of the following is a quantitative test for G6PD? a. Fluorescent spot test b. Quantitative NADP rate reduction test c. NADP dye reduction test d. Ascorbate cyanide test
b. Quantitative NADP rate reduction test
Which test is a better indicator of folate status in the body over the past month? a. Folate level b. RBC folate level c. Vitamin B12 level d. MMA
b. RBC folate level
Aplastic anemia is usually first noted by bleeding and the presence of petechial hemorrhages. What clinical sign in the early stages of the disease should cause doubt on the diagnosis of aplastic anemia? a. Hepatosplenomegaly b. Splenomegaly c. Lymph node enlargement d. Fever
b. Splenomegaly
Which of the following tests is used to screen for PNH? a. Immunophenotyping b. Sugar-water test c. Acidified serum test d. All of the above
b. Sugar-water test
What is a viable explanation as to why some individuals suffer stem cell damage when exposed to chemical agents but others do not? a. Longer life span of some red cells b. Superior detoxification c. Autoimmune disorders d. Lack of exposure
b. Superior detoxification
Acquired chronic pure red cell aplasia is a rare disorder encountered in association with several autoimmune disorders. What does the mechanism appear to be? a. B cell-mediated immunosuppression of neutrophils b. T cell-mediated immunosuppression of erythropoiesis c. Cytokine-mediated immunosuppression of erythropoiesis d. Increased serum erythropoietin
b. T cell-mediated immunosuppression of erythropoiesis
It is much easier to become folate-deficient than B12-deficient because: a. The body utilizes folate more quickly than it does vitamin B12. b. The body has larger vitamin B12 stores than folate stores. c. The body has a greater need for folate than for vitamin B12. d. It has not been determined why the body needs folate more than vitamin B12.
b. The body has larger vitamin B12 stores than folate stores.
Which of the following puts a patient taking chloramphenicol at risk for developing aplastic anemia? a. The drug promotes an immune response against stem cells. b. The drug is toxic to the bone marrow. c. The drug results in stem cell resistance to essential cytokines. d. The drug causes decreased cytokine production.
b. The drug is toxic to the bone marrow.
Which of the following subtypes of PNH is characterized by moderate hemolysis and intermediate GPI expression? a. Type I b. Type II c. Type III d. Cannot be determined based on the information given
b. Type II
What CBC parameter would help differentiate Fanconi's syndrome from Diamond-Blackfan anemia? a. MCV b. WBC and PLT counts c. RBC counts d. Differential
b. WBC and PLT counts
Which of the following disorders is characterized by increased erythrocyte membrane sphingomyelin and decreased lecithin? a. HS b. HE c. Acanthocytosis d. HPP
c. Acanthocytosis
The presence of which poikilocyte in the peripheral blood could indicate G6PD deficiency? a. Schistocyte b. Spherocyte c. Blister cell d. Elliptocyte
c. Blister cell
Chronic renal disease is a common cause of anemia. Which of the following is one of the possible causes of anemia in chronic renal disease? a. Increased erythropoietin production b. Increased erythrocyte survival c. Blood loss d. Iron production
c. Blood loss
An analyzed blood specimen reveals the following: the MCV is increased, the hemoglobin level is decreased, and the reticulocyte count is normal or low. What test could give more information on the cause of the anemia without drawing another blood specimen? a. Serum iron b. Vitamin B12 assay c. Blood smear morphological exam d. Serum alcohol
c. Blood smear morphological exam
Predict the levels of MMA and homocysteine in patients with Vitamin B12 deficiency. a. Both will be normal. b. Both will be decreased. c. Both will be increased. d. MMA will be increased and homocysteine will be normal.
c. Both will be increased.
Hypoproliferative anemia is defined as: a. Decreased hematopoiesis in the bone marrow b. Decreased hematopoiesis in the liver c. Decreased erythropoiesis in the bone marrow d. Ineffective erythropoiesis in the bone marrow
c. Decreased erythropoiesis in the bone marrow
A 55-year-old male is undergoing dialysis to treat his renal failure. Routine blood examination shows a low RBC count, low H&H, and a normal MCV. What is the most important factor contributing to this anemia? a. Ineffective erythropoiesis b. Hemolysis c. Decreased erythropoietin production d. Presence of cytotoxic antibodies to erythropoietin-sensitive cells
c. Decreased erythropoietin production
A 55-year-old male consulted his physician because of weakness, lightheadedness, vertigo, and angina. The patient also complained of sores on his tongue. The physical examination revealed jaundice. The pulse rate was increased, and the spleen and liver were slightly enlarged. The tongue appeared smooth and bright red in color. Family history revealed no clues. Neurological exam revealed quickening of reflex responses. Patient history indicated a total gastrectomy five years earlier for stomach cancer and intermittent heart problems that had not required surgery. The physician ordered the following tests: TEST RESULTS WBC 3.1 ´ 109/L RBC 2.45 ´ 1012/L HGB 10.5 gm/dL HCT 29.0% MCV 118 fl MCH 42.8 pg MCHC 36.2 gm/dL RDW 15.7 PLT 83 ´ 109/L Differential: Polys 49% Stabs 6% Lymphs 30% Mono 8% Eos 5% Basos 2% Peripheral blood: macro-ovalocytes, no hypersegmentation noted. What would be the results of vitamin B12 and/or folate levels in a diagnosis of pernicious anemia? a. Increased folate b. Increased vitamin B12 c. Decreased vitamin B12 d. Decreased folate and vitamin B12
c. Decreased vitamin B12
The SAO variant of HE is associated with: a. Defective pectrin tetramer formation b. Defective protein 4.1 c. Defective band 3 protein and abnormally tight binding to ankyrin d. All of the above
c. Defective band 3 protein and abnormally tight binding to ankyrin
A 3-month-old infant is exhibiting pallor and fatigue. The pediatrician orders a CBC, which displays a low RBC count, H&H, and normal WBC and PLT counts. EPO levels are increased. BM aspirate shows prominent erythroid hypoplasia. Cytogenetic analysis reveals the presence of RPS19 mutation. Based on these findings, what is the patient most likely suffering from? a. CDA II b. Pure red cell aplasia c. Diamond-Blackfan anemia d. Fanconi's syndrome
c. Diamond-Blackfan anemia
Which of the following tests would help differentiate TEC from DBA? a. RBC count and RBC indices b. Bone marrow cellularity and M:E ratio c. Fetal erythrocyte characteristics d. Ham test
c. Fetal erythrocyte characteristics
Patients with renal disease are at risk for developing megaloblastic anemia because of: a. Decreased EPO production and utilization b. Increased NADPH production c. Folate deficiency d. Acute blood loss
c. Folate deficiency
Which of the following is the most common RBC enzymopathy? a. PK b. HK c. G6PD d. GPI
c. G6PD
Which of these disorders has a defective vertical protein interaction between RBC skeleton and the membrane? a. Hereditary elliptocytosis b. Hereditary pyropoikilocytosis c. Hereditary spherocytosis d. Acanthocytosis
c. Hereditary spherocytosis
Aplastic anemia in the acquired form can result from drugs or chemical agent exposure. If the link cannot be made to any environmental factor, what form of anemia does the client have? a. Pure red cell aplasia b. Anemia of chronic renal disease c. Idiopathic d. Chronic
c. Idiopathic
Neurological symptoms are prevalent in Vitamin B12 deficiency because of: a. Antibodies to intrinsic factor b. Decreased secretion of intrinsic factor c. Improper fatty acid metabolism d. Improper metabolism of methionine
c. Improper fatty acid metabolism
The function of the MIRL is to: a. Induce red cell agglutination b. Prevent activation of C3b converting enzyme c. Interfere with C8/C9 activation d. All of the above
c. Interfere with C8/C9 activation
Diamond-Blackfan syndrome anemia is a rare congenital progressive erythrocyte aplasia. What is the most probable defect causing this rare disorder? a. Deficiency of erythropoietin b. Antibodies against erythropoietin c. Intrinsic defect of erythroid progenitor cells d. Increased reticulocytes
c. Intrinsic defect of erythroid progenitor cells
The majority of PA patients have antibodies against what component necessary for vitamin B12 absorption? a. Parietal cells b. Vitamin B12 c. Intrinsic factor d. Blocking antibody
c. Intrinsic factor
What congenital defects are found in Fanconi's syndrome? a. Hypergonadism b. Skin hypopigmentation c. Mental retardation d. Thrombocytic purpura
c. Mental retardation
In hereditary spherocytosis, the erythrocytes become trapped in the splenic cords and run out of ATP to pump out excessive ions. What is the ion involved? a. K+ b. Chloride c. Na+ d. Oxygen
c. Na+
What is the principal confirmation test in the diagnosis of HS? a. Erythrocyte indices b. Erythrocyte survival test c. Osmotic fragility test d. Hemoglobin electrophoresis
c. Osmotic fragility test
A sample has the following test results: hemoglobin 9 g/dL, autohemolysis increased after 48 hours, and increased hemolysis observed with the Ham test and sucrose hemolysis test. Based on these findings, what is the patient most likely suffering from? a. HS b. HE c. PNH d. PCH
c. PNH
Hereditary pyropoikilocytosis differs in red cell morphology from other erythrocyte membrane defects by what characteristic cell shape? a. Spherocyte b. Elliptocyte c. Schistocyte d. Target cell
c. Schistocyte
The characteristic "fish-mouth" central pallor seen in overhydrated hereditary stomatocytosis is caused by an excessive influx of what in the RBCs? a. Potassium b. Chloride c. Sodium and water d. Sodium
c. Sodium and water
Aplastic anemia is most often caused by: a. Drugs b. Radiation c. Unknown causes d. Infectious agents
c. Unknown causes
Paroxysmal nocturnal hemoglobinuria has four basic disease mechanisms: hyperhemolysis, venous thrombosis, infection, and bone marrow hypoplasia. Which of these mechanisms is the most severe complication? a. Hyperhemolysis b. Infection c. Venous thrombosis d. Bone marrow hypoplasia
c. Venous thrombosis
Megaloblastic anemia is most often caused by deficiencies of what two components? a. Iron and transferrin b. Ferritin and hemoglobin c. Vitamin B12 and folate d. Iron and liver disease
c. Vitamin B12 and folate
A 55-year-old male consulted his physician because of weakness, lightheadedness, vertigo, and angina. The patient also complained of sores on his tongue. The physical examination revealed jaundice. The pulse rate was increased, and the spleen and liver were slightly enlarged. The tongue appeared smooth and bright red in color. Family history revealed no clues. Neurological exam revealed quickening of reflex responses. Patient history indicated a total gastrectomy five years earlier for stomach cancer and intermittent heart problems that had not required surgery. The physician ordered the following tests: TEST RESULTS WBC 3.1 ´ 109/L RBC 2.45 ´ 1012/L HGB 10.5 gm/dL HCT 29.0% MCV 118 fl MCH 42.8 pg MCHC 36.2 gm/dL RDW 15.7 PLT 83 ´ 109/L Differential: Polys 49% Stabs 6% Lymphs 30% Mono 8% Eos 5% Basos 2% Peripheral blood: macro-ovalocytes, no hypersegmentation noted. What is (are) the most appropriate reflex test(s)? a. Iron studies b. Bilirubin c. Vitamin B12 and folate levels d. Hemoglobin electrophoresis
c. Vitamin B12 and folate levels
The defect in DNA synthesis associated with megaloblastic anemia affects which cells? a. Only erythrocytes b. Only gastric cells and WBCs c. Only neurological cells d. All dividing cells
d. All dividing cells
Exposure to which of the following does not induce hemolysis from G6PD deficiency? a. Primaquine b. Fava beans c. Divicine d. Aspirin
d. Aspirin
All of the following are considered diagnostic criteria for aplastic anemia except: a. Granulocyte count 0.3 x 109/L b. Platelet count 5 x 109/L c. Anemia with absolute reticulocyte count of 22 x 109/L d. BM cellularity of 32%
d. BM cellularity of 32%
Patients with blind loop syndrome can have vitamin B12 deficiency complications resulting from: a. Inadequate diet b. Increased minimum daily requirements c. Malabsorption d. Biologic competition
d. Biologic competition
Immunophenotyping is performed on a patient suspected of having PNH. What monoclonal antibody specificity should be ordered? a. CD33 and CD13 b. CD56 and CD4 c. CD3 and CD8 d. CD55 and CD59
d. CD55 and CD59
The screening sucrose hemolysis (sugar-water) test is useful in identifying PNH cells by what mechanism? a. Immunophenotyping b. Hemoglobin electrophoresis c. Erythrocyte survival test d. Complement lysis
d. Complement lysis
Which of the following does not support the diagnosis of G6PD deficiency? a. Presence of Heinz bodies with new methylene blue stain b. Reticulocytosis c. Hemolytic morphology during oxidative event d. Decreased mean cell volume
d. Decreased mean cell volume
A 5-year-old boy with malformed thumbs and microcephaly has been suffering from prolonged bleeding episodes, extreme fatigue, and persistent repetitive infections. Cytogenetic analysis shows increased chromosome breakage with the addition of diepoxybutane. Based on these findings, what is the patient most likely suffering from? a. CDA II b. Pure red cell aplasia c. Diamond-Blackfan anemia d. Fanconi's syndrome
d. Fanconi's syndrome
Which of the following sets of disorders is characterized by increased osmotic fragility? a. HE and PNH b. HS and hereditary overhydrated stomatocytosis c. HS and hereditary dehydrated stomatocytosis d. HS and thalassemia
d. HS and thalassemia
Which of the following disorders is caused by abnormal permeability of the RBC membrane? a. Hereditary elliptocytosis b. Hereditary spherocytosis c. PNH d. Hereditary dehydrated stomatocytosis
d. Hereditary dehydrated stomatocytosis
Recent evidence suggests that the pathophysiology of most cases of acquired aplastic anemia is most likely: a. Drug exposure b. Viral infections c. Defective stem cells d. Immunologic suppression of hematopoiesis
d. Immunologic suppression of hematopoiesis
All of the following disorders can lead to macrocytic anemia except: a. Alcoholism b. Liver disease c. Hypothyroidism d. Iron deficiency
d. Iron deficiency
A 55-year-old male consulted his physician because of weakness, lightheadedness, vertigo, and angina. The patient also complained of sores on his tongue. The physical examination revealed jaundice. The pulse rate was increased, and the spleen and liver were slightly enlarged. The tongue appeared smooth and bright red in color. Family history revealed no clues. Neurological exam revealed quickening of reflex responses. Patient history indicated a total gastrectomy five years earlier for stomach cancer and intermittent heart problems that had not required surgery. The physician ordered the following tests: TEST RESULTS WBC 3.1 ´ 109/L RBC 2.45 ´ 1012/L HGB 10.5 gm/dL HCT 29.0% MCV 118 fl MCH 42.8 pg MCHC 36.2 gm/dL RDW 15.7 PLT 83 ´ 109/L Differential: Polys 49% Stabs 6% Lymphs 30% Mono 8% Eos 5% Basos 2% Peripheral blood: macro-ovalocytes, no hypersegmentation noted. All of the following are expected laboratory findings of PA except: a. Decreased vitamin B12 levels b. Increased MMA in the urine c. Part I of Schilling test = 5%; part II of Schilling test = 10% d. Part I of Schilling test = 2.5%; part II of Schilling test = 2.5%
d. Part I of Schilling test = 2.5%; part II of Schilling test = 2.5%
Which clinical manifestation is common in vitamin B12 deficiency but not in folate deficiency? a. Glossitis b. Pallor c. Fatigue d. Peripheral neuropathy
d. Peripheral neuropathy
Autoantibodies against parietal cells and intrinsic factor are associated with: a. Grave's disease b. Hashimoto's thyroiditis c. Addison's disease d. Pernicious anemia
d. Pernicious anemia
All of the following support the diagnosis of G6PD deficiency except: a. Presence of Heinz bodies with new methylene blue stain b. "Bite cells" c. Hemolytic morphology during oxidative event d. Reticulocytopenia
d. Reticulocytopenia
Which of the following are key features found in nonmegaloblastic macrocytosis? a. Oval red blood cells b. Hypersegmented neutrophils c. Jaundice, glossitis, and neuropathy d. Round macrocytes
d. Round macrocytes
The mode of inheritance for G6PD deficiency is: a. Autosomal recessive b. Autosomal dominant c. No pattern established d. Sex linked
d. Sex linked
Vitamin B12 is a cofactor used in the conversion of _______ to _______. a. Hydroxocobalamin; thymidine b. 5-methyltetrahydrofolate; tetrahydrofolate c. Folate; methionine d. Succinyl CoA; methylmalonic acid
b. 5-methyltetrahydrofolate; tetrahydrofolate
Which of the following treatments offers the best prognosis for aplastic anemia? a. Synthetic cytokines b. Bone marrow transplant c. Immunosuppressive therapy d. Stem cell transplant
b. Bone marrow transplant
Which CDA is characterized by a positive Ham test and a negative sucrose lysis test? a. CDA I b. CDA II c. CDA III d. CDA IV
b. CDA II
In PNH, cells are susceptible to complement-induced lysis. This is thought to result from the lack of what two regulating factors? a. LCAT and lipoprotein b. DAF and MIRL c. HDL and VLDL d. Beta and alpha lipoprotein
b. DAF and MIRL
Which of the following disorders has an abnormal erythrocyte membrane that is abnormally permeable, resulting in the loss of K+ and water and decreased deformability? a. Overhydrated hereditary stomatocytosis b. Dehydrated hereditary stomatocytosis c. Hereditary elliptocytosis d. Hereditary spherocytosis
b. Dehydrated hereditary stomatocytosis
How can infection with the Epstein-Barr virus (EBV) lead to aplastic anemia? a. EBV causes defects in precursor cells. b. EBV infects stem cells, and an immune response to destroy the stem cells is initiated. c. EBV creates immune complexes on the surface of the stem cell that damages it. d. Infection with the virus shuts down cytokine production by macrophages.
b. EBV infects stem cells, and an immune response to destroy the stem cells is initiated.
The majority of glucose catabolism occurs via the: a. Hexose monophosphate shunt b. Embden-Meyerhof pathway c. Luebering-Rapoport pathway d. Synthesis of nucleotides
b. Embden-Meyerhof pathway
Which of the following is used to screen for G6PD deficiency? a. Supravital stain b. Fluorescent spot test c. PB smear d. Cytochemical staining
b. Fluorescent spot test
Which of the following causes horizontal interactions of skeletal protein abnormalities? a. Ankyrin b. Glycophorin C c. Band 3 d. Protein 4.2
b. Glycophorin C
Defects in integral proteins, protein 4.1, and spectrin are associated with which of the following disorders? a. HS b. HE c. Acanthocytosis d. PNH
b. HE
Mary, a 7-year-old, was admitted to the hospital after experiencing severe anemia following a respiratory infection. Her father has a history of RBC membrane abnormality and recently underwent a splenectomy to help alleviate the problem. Upon examination, it was revealed that Mary had hepatosplenomegaly and slight bruising on her forearms. A CBC was ordered, with the following results: HGB 9.0 g/dL HCT 24% WBC 11 × 109/L RBC 3.8 × 1012/L MCV 102 fl MCH 25 pg MCHC 40% PLT 275 × 109/L Peripheral smear: moderate macrocytes and stomatocytes Which of the following is most consistent with a presumptive diagnosis in this case? a. PNH b. HS c. DHS d. HE
b. HS
