Question 1

What common renal complication is associated with sickle cell anemia?

Accepted Answer

A)  Renal papillary necrosis 
B)  Glomerular nephritis 
C)  Dactylitis 
D)  Pyelonephritis 
E)  A and D 
A)  Renal papillary necrosis 
B)  Glomerular nephritis 
C)  Dactylitis 
D)  Pyelonephritis 
E)  A and D

Question 2

Which of the following hemoglobins migrates in the same position as HbA2 on hemoglobin electrophoresis at alkaline pH?

Accepted Answer

A)  HbC 
B)  HbO Arab 
C)  HbE 
D)  All of the above 
E)  None of the above 
A)  HbC 
B)  HbO Arab 
C)  HbE 
D)  All of the above 
E)  None of the above

Question 3

Which of the following hemoglobins migrates to the same position as HbS on hemoglobin electrophoresis at alkaline pH?

Accepted Answer

A)  HbD 
B)  HbC 
C)  HbG 
D)  A and C 
E)  B and C 
A)  HbD 
B)  HbC 
C)  HbG 
D)  A and C 
E)  B and C

Question 4

Which of the following classic hematologic features of sickle cell anemia can be observed on the peripheral blood smear?

Accepted Answer

A)  Target cells 
B)  Nucleated red cells 
C)  Sickle cells 
D)  Anisocytosis and poikilocytosis 
E)  All of the above 
A)  Target cells 
B)  Nucleated red cells 
C)  Sickle cells 
D)  Anisocytosis and poikilocytosis 
E)  All of the above

Question 5

Which of the following should patients be promptly treated with for acquired methemoglobinemia?

Accepted Answer

A)  Intravenous methylene blue (a strong reducing substance) 
B)  Oxygen administration 
C)  Exchange transfusion 
D)  All of the above 
E)  None of the above 
A)  Intravenous methylene blue (a strong reducing substance) 
B)  Oxygen administration 
C)  Exchange transfusion 
D)  All of the above 
E)  None of the above

Question 6

Which of the following is characteristic of an "aplastic" type of sickle cell crisis?

Accepted Answer

A)  Temporary bone marrow aplasia 
B)  Usually associated with infections 
C)  Associated with a low reticulocytic count 
D)  All of the above 
E)  None of the above 
A)  Temporary bone marrow aplasia 
B)  Usually associated with infections 
C)  Associated with a low reticulocytic count 
D)  All of the above 
E)  None of the above

Question 7

Which of the following represents quantitative hemoglobinopathies characterized by a decreased production of hemoglobin with a decreased synthesis of one particular globin chain?

Accepted Answer

A)  Sickle cell anemia 
B)  Hemoglobin C disease 
C)  Thalassemia 
D)  All of the above 
E)  None of the above 
A)  Sickle cell anemia 
B)  Hemoglobin C disease 
C)  Thalassemia 
D)  All of the above 
E)  None of the above

Question 8

Which process listed below is commonly responsible for the symptoms of the hand-foot syndrome observed in sickle cell anemia?

Accepted Answer

A)  Infarction 
B)  Infection 
C)  Bone marrow hyperplasia 
D)  Autosplenectomy 
E)  Ulceration 
A)  Infarction 
B)  Infection 
C)  Bone marrow hyperplasia 
D)  Autosplenectomy 
E)  Ulceration

Question 9

Which of the following may be associated with inherited decreased affinity hemoglobins?

Accepted Answer

A)  Mild anemia 
B)  Cyanosis 
C)  Shift to the right of the oxygen-dissociation curve (high P50 levels) 
D)  All of the above 
E)  None of the above 
A)  Mild anemia 
B)  Cyanosis 
C)  Shift to the right of the oxygen-dissociation curve (high P50 levels) 
D)  All of the above 
E)  None of the above

Question 10

The majority of hemoglobinopathies result from abnormalities in what chain?

Accepted Answer

A)  Alpha 
B)  Beta 
C)  Gamma 
D)  Delta 
E)  Epsilon 
A)  Alpha 
B)  Beta 
C)  Gamma 
D)  Delta 
E)  Epsilon

Question 11

Which of the following red cell inclusions may be seen in the RBCs of patients with sickle cell anemia as a result of rapid RBC turnover and "stressed" erythropoiesis?

Accepted Answer

A)  Siderotic granules 
B)  Cabot rings 
C)  Howell-Jolly bodies 
D)  A and B 
E)  A and C 
A)  Siderotic granules 
B)  Cabot rings 
C)  Howell-Jolly bodies 
D)  A and B 
E)  A and C

Question 12

Which of the following sickle cell crises is the hallmark of sickle cell anemia?

Accepted Answer

A)  Hemolytic 
B)  Aplastic 
C)  Painful or vaso-occlusive 
D)  Any of the above 
E)  None of the above 
A)  Hemolytic 
B)  Aplastic 
C)  Painful or vaso-occlusive 
D)  Any of the above 
E)  None of the above

Question 13

Which of the following therapies represents the "mainstay" of treatment for painful crises to correct fluid and electrolyte deficits in patients with sickle cell anemia?

Accepted Answer

A)  Aspirin 
B)  Hydration (administration of fluid volume) 
C)  Antibiotics 
D)  Transfusion therapy 
E)  Plasmapheresis 
A)  Aspirin 
B)  Hydration (administration of fluid volume) 
C)  Antibiotics 
D)  Transfusion therapy 
E)  Plasmapheresis

Question 14

The gene for hemoglobin S occurs with greatest frequency in which country?

Accepted Answer

A)  United States 
B)  Europe 
C)  Central Africa 
D)  Asia 
E)  Australia 
A)  United States 
B)  Europe 
C)  Central Africa 
D)  Asia 
E)  Australia

Question 15

Which of the following is characteristic of inherited high-affinity hemoglobins?

Accepted Answer

A)  Shift to the left of oxygen dissociation curve (low P50 levels) 
B)  Erythrocytosis and polycythemia 
C)  Increase in hemoglobin, hematocrit, and RBC count 
D)  All of the above 
E)  None of the above 
A)  Shift to the left of oxygen dissociation curve (low P50 levels) 
B)  Erythrocytosis and polycythemia 
C)  Increase in hemoglobin, hematocrit, and RBC count 
D)  All of the above 
E)  None of the above

Question 16

At the molecular level, which is the most common cause of a hemoglobinopathy?

Accepted Answer

A)  A single-base DNA substitution in the corresponding triplet codon 
B)  Multiple-base substitutions in the triplet codon 
C)  The production of long or short subunits 
D)  The occurrence of fusion subunits 
E)  All of the above 
A)  A single-base DNA substitution in the corresponding triplet codon 
B)  Multiple-base substitutions in the triplet codon 
C)  The production of long or short subunits 
D)  The occurrence of fusion subunits 
E)  All of the above

Question 17

Which of the following is characteristic of HbC disease?

Accepted Answer

A)  Mild chronic hemolytic anemia 
B)  Splenomegaly 
C)  Normocytic, normochromic anemia with numerous target cells (50-90%) and occasional folded cells 
D)  Hemoglobin C ("bar of gold") crystals present on peripheral smear of splenectomized patients 
E)  All of the above 
A)  Mild chronic hemolytic anemia 
B)  Splenomegaly 
C)  Normocytic, normochromic anemia with numerous target cells (50-90%) and occasional folded cells 
D)  Hemoglobin C ("bar of gold") crystals present on peripheral smear of splenectomized patients 
E)  All of the above

Question 18

Which of the following laboratory findings may be characteristic of sickle cell anemia?

Accepted Answer

A)  An average reticulocyte count between 5% and 20% 
B)  Neutrophilic leukocytosis with a shift to the left 
C)  Thrombocytosis 
D)  Marked erythroid hyperplasia 
E)  All of the above 
A)  An average reticulocyte count between 5% and 20% 
B)  Neutrophilic leukocytosis with a shift to the left 
C)  Thrombocytosis 
D)  Marked erythroid hyperplasia 
E)  All of the above

Question 19

Increases in which of the following hemoglobins can occur in adult patients with sickle cell anemia, thereby improving the anemia by modifying the degree and severity of the sickling?

Accepted Answer

A)  HbA2 
B)  Hb Gower 
C)  Hb Portland 
D)  HbF 
E)  HbH 
A)  HbA2 
B)  Hb Gower 
C)  Hb Portland 
D)  HbF 
E)  HbH

Question 20

Which of the following hemoglobin(s) is/are present in hemoglobin SC disease?

Accepted Answer

A)  HbS 
B)  HbA 
C)  HbC 
D)  A and C 
E)  All of the above 
A)  HbS 
B)  HbA 
C)  HbC 
D)  A and C 
E)  All of the above

Exam 11: Hemolytic Anemias: Intracorpuscular Defects: III the Hemoglobinopathies

What common renal complication is associated with sickle cell anemia?

Which of the following hemoglobins migrates in the same position as HbA2 on hemoglobin electrophoresis at alkaline pH?

Which of the following hemoglobins migrates to the same position as HbS on hemoglobin electrophoresis at alkaline pH?

Which of the following classic hematologic features of sickle cell anemia can be observed on the peripheral blood smear?

Which of the following should patients be promptly treated with for acquired methemoglobinemia?

Which of the following is characteristic of an "aplastic" type of sickle cell crisis?

Which of the following represents quantitative hemoglobinopathies characterized by a decreased production of hemoglobin with a decreased synthesis of one particular globin chain?

Which process listed below is commonly responsible for the symptoms of the hand-foot syndrome observed in sickle cell anemia?

Which of the following may be associated with inherited decreased affinity hemoglobins?

The majority of hemoglobinopathies result from abnormalities in what chain?

Which of the following red cell inclusions may be seen in the RBCs of patients with sickle cell anemia as a result of rapid RBC turnover and "stressed" erythropoiesis?

Which of the following sickle cell crises is the hallmark of sickle cell anemia?

Which of the following therapies represents the "mainstay" of treatment for painful crises to correct fluid and electrolyte deficits in patients with sickle cell anemia?

The gene for hemoglobin S occurs with greatest frequency in which country?

Which of the following is characteristic of inherited high-affinity hemoglobins?

At the molecular level, which is the most common cause of a hemoglobinopathy?

Which of the following is characteristic of HbC disease?

Which of the following laboratory findings may be characteristic of sickle cell anemia?

Increases in which of the following hemoglobins can occur in adult patients with sickle cell anemia, thereby improving the anemia by modifying the degree and severity of the sickling?

Which of the following hemoglobin(s) is/are present in hemoglobin SC disease?

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: Iv Thalassemia

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

Which of the following hemoglobins migrates in the same position as HbA₂ on hemoglobin electrophoresis at alkaline pH?