Question 1

What is another name for thalassemia major?

Accepted Answer

A)  Cooley's anemia 
B)  Fanconi's anemia 
C)  Alpha thalassemia 
D)  Schilling's syndrome 
E)  None of the above 
A)  Cooley's anemia 
B)  Fanconi's anemia 
C)  Alpha thalassemia 
D)  Schilling's syndrome 
E)  None of the above A

Question 2

Decreased or absent alpha-chain production will result in:

Accepted Answer

A)  Decreased gamma chain production during fetal life and adult life 
B)  Excess gamma chain production during fetal life and at birth 
C)  No gamma chain production during fetal life 
D)  No gamma chain production during adult life 
E)  None of the above 
A)  Decreased gamma chain production during fetal life and adult life 
B)  Excess gamma chain production during fetal life and at birth 
C)  No gamma chain production during fetal life 
D)  No gamma chain production during adult life 
E)  None of the above B

Question 3

The characteristic facial changes in a patient diagnosed with thalassemia major include hypertrophy of maxilla, widely spared eyes, and prominent cheekbones. This is the result of:

Accepted Answer

A)  Expansion of marrow space 
B)  Extramedullary hematopoiesis 
C)  Vitamin B12 deficiency 
D)  Increase in hemoglobins 
E)  None of the above 
A)  Expansion of marrow space 
B)  Extramedullary hematopoiesis 
C)  Vitamin B12 deficiency 
D)  Increase in hemoglobins 
E)  None of the above A

Question 4

What lethal disease is characterized by homozygosity of the F1F1F1S1F1F1F100-thalassemia gene haplotype in utero?

Accepted Answer

A)  Hydrops fetalis 
B)  Hemolytic disease of the newborn (HDN) 
C)  Hemoglobin Portland disease 
D)  Hemoglobin H disease 
E)  Hemoglobin Zurich 
A)  Hydrops fetalis 
B)  Hemolytic disease of the newborn (HDN) 
C)  Hemoglobin Portland disease 
D)  Hemoglobin H disease 
E)  Hemoglobin Zurich

Question 5

It has been hypothesized that a person who is heterozygous for the thalassemia gene is resistant to malaria caused by __________.

Accepted Answer

A)  Plasmodium ovale 
B)  Plasmodium falciparum 
C)  Plasmodium vivax 
D)  Plasmodium malariae 
E)  None of the above 
A)  Plasmodium ovale 
B)  Plasmodium falciparum 
C)  Plasmodium vivax 
D)  Plasmodium malariae 
E)  None of the above

Question 6

A 13-year-old boy presents with the following hematologic results: RBC = 5.76 million, Hb, 10.4 g/dL; Hct, 35.9%; MCV (mean corpuscular volume), 63 fL: MCH (mean corpuscular hemoglobin), 18.2 pg; MCHC (mean corpuscular hemoglobin concentration), 29%; and RDW (red blood cell distribution width), 13.5. Morphology includes mild anisocytosis and occasional target cells. All iron studies are normal. Electrophoresis on cellulose acetate at pH 8.4 indicates an increase in HbF and HbA2. What thalassemia syndrome is evident?

Accepted Answer

A)  αα-thalassemia 
B)  ββ-thalassemia 
C)  Hemoglobin E disease 
D)  Sideroblastic anemia 
E)  None of the above 
A)  αα-thalassemia 
B)  ββ-thalassemia 
C)  Hemoglobin E disease 
D)  Sideroblastic anemia 
E)  None of the above

Question 7

What percentage of normal hemoglobin consists of hemoglobin A?

Accepted Answer

A)  Gene addition 
B)  Gene fusion 
C)  Point mutation 
D)  Point elongation 
E)  None of the above 
A)  Gene addition 
B)  Gene fusion 
C)  Point mutation 
D)  Point elongation 
E)  None of the above

Question 8

Which abnormal hemoglobin is produced from a fusion of delta and beta chains?

Accepted Answer

A)  Hemoglobin Lepore 
B)  Hemoglobin Portland 
C)  Hemoglobin Constant Spring 
D)  Hemoglobin Bart's 
E)  Hemoglobin Zurich 
A)  Hemoglobin Lepore 
B)  Hemoglobin Portland 
C)  Hemoglobin Constant Spring 
D)  Hemoglobin Bart's 
E)  Hemoglobin Zurich

Question 9

What hemoglobin structures make up hemoglobin A2?

Accepted Answer

A)  Zeta 2 epsilon 2 
B)  Alpha 2 beta 2 
C)  Alpha 2 gamma 2 
D)  Alpha 2 delta 2 
E)  None of the above 
A)  Zeta 2 epsilon 2 
B)  Alpha 2 beta 2 
C)  Alpha 2 gamma 2 
D)  Alpha 2 delta 2 
E)  None of the above

Question 10

Which type of F1F1F1S1F1F1F10+ thalassemia produces the least amount of beta chains?

Accepted Answer

A)  Type 1 
B)  Type 2 
C)  Type 3 
D)  Type 4 
E)  None of the above 
A)  Type 1 
B)  Type 2 
C)  Type 3 
D)  Type 4 
E)  None of the above

Question 11

Which form of HPFH is characterized by uniform distribution among red cells when stained by the Kleinhauer-Betke method?

Accepted Answer

A)  Hypocellular 
B)  Pancellular 
C)  Heterocellular 
D)  Hypercellular 
E)  Acellular 
A)  Hypocellular 
B)  Pancellular 
C)  Heterocellular 
D)  Hypercellular 
E)  Acellular

Question 12

Homozygosity for the F1F1F1S1F1F1F100 or F1F1F1S1F1F1F10+ thalassemia gene results in:

Accepted Answer

A)  Hemoglobin H disease 
B)  Alpha thalassemia 
C)  Thalassemia major 
D)  Thalassemia minor 
E)  None of the above 
A)  Hemoglobin H disease 
B)  Alpha thalassemia 
C)  Thalassemia major 
D)  Thalassemia minor 
E)  None of the above

Question 13

What is hemoglobin F composed of?

Accepted Answer

A)  Alpha 2, beta 2 
B)  Alpha 2, gamma 2 
C)  Alpha 2, delta 2 
D)  Alpha 2, zeta 2 
E)  None of the above 
A)  Alpha 2, beta 2 
B)  Alpha 2, gamma 2 
C)  Alpha 2, delta 2 
D)  Alpha 2, zeta 2 
E)  None of the above

Question 14

A person native to Thailand would have more probability of presenting with:

Accepted Answer

A)  Thalassemia major 
B)  Alpha thalassemia 
C)  Beta thalassemia 
D)  Gamma-beta thalassemia 
E)  None of the above 
A)  Thalassemia major 
B)  Alpha thalassemia 
C)  Beta thalassemia 
D)  Gamma-beta thalassemia 
E)  None of the above

Question 15

Which non-alpha thalassemias may be expected to have similar if not identical hemoglobin A, A2, and F levels?

Accepted Answer

A)  Homozygous hereditary persistence of fetal hemoglobin (HPFH) and homozygous delta-beta thalassemia 
B)  Homozygous Hgb Lepare and thalassemia intermedia 
C)  Thalassemia major and thalassemia minor 
D)  Heterozygous HPFH and homozygous HPFH 
E)  None of the above 
A)  Homozygous hereditary persistence of fetal hemoglobin (HPFH) and homozygous delta-beta thalassemia 
B)  Homozygous Hgb Lepare and thalassemia intermedia 
C)  Thalassemia major and thalassemia minor 
D)  Heterozygous HPFH and homozygous HPFH 
E)  None of the above

Question 16

In contrast to beta thalassemia, alpha thalassemia usually manifests:

Accepted Answer

A)  After age 40 
B)  At the onset of puberty 
C)  Immediately at birth 
D)  Only in postmenopausal women 
E)  After age 10 
A)  After age 40 
B)  At the onset of puberty 
C)  Immediately at birth 
D)  Only in postmenopausal women 
E)  After age 10

Question 17

Thalassemia is found:

Accepted Answer

A)  Only in the Mediterranean Sea area 
B)  Only in Australia and South Pacific Islands 
C)  Only in Southeast Asia 
D)  Only on the African continent 
E)  Worldwide 
A)  Only in the Mediterranean Sea area 
B)  Only in Australia and South Pacific Islands 
C)  Only in Southeast Asia 
D)  Only on the African continent 
E)  Worldwide

Question 18

Gene deletions are the cause of the majority of alpha thalassemia cases because:

Accepted Answer

A)  There are no areas of homology between the two closely linked alpha genes globin. 
B)  The F1F1F1S1F1F1F102 globin gene is expressed at two to three times the rate of F1F1F1S1F1F1F101 globin gene. 
C)  Great homology in the gene blocks render the areas more susceptible to deletion error during crossover. 
D)  Alpha genes are juxtapositioned to numerous transcriptional mutants. 
E)  None of the above 
A)  There are no areas of homology between the two closely linked alpha genes globin. 
B)  The F1F1F1S1F1F1F102 globin gene is expressed at two to three times the rate of F1F1F1S1F1F1F101 globin gene. 
C)  Great homology in the gene blocks render the areas more susceptible to deletion error during crossover. 
D)  Alpha genes are juxtapositioned to numerous transcriptional mutants. 
E)  None of the above

Question 19

Hemoglobin A consists of:

Accepted Answer

A)  Two alpha (F1F1F1S1F1F1F10) chains and two gamma (F1F1F1S1F1F1F10) chains 
B)  Two alpha chains and two delta (F1F1F1S1F1F1F10) chains 
C)  Two alpha chains and two beta (F1F1F1S1F1F1F10) chains 
D)  Two alpha chains and two epsilon (F1F1F1S1F1F1F10) chains 
E)  None of the above 
A)  Two alpha (F1F1F1S1F1F1F10) chains and two gamma (F1F1F1S1F1F1F10) chains 
B)  Two alpha chains and two delta (F1F1F1S1F1F1F10) chains 
C)  Two alpha chains and two beta (F1F1F1S1F1F1F10) chains 
D)  Two alpha chains and two epsilon (F1F1F1S1F1F1F10) chains 
E)  None of the above

Question 20

The main concern(s) regarding patients with thalassemia major treated with hypertransfusion include(s):

Accepted Answer

A)  Iron overload 
B)  Alloimmunization 
C)  Transfusion-transmitted disease 
D)  All of the above 
E)  None of the above 
A)  Iron overload 
B)  Alloimmunization 
C)  Transfusion-transmitted disease 
D)  All of the above 
E)  None of the above

Exam 12: Hemolytic Anemias: Intracorpuscular Defects: Iv Thalassemia

What is another name for thalassemia major?

Decreased or absent alpha-chain production will result in:

The characteristic facial changes in a patient diagnosed with thalassemia major include hypertrophy of maxilla, widely spared eyes, and prominent cheekbones. This is the result of:

What lethal disease is characterized by homozygosity of the 0-thalassemia gene haplotype in utero?

It has been hypothesized that a person who is heterozygous for the thalassemia gene is resistant to malaria caused by __________.

What percentage of normal hemoglobin consists of hemoglobin A?

Which abnormal hemoglobin is produced from a fusion of delta and beta chains?

What hemoglobin structures make up hemoglobin A2?

Which type of + thalassemia produces the least amount of beta chains?

Which form of HPFH is characterized by uniform distribution among red cells when stained by the Kleinhauer-Betke method?

Homozygosity for the 0 or + thalassemia gene results in:

What is hemoglobin F composed of?

A person native to Thailand would have more probability of presenting with:

Which non-alpha thalassemias may be expected to have similar if not identical hemoglobin A, A2, and F levels?

In contrast to beta thalassemia, alpha thalassemia usually manifests:

Thalassemia is found:

Gene deletions are the cause of the majority of alpha thalassemia cases because:

Hemoglobin A consists of:

The main concern(s) regarding patients with thalassemia major treated with hypertransfusion include(s):

Morphology of Human Blood and Marrow Cells: Hematopoiesis

Bone Marrow

The Red Blood Cell: Structure and Function

Anemia: Diagnosis and Clinical Considerations

Evaluation of Red Blood Cell Morphology and Introduction to Platelet and White Blood Cell Morphology

Iron Metabolism and Hypochromic Anemias

Megaloblastic Anemias

Aplastic Anemia Including Pure Red Cell Aplasia and Congenital Dyserythropoietic Anemia and Paroxysmal Nocturnal Hemoglobinuria

Introduction to Hemolytic Anemias: Intracorpuscular Defects: I Hereditary Defects of the Red Cell Membrane

Hemolytic Anemias: Intracorpuscular Defects: II Hereditary Enzyme Deficiencies

Hemolytic Anemias: Intracorpuscular Defects: III the Hemoglobinopathies

Hemolytic Anemias: Extracorpuscular Defects

Hypoproliferative Anemia: Anemia Associated With Systemic Diseases

Cell Biology, Disorders of Neutrophils, Infectious Mononucleosis, and Reactive Lymphocytosis

Introduction to Leukemia and the Acute Leukemias

Chronic Myeloproliferative Disorders I: Chronic Myelogenous Leukemia

Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

Myelodysplastic Syndromes

Chronic Lymphocytic Leukemia and Related Lymphoproliferative Disorders

The Lymphomas

Multiple Myeloma and Related Plasma Cell Disorders

Lipid Lysosomal Storage Diseases and Histiocytosis

Introduction to Hemostasis

Disorders of Primary Hemostasis: Quantitative and Qualitative Platelet Disorders and Vascular Disorders

Disorders of Plasma Clotting Factors

Interaction of the Fibrinolytic, Coagulation, and Kinin Systems; Disseminated Intravascular Coagulation; and Related Pathology

Introduction to Thrombosis and Anticoagulant Therapy

Quality Control in the Hematology Laboratory

Body Fluid Examination: the Qualitative, Quantitative, and Morphologic Analysis of Serous, Cerebrospinal, and Synovial Fluids

Hematology Methods

Principles of Automated Differential Analysis

Coagulation Procedures

Applications of Flow Cytometry to Hematology and Hemostasis

Molecular Diagnostic Techniques in Hematopathology

Special Stainscytochemistry

Filters

What lethal disease is characterized by homozygosity of the ⁰-thalassemia gene haplotype in utero?

What hemoglobin structures make up hemoglobin A₂?

Homozygosity for the ⁰ or ⁺ thalassemia gene results in:

Which non-alpha thalassemias may be expected to have similar if not identical hemoglobin A, A₂, and F levels?