Question 1

The anemia of thalassemia is morphologically characterized as __________ anemia.

Accepted Answer

A)  Macrocytic/hyperchromic 
B)  Microcytic/hypochromic 
C)  Normocytic/normochromic 
D)  Microcytic/normochromic 
E)  None of the above 
A)  Macrocytic/hyperchromic 
B)  Microcytic/hypochromic 
C)  Normocytic/normochromic 
D)  Microcytic/normochromic 
E)  None of the above

Question 2

Therapy for thalassemia major patients may include:

Accepted Answer

A)  High blood transfusion program 
B)  Splenectomy 
C)  Bone marrow transplant 
D)  All of the above 
E)  None of the above 
A)  High blood transfusion program 
B)  Splenectomy 
C)  Bone marrow transplant 
D)  All of the above 
E)  None of the above

Question 3

Which of the following findings would not be indicative of heterozygous F1F1F1S1F1F1F10 thalassemia?

Accepted Answer

A)  Hemoglobin A2 level of 3.5% to 7% 
B)  Hemoglobin F level of 2% to 5% 
C)  Hemoglobin A level of 65% to 85% 
D)  Hemoglobin A level of 90% to 95% 
E)  D and A are correct 
A)  Hemoglobin A2 level of 3.5% to 7% 
B)  Hemoglobin F level of 2% to 5% 
C)  Hemoglobin A level of 65% to 85% 
D)  Hemoglobin A level of 90% to 95% 
E)  D and A are correct

Question 4

Hemoglobin electrophoresis of a 2-year-old patient showed migration of A2 and F on cellulose acetate at pH 8.4. Quantitation studies showed 5% A2 and 95% F. What thalassemia is most likely present?

Accepted Answer

A)  Alpha thalassemia 
B)  Beta thalassemia 
C)  Beta-sickle thalassemia 
D)  Alpha-sickle thalassemia 
E)  None of the above 
A)  Alpha thalassemia 
B)  Beta thalassemia 
C)  Beta-sickle thalassemia 
D)  Alpha-sickle thalassemia 
E)  None of the above

Question 5

What thalassemia-associated variant will result in sickling crisis in severe hypoxic conditions?

Accepted Answer

A)  Beta thalassemia/hemoglobin E 
B)  Beta thalassemia/hemoglobin C 
C)  Beta thalassemia/hemoglobin S 
D)  Alpha thalassemia/sickle cell 
E)  Hemoglobin SC 
A)  Beta thalassemia/hemoglobin E 
B)  Beta thalassemia/hemoglobin C 
C)  Beta thalassemia/hemoglobin S 
D)  Alpha thalassemia/sickle cell 
E)  Hemoglobin SC

Question 6

The distinguishing feature of both alpha thalassemia-one-trait and alpha thalassemia-two-trait conditions compared to Bart's hydrops fetalis or hemoglobin H disease is:

Accepted Answer

A)  Lack of hemoglobin Bart's at birth 
B)  Disappearance of hemoglobin Bart's with development 
C)  Persistence of high levels of fetal hemoglobin into adulthood. 
D)  Excessive amounts of hemoglobin Portland at birth 
E)  None of the above 
A)  Lack of hemoglobin Bart's at birth 
B)  Disappearance of hemoglobin Bart's with development 
C)  Persistence of high levels of fetal hemoglobin into adulthood. 
D)  Excessive amounts of hemoglobin Portland at birth 
E)  None of the above

Question 7

Which clinical expression of thalassemia is associated with a healthy silent carrier showing no clinical symptoms and minimal to no hematologic abnormalities?

Accepted Answer

A)  Thalassemia major 
B)  Thalassemia minor 
C)  Thalassemia intermedia 
D)  Bart's hydrops fetalis 
E)  None of the above 
A)  Thalassemia major 
B)  Thalassemia minor 
C)  Thalassemia intermedia 
D)  Bart's hydrops fetalis 
E)  None of the above

Question 8

A 5-year-old boy presents with a hemoglobin and hematocrit of 10 g/dL and 30% respectively, a serum bilirubin of 3.0 mg/dL, a RBC count of 4.0 million/µL, and splenomegaly. Which clinical expression of thalassemia best correlates with the data?

Accepted Answer

A)  Thalassemia major 
B)  Thalassemia minor 
C)  Thalassemia intermedia 
D)  Bart's hydrops fetalis 
E)  HPFH 
A)  Thalassemia major 
B)  Thalassemia minor 
C)  Thalassemia intermedia 
D)  Bart's hydrops fetalis 
E)  HPFH

Question 9

All of the following comprise the clinical categories of alpha thalassemia except:

Accepted Answer

A)  Bart's hydrops fetalis syndrome 
B)  Hemoglobin H disease 
C)  Hereditary persistence of fetal hemoglobin 
D)  Alpha thalassemia 1 
E)  Alpha thalassemia 2 
A)  Bart's hydrops fetalis syndrome 
B)  Hemoglobin H disease 
C)  Hereditary persistence of fetal hemoglobin 
D)  Alpha thalassemia 1 
E)  Alpha thalassemia 2

Question 10

What hemoglobin consists of four gamma chains?

Accepted Answer

A)  Hemoglobin H 
B)  Hemoglobin Bart's 
C)  Hemoglobin F 
D)  Hemoglobin A2 
E)  None of the above 
A)  Hemoglobin H 
B)  Hemoglobin Bart's 
C)  Hemoglobin F 
D)  Hemoglobin A2 
E)  None of the above

Exam 12: Hemolytic Anemias: Intracorpuscular Defects: Iv Thalassemia

The anemia of thalassemia is morphologically characterized as __________ anemia.

Therapy for thalassemia major patients may include:

Which of the following findings would not be indicative of heterozygous  thalassemia?

Hemoglobin electrophoresis of a 2-year-old patient showed migration of A2 and F on cellulose acetate at pH 8.4. Quantitation studies showed 5% A2 and 95% F. What thalassemia is most likely present?

What thalassemia-associated variant will result in sickling crisis in severe hypoxic conditions?

The distinguishing feature of both alpha thalassemia-one-trait and alpha thalassemia-two-trait conditions compared to Bart's hydrops fetalis or hemoglobin H disease is:

Which clinical expression of thalassemia is associated with a healthy silent carrier showing no clinical symptoms and minimal to no hematologic abnormalities?

A 5-year-old boy presents with a hemoglobin and hematocrit of 10 g/dL and 30% respectively, a serum bilirubin of 3.0 mg/dL, a RBC count of 4.0 million/µL, and splenomegaly. Which clinical expression of thalassemia best correlates with the data?

All of the following comprise the clinical categories of alpha thalassemia except:

What hemoglobin consists of four gamma chains?

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

Hemoglobin electrophoresis of a 2-year-old patient showed migration of A₂ and F on cellulose acetate at pH 8.4. Quantitation studies showed 5% A₂ and 95% F. What thalassemia is most likely present?