Hematology Exam #2

Impaired alpha chain production in alpha thalassemias causes: A) Normal hemoglobin distribution B) Production of Hb Bart's and Hb H. C) Overproduction of hemoglobin F and A2 D) Fusion of alpha and gamma chains

B

Microcytic hypochromic anemias are associated with which functional abnormality? A) Defective folate utilization B) Defective hemoglobin synthesis C) Fibrotic bone marrow D) Bone marrow hypoplasia

B

Platelet estimates are usually performed on a blood smear using which microscopic magnification? A) 10x B) 1000x C) 400x D) 100x

B

Sickle cell trait is not as severe as sickle cell anemia, so why is the sickle cell trait important to diagnose? A) Cells still sickle under the same conditions as with the disease. B) One in four children born to trait parents have the disease. C) Sickle cell trait can mask other diseases. D) Doing so eliminates the need for pharmacologic agent treatment.

B

The most common form of hereditary hemochromatosis is the result of which gene mutation? A) Ferroportin gene B) HFE C) HJV D) Hepcidin gene

B

The peripheral blood smear contains a normocytic, normochromic morphology with marked reticulocytosis. The hemoglobin solubility test is positive. What confirmatory test should follow? A) Haptoglobin analysis B) Hemoglobin electrophoresis C) Bilirubin analysis D) Bone marrow aspiration

B

The strength and type of charge on a hemoglobin molecule enables the separation of hemoglobin variants by electrophoresis. What other significant factor controls this separation? A) Solubility of the molecule B) pH of the medium C) Elevation of hemoglobin concentration D) Heat precipitation

B

What RBC inclusions cannot be seen with Romanowsky stain but can be visualized with supravital stain? A) Pappenheimer bodies B) Heinz bodies C) Cabot rings D) Howell-Jolly bodies

B

What is another name for the phenotype of α-thalassemia major? A) Hemoglobin H disease B) Hydrops fetalis C) β-thalassemia major D) Silent carrier

B

What measurements are used in the percent saturation calculation? A) Ferritin and bone marrow iron B) Serum iron and TIBC C) Transferrin and ferritin D) Serum iron and ferritin

B

What test is the most sensitive and specific for detecting iron-deficiency anemia? A) RDW B) Ferritin C) Vitamin B12 assay D) Transferrin saturation

B

Which laboratory method detects hemoglobinopathies? A) Spectrophotometric analysis at 450nm B) Separation and quantification of protein subunits through an electrical field C) Careful examination of peripheral blood cellular components D) Turbidimetric analysis of whole blood

B

All of the following laboratory tests can be used for evaluation of hemolysis except: A) Total protein B) Hemopexin C) Haptoglobin D) Bilirubin

?

Iron-deficiency anemia can occur from what three mechanisms? A) ALAS deficiency, blood loss, decreased transferrin B) Inadequate iron intake, impaired iron absorption, decreased hepcidin C) Mutation of HFE gene, decreased transferrin, increased hepcidin D) Impaired iron absorption, blood loss, inadequate iron intake

?

When three of the four alpha genes are deleted, what is the disorder known as? A) Hemoglobin H disease B) α-thalassemia C) Hydrops fetalis D) β-thalassemia

?

A 2-year-old black male was being evaluated at County Clinic for swollen hands and feet that were extremely painful. Upon physical examination, the child was found to have a fever and hepatosplenomegaly and was physically small for his age. The child was of Jamaican descent. Patient history revealed that the child's birth weight was normal, but since then, he had been consistently underweight and undersized for his age group. Family history was not readily available. The physician had the child admitted to the hospital for workup and ordered the following CBC. TEST RESULTS WBC 16.6 ´ 109/L RBC 2.53 ´ 1012/L HGB 7.5 gm/dL HCT 21.4% MCV 84.6 fl MCH 18.9 pg MCHC 35.0 g/dL RDW 18.9 PLT 511 ´ 109//L Differential: Polys 84% Stabs (bands?) 10% Stab not commonly used Lymphs 5% Eos 1% NRBCs 12 Based on these findings, what test would be the logical next step? A) Sickle solubility test B) Osmotic fragility C) Peripheral blood smear D) Bone marrow aspirate

A

A patient sample has an RBC count of 4.30 × 1012/L and a reticulocyte count of 4%. What is the absolute retic count? A) 172 × 109/L B) 350 × 109/L C) 90 × 109/L D) 142 × 109/L

A

Which of the following patients would have the highest percentage of hemoglobin S? A) An adult with SC disease B) An adult with sickle cell disease C) A neonate with sickle cell disease D) An adult with sickle cell trait

B

Which poikilocyte is indicated by the arrows in the following image? A) Macrocytes B) Elliptocytes C) Spherocytes D) Schistocytes

B

A bone marrow aspirate is hypercellular and has an M:E ratio of 1:4. What can be concluded from this? There is: A) Erythroid hypoplasia B) Myeloid hyperplasia C) Erythroid hyperplasia D) Myeloid hypoplasia

C

Accumulation of heterogeneous aggregates of iron is called: A) Ferritinemia B) Transferrinuria C) Hemosiderosis D) Sideroblastosis

C

Disease that results from a molecular defect in hemoglobin synthesis in RBCs is termed: A) Asynchrony B) Porphyria C) Hemoglobinopathy D) Thalassemia

C

During a discussion of RBC indices, a medical technologist of Cambodian origin mentioned that her own red cells had been small in a CBC performed 2 years earlier in a routine physical. A more recent CBC showed the following results. TEST RESULTS WBC 9.3 ´ 109/L RBC 6.11 ´ 1012/L HGB 13.0 g/dL HCT 41.8% MCV 69.6 fL MCH 22.5 pg MCHC 31.0 g/dL RDW 15.8 PLT 351 ´ 109/L Differential Within normal limits Based on the demographics of this patient, what disorder is suspected? A) Sicklecell disease B) Hemoglobin C disease C) Hemoglobin E disease

C

Hemoglobin C disease has altered solubility properties similar to hemoglobin S disease. What accounts for this altered solubility? A) The hemoglobin remains in the tense structure. B) Heme iron is in the ferric form rather than ferrous form. C) A nonpolar amino acid is substituted for a polar amino acid. D) A polar amino acid is substituted near the pocket where heme binds.

C

Howell-Jolly bodies are red cell inclusions associated with what disease state? A) Lead poisoning B) G6PD deficiency C) Megaloblastic anemia D) Normal reticulocytes

C

Identify the poikilocytes indicated by the arrows in the following image. A) Drepanocytes B) Acanthocytes C) Spherocytes D) Schizocytes

C

Molecular analysis on a 4-month-old Chinese patient revealed three alpha genes deleted on chromosome 16. The physician ordered a hemoglobin electrophoresis on this patient. What is the expected finding? A) 80% A1; 10% F; 10% A2 B) 95% Hgb Bart's C) 95% Hgb H D) 80% F; 15% A1; 5% A2

C

Patients with sickle cell disease suffer from vaso-occlusive crises because: A) The altered hemoglobin facilitates the formation of bar-shaped hemoglobin crystals that occlude blood vessels. B) The altered hemoglobin interferes with globin chain assembly, which impedes blood flow in the vessels. C) The altered hemoglobin facilitates tactoid crystal formation that alters the shape of the red blood cell, resulting in impeded blood flow in vessels. D) The altered hemoglobin impairs the hemoglobin's ability to effectively transport oxygen that causes vaso-occlusion.

C

Remnants of DNA inside an RBC produce which type of inclusion? A) Basophilic stippling B) Cabot rings C) Howell-Jolly bodies D) Pappenheimer bodies

C

The geographic prevalence of α-thalassemia is highest in which ancestry group? A) Canadian, African, Indian B) Mediterranean, American Indian C) Asian, Mediterranean, African D) South American, African, Asian

C

What disease state is associated with helmet cells? A) Iron deficiency B) Thalassemia C) Heart valve hemolysis D) Diabetes

C

What is one of the disease states associated with the presence of target cells? A) Uremia B) Hemolytic anemia C) Thalassemia D) Sickle cell anemia

C

What is the purpose of treating sickle cell patients with hydroxyurea? A) To dilute the amount of sickled red blood cells B) To destroy the sickling red blood cells by initiating an immune response C) To increase the level of hemoglobin F that will reduce intracellular sickling D) To convert Hgb S into Hgb A

C

What is the treatment of choice for thalassemias? A) Radiation B) Bone marrow transplant C) Supportive transfusions D) Chemotherapy

C

What should be reported about the RBCs in the following image and what affect would this have on the CBC results? A) codocytes; increased RBC count B) spherocytes; increased MCV C) rouleaux; no affect on the CBC results D) agglutination; increased MCV and decreased RBC count

C

What should be reported about the RBCs indicated by the arrows in the following image? A) Drepanocytes B) Macrocytes C) Hypochromasia D) Ovalocytes

C

What staining method can be used to differentiate Pappenheimer bodies from reticulocytes? A) PAS B) Phase microscopy on wet prep C) Perl's Prussian blue D) New methylene blue

C

What test could provide the diagnosis differential for β-thalassemia minor and iron deficiency? A) Iron studies B) Bone marrow aspirate C) Hemoglobin electrophoresis D) PB smear

C

When a diagnosis of thalassemia is suspected, which test should always be done? A) Bone marrow aspiration B) Vitamin B12 assay C) Hemoglobin electrophoresis D) Iron studies

C

Which RBC inclusion is characterized by diffusely staining RNA precipitate that can be seen when blood is stained with Wright′s stain? A) Howell-Jolly bodies B) Pappenheimer bodies C) Basophilic stippling D) Heinz bodies

C

Which of the following represents an example of an intrinsic erythrocyte defect? A) Antibody-coated RBCs B) Snake venom C) Hb S D) Disseminated intravascular coagulation

C

A peripheral blood smear has an even distribution of both normocytic normochromic cells and microcytic hypochromic cells. What is this phenomenon called? A) Extramedullary hematopoiesis B) Nuclear-cytoplasmic asynchrony C) Monoclonal cell population D) Dimorphic cell population

D

A storage form of iron is: A) Hemoglobin B) Pappenheimer bodies C) Transferrin D) Ferritin

D

Alcoholics have an increased incidence of sideroblastic anemia because of which of the following? A) Impaired transport of iron into erythroid precursors B) Impaired utilization of globin chains C) Abnormal stem cells D) Abnormal activity of enzymes of heme synthesis

D

An instrument printout indicates an MCV of 63 fl and an MCHC of 26 g/dl. How will the red cells appear under the microscope? A) Normocytic, hypochromic B) Normocytic, normochromic C) Macrocytic, normochromic D) Microcytic, hypochromic

D

Choose the thalassemia with the best prognosis from the following choices. A) Hgb H disease B) Beta thalassemia minor C) Beta thalassemia intermedia D) Silent carrier alpha thalassemia

D

How would you differentiate between an acanthocyte and an echinocyte? A) The presence or absence of a fish-mouth central pallor B) The size of the cell C) The number of spikes D) The variety in length and distribution of the spikes

D

In hemoglobin C disease, how does the mutation affect the hemoglobin molecule? A) It is unstable. B) It has increased oxygen affinity. C) It cannot carry oxygen. D) It forms insoluble aggregates.

D

Thalassemias are defined as: A) Qualitative defects in globin chain synthesis B) Kinetic defects of iron in heme synthesis C) Structural defects in heme synthesis D) Quantitative defects in globin chain synthesis

D

The following data were reported by an instrument: RBC = 5.00 × 1012/L, hemoglobin = 8.5 g/dL and hematocrit = 32%. Calculate the MCHC. A) 17.0 B) 29.5 C) 64.0 D) 26.6

D

The hereditary form of sideroblastic anemia is most commonly the result of: A) Increased absorption of iron B) Mutation of the HFE gene C) Decreased hepcidin synthesis D) Abnormal ALAS

D

What organ is the primary storage depot for iron? A) Bone marrow B) Stomach C) Duodenum D) Liver

D

What preoperative therapy is effective in a diagnosed sickle cell patient? A) Stem cell transplant B) Gene therapy C) Hydroxyurea administration D) Transfusion of red blood cells

D

Which of the following clinical findings is associated with anemia: A) Fatigue B) Dyspnea C) Skin pallor D) All of the above

D

Which of the following has the highest prevalence worldwide? A) Hgb D B) Hgb C C) Hgb E D) Hgb S

D

Which of the following poikilocytes would be associated with a hemolytic anemia? A) Oval macrocytes B) Echinocytes C) Dacryocytes D) Spherocytes

D

Why are bone changes and deformities common findings in β-thalassemias? A) The globin chain synthesis is increased. B) The impaired globin chain synthesis causes erythroid hypoplasia. C) The impaired globin chain synthesis causes impaired oxygen delivery to the bone tissue, which causes bone deformities. D) The impaired globin chain synthesis causes increased erythropoiesis resulting in bone marrow expansion.

D

α-thalassemia major results from deletion in _____ alleles of the alpha chain gene. A) 2 B) 1 C) 3 D) 4

D

A patient with a bleeding problem is complaining to her physician about excessive fatigue. Physical examination reveals a thin, pale woman with pale mucosal membranes. Significant CBC findings include an MCV of 70 fl. Based on these findings, follow-up testing should be ordered for: A) Iron studies B) Vitamin B12 and folate assays C) Coagulation screening tests D) A bone marrow aspirate

A

All of the following can lead to the development of thalassemias except: A) Exposure to a mutagenic agent B) Deletion mutation C) Frameshift mutation D) Substitution mutation

A

An increased red cell distribution width (RDW) usually indicates what abnormality? A) Variation in erythrocyte size B) Decrease in ratio of hemoglobin mass to volume C) Increase of average weight of hemoglobin D) Increase of average erythrocyte volume

A

An instrument printout reveals an RDW of 33. Which of the following would best correlate with those results? A) The presence of a dimorphic cell population on the PB smear B) The presence of macro-ovalocytes on the PB smear C) The presence of marked polychromasia on the PB smear D) The presence of spherocytes on the PB smear

A

Anemia of chronic disease is characterized by: A) A block in iron release from macrophages B) A block in insertion of iron into the porphyrin ring of heme C) A block in globin synthesis D) An inadequate intake of iron

A

Codocytes are associated with all of the following conditions except: A) Hemolytic anemia B) Thalassemias C) Hemoglobinopathies D) Iron-deficiency anemia

A

Evolution dictates that patients with sickle cell disease have an inherent resistance to which of the following? A) Plasmodium infections B) Hemolytic transfusion reactions C) Babesia infections D) Acute leukemia

A

Hemoglobinopathy results from a genetic abnormality of which structure within the hemoglobin molecule? A) Globin chain B) Heme C) Iron D) Porphyrin

A

Identify the RBC inclusion in the following image. A) Howell Jolly body B) Siderotic granule C) Heinz body D) Basophilic stippling

A

Impaired beta chain production translates to what on hemoglobin electrophoresis? A) An increase in Hb F and Hb A2 B) An increase in Hb A1 C) A decrease in Hb S D) An increase in Hb H

A

Thalassemias are produced as a result of: A) Quantitative defects in globin chain synthesis B) Structural defects in heme synthesis C) Qualitative defects in globin chain synthesis D) Molecular defects in hemoglobin synthesis

A

The defect in the following choices that will produce hemoglobin C disease are: A) Beta 6 substitution of lysine for glutamic acid B) Alpha 26 substitution of glutamic acid for threonine C) Beta 6 substitution of glutamic acid for valine D) Alpha 4 substitution of thymidine for methionine

A

The peripheral blood smear of a patient with anemia reveals normochromic RBCs. Which of the following is likely to be the cause of the anemia? A) Chronic inflammation B) Thalassemia C) Porphyrias D) Sickle cell anemia

A

What red cell morphology marks most types of thalassemia? A) Target cells B) Normal findings C) Basophilic stippling D) Polychromasia

A

Which laboratory test measures the maximum amount of iron able to be bound to transferrin in the serum? A) TIBC B) Percent transferrin saturation C) Serum iron D) Ferritin

A

Which of the following is not characteristic of iron deficiency anemia? Question options: A) Decreased ZPP B) Decreased serum iron C) Decreased serum ferritin D) Increased TIBC

A

12. Calculate the MCV from the results of a patient′s CBC:RBC = 3.50 × 1012/LHemoglobin = 12.0 g/dLHematocrit = 40% A) 30 B) 114 C) 31 D) 100

B

A fusion of delta and beta globin chains produces which of the following hemoglobins? A) Hemoglobin Portland B) Hemoglobin Lepore C) Hemoglobin Bart's D) Hemoglobin F

B

A patient experiencing intravascular hemolysis would likely show which of the following: A) Decreased reticulocyte count B) Hemosiderinuria C) Leukocytosis D) Negative DAT

B

All of the following disorders would have normal-to-elevated ferritin levels except: A) Sideroblastic anemia B) Iron-deficiency anemia C) Lead poisoning D) Anemia of chronic disease

B

All of the following morphology are typically seen in beta thalassemia patients except: A) Microcytes B) Drepanocytes C) Polychromasia D) Codocytes

B

CBC results on a pregnant patient indicate an MCV of 112 fl. What would be the most appropriate reflex test? A) Bilirubin B) Folate assay C) LDH D) Iron studies

B

During a discussion of RBC indices, a medical technologist of Cambodian origin mentioned that her own red cells had been small in a CBC performed 2 years earlier in a routine physical. A more recent CBC showed the following results. TEST RESULTS WBC 9.3 ´ 109/L RBC 6.11 ´ 1012/L HGB 13.0 g/dL HCT 41.8% MCV 69.6 fL MCH 22.5 pg MCHC 31.0 g/dL RDW 15.8 PLT 351 ´ 109/L Differential Within normal limits What reflex test is most appropriate for investigating these results? A) Vitamin B12 and folate assays B) Hemoglobin electrophoresis and iron studies C) Cytogenetic analysis D) Bone marrow aspirate

B

Hemoglobin H disease can be detected by which of the following laboratory methods? A) Bone marrow aspirate B) Brilliant cresyl blue staining for Hb H inclusions C) Complete blood count D) PB smear for Heinz bodies

B

Identify the poikilocytes in the following image and indicate a possible mechanism for their formation. A) Stomatocytes; liver disease B) Schisocytes; intravascular damage C) Codocytes; intracellular defect D) Elliptocytes; iron deficiency

B

If a patient had hemoglobin S and hemoglobin A identified by electrophoresis and a positive hemoglobin solubility test, what is the patient's most likely condition? A) Hemoglobin SC disease B) Sickle cell trait C) Hemoglobin S/D disease D) Hemoglobin C trait

B