Question 1

What is the most common enzyme deficiency of the glycolytic pathway?

Accepted Answer

A)  Aldolase 
B)  Enolase 
C)  Lactic dehydrogenase (LDH) 
D)  Pyruvate kinase (PK) 
A)  Aldolase 
B)  Enolase 
C)  Lactic dehydrogenase (LDH) 
D)  Pyruvate kinase (PK) D

Question 2

Red cells acquiring a reduced surface area from losing unsupported membrane because of a deficiency of skeletal proteins describes hereditary:

Accepted Answer

A)  Spherocytosis 
B)  Stomatocytosis 
C)  Pyropoikilocytosis 
D)  Acanthocytosis 
A)  Spherocytosis 
B)  Stomatocytosis 
C)  Pyropoikilocytosis 
D)  Acanthocytosis A

Question 3

A patient with a previously compensated hereditary spherocytosis has a severe drop in hemoglobin after a parvovirus infection. This represents what type of crisis?

Accepted Answer

A)  Megaloblastic 
B)  Hemolytic 
C)  Aplastic 
D)  Anemic 
A)  Megaloblastic 
B)  Hemolytic 
C)  Aplastic 
D)  Anemic C

Question 4

What red cell morphology is associated with abetalipoproteinemia, liver disease, and McLeod blood group?

Accepted Answer

A)  Acanthocytes 
B)  Stomatocytes 
C)  Spherocytes 
D)  Elliptocytes 
A)  Acanthocytes 
B)  Stomatocytes 
C)  Spherocytes 
D)  Elliptocytes

Question 5

What destroys the red cells in paroxysmal nocturnal hemoglobinuria?

Accepted Answer

A)  Macrophages in the bone marrow 
B)  Antibodies against red cell membrane antigens 
C)  Increased susceptibility to complement 
D)  Macrophages in the spleen 
A)  Macrophages in the bone marrow 
B)  Antibodies against red cell membrane antigens 
C)  Increased susceptibility to complement 
D)  Macrophages in the spleen

Question 6

What is the most common red cell morphologic finding in PK deficiency?

Accepted Answer

A)  Elliptocytes 
B)  Polychromasia and echinocytes (crenated) 
C)  Heinz bodies and spherocytes 
D)  Microcytes and hypochromasia 
A)  Elliptocytes 
B)  Polychromasia and echinocytes (crenated) 
C)  Heinz bodies and spherocytes 
D)  Microcytes and hypochromasia

Question 7

The inability to attach proteins requiring a glycosylphosphatidylinositol (GPI) link to the membrane surface is found in:

Accepted Answer

A)  Paroxysmal nocturnal hemoglobinuria 
B)  Hereditary spherocytosis 
C)  Paroxysmal cold hemoglobinuria 
D)  Hereditary elliptocytosis 
A)  Paroxysmal nocturnal hemoglobinuria 
B)  Hereditary spherocytosis 
C)  Paroxysmal cold hemoglobinuria 
D)  Hereditary elliptocytosis

Question 8

Which of the following is a true statement regarding G6PD?

Accepted Answer

A)  The gene is located on chromosome 22. 
B)  The gene has the greatest degree of variability in the human genome. 
C)  The deficiency is primarily expressed as a clinical disease in heterozygous female subjects. 
D)  The normal enzyme is called G6PD A. 
A)  The gene is located on chromosome 22. 
B)  The gene has the greatest degree of variability in the human genome. 
C)  The deficiency is primarily expressed as a clinical disease in heterozygous female subjects. 
D)  The normal enzyme is called G6PD A.

Question 9

What treatment is most common for hereditary elliptocytosis patients who are anemic and show signs of hemolysis?

Accepted Answer

A)  No treatment available 
B)  Drugs to prolong RBC life span 
C)  Splenectomy 
D)  Transfusions 
A)  No treatment available 
B)  Drugs to prolong RBC life span 
C)  Splenectomy 
D)  Transfusions

Question 10

What ethnic group most often has glucose-6-phosphate dehydrogenase (G6PD) A- in the United States?

Accepted Answer

A)  Northern European male subjects 
B)  Asian American female subjects 
C)  African American female subjects 
D)  African American male subjects 
A)  Northern European male subjects 
B)  Asian American female subjects 
C)  African American female subjects 
D)  African American male subjects

Question 11

Which of the following can distinguish immune hemolytic anemia from hereditary spherocytosis in a patient with spherocytes?

Accepted Answer

A)  Osmotic fragility 
B)  Reticulocyte count 
C)  Presence of polychromasia 
D)  Direct antiglobulin test 
A)  Osmotic fragility 
B)  Reticulocyte count 
C)  Presence of polychromasia 
D)  Direct antiglobulin test

Question 12

Cells with a slit (stomatocytes) instead of a round central pallor can be found in all of the following except:

Accepted Answer

A)  Liver disease of alcoholism 
B)  Rhnull disease 
C)  Hereditary elliptocytosis 
D)  Blood films with a drying artifact 
A)  Liver disease of alcoholism 
B)  Rhnull disease 
C)  Hereditary elliptocytosis 
D)  Blood films with a drying artifact

Question 13

What red cell morphology, using Wright stain, is seen in patients with G6PD deficiency during a hemolytic episode?

Accepted Answer

A)  Bite cells and polychromasia 
B)  Heinz bodies 
C)  Acanthocytes and crenated cells 
D)  Red cells are normal 
A)  Bite cells and polychromasia 
B)  Heinz bodies 
C)  Acanthocytes and crenated cells 
D)  Red cells are normal

Question 14

Which of the following is normal in hereditary spherocytosis?

Accepted Answer

A)  Red cell morphology 
B)  Direct antiglobulin test 
C)  Osmotic fragility 
D)  Reticulocyte count 
A)  Red cell morphology 
B)  Direct antiglobulin test 
C)  Osmotic fragility 
D)  Reticulocyte count

Question 15

Paroxysmal nocturnal hemoglobinuria is:

Accepted Answer

A)  Inherited as an X-linked recessive trait 
B)  Acquired as a clonal disorder 
C)  Inherited as an autosomal dominant disorder 
D)  Acquired after a viral infection 
A)  Inherited as an X-linked recessive trait 
B)  Acquired as a clonal disorder 
C)  Inherited as an autosomal dominant disorder 
D)  Acquired after a viral infection

Question 16

Why is G6PD important for normal red cell survival?

Accepted Answer

A)  Hemoglobin oxygen affinity is increased in its absence. 
B)  It is required to regenerate reduced glutathione. 
C)  It is required for insertion of iron into the protoporphyrin ring to form heme. 
D)  ($\alpha$) Chains are produced in excess in its absence. 
A)  Hemoglobin oxygen affinity is increased in its absence. 
B)  It is required to regenerate reduced glutathione. 
C)  It is required for insertion of iron into the protoporphyrin ring to form heme. 
D)  ($\alpha$) Chains are produced in excess in its absence.

Question 17

In most patients with hereditary spherocytosis, the condition is:

Accepted Answer

A)  Inherited as an autosomal dominant trait 
B)  Inherited as an autosomal recessive trait 
C)  Inherited as an X-linked recessive trait 
D)  Acquired after viral infection 
A)  Inherited as an autosomal dominant trait 
B)  Inherited as an autosomal recessive trait 
C)  Inherited as an X-linked recessive trait 
D)  Acquired after viral infection

Question 18

All of the following would be expected 1 year after a splenectomy on a patient with severe hereditary spherocytosis except:

Accepted Answer

A)  Spherocytes on the blood film 
B)  Howell-Jolly bodies 
C)  Reticulocyte count of 10% 
D)  Siderocytes 
A)  Spherocytes on the blood film 
B)  Howell-Jolly bodies 
C)  Reticulocyte count of 10% 
D)  Siderocytes

Question 19

Which of the following is a typical finding in hereditary spherocytosis?

Accepted Answer

A)  Increased mean cell hemoglobin concentration (MCH
C)  
B)  Normal reticulocytes 
C)  Reduced fragility of red cells 
D)  Normal spectrin 
A)  Increased mean cell hemoglobin concentration (MCH
C)  
B)  Normal reticulocytes 
C)  Reduced fragility of red cells 
D)  Normal spectrin

Question 20

A patient with a small number of spherocytes, increased reticulocytes, and increased MCHC has a normal osmotic fragility. Select the best course of action.

Accepted Answer

A)  Dismiss the spherocytes as an artifact. 
B)  Repeat the osmotic fragility after incubating the blood at 37 $\degree$ C for 24 hours. 
C)  Check the cell counter for errors in the MCHC. 
D)  Collect a new blood sample. 
A)  Dismiss the spherocytes as an artifact. 
B)  Repeat the osmotic fragility after incubating the blood at 37 $\degree$ C for 24 hours. 
C)  Check the cell counter for errors in the MCHC. 
D)  Collect a new blood sample.

Exam 23: Intrinsic Defects Leading to Increased Erythrocyte Destruction

What is the most common enzyme deficiency of the glycolytic pathway?

Red cells acquiring a reduced surface area from losing unsupported membrane because of a deficiency of skeletal proteins describes hereditary:

A patient with a previously compensated hereditary spherocytosis has a severe drop in hemoglobin after a parvovirus infection. This represents what type of crisis?

What red cell morphology is associated with abetalipoproteinemia, liver disease, and McLeod blood group?

What destroys the red cells in paroxysmal nocturnal hemoglobinuria?

What is the most common red cell morphologic finding in PK deficiency?

The inability to attach proteins requiring a glycosylphosphatidylinositol (GPI) link to the membrane surface is found in:

Which of the following is a true statement regarding G6PD?

What treatment is most common for hereditary elliptocytosis patients who are anemic and show signs of hemolysis?

What ethnic group most often has glucose-6-phosphate dehydrogenase (G6PD) A- in the United States?

Which of the following can distinguish immune hemolytic anemia from hereditary spherocytosis in a patient with spherocytes?

Cells with a slit (stomatocytes) instead of a round central pallor can be found in all of the following except:

What red cell morphology, using Wright stain, is seen in patients with G6PD deficiency during a hemolytic episode?

Which of the following is normal in hereditary spherocytosis?

Paroxysmal nocturnal hemoglobinuria is:

Why is G6PD important for normal red cell survival?

In most patients with hereditary spherocytosis, the condition is:

All of the following would be expected 1 year after a splenectomy on a patient with severe hereditary spherocytosis except:

Which of the following is a typical finding in hereditary spherocytosis?

A patient with a small number of spherocytes, increased reticulocytes, and increased MCHC has a normal osmotic fragility. Select the best course of action.

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Energy Metabolism and Membrane Physiology of the Erythrocyte

Hemoglobin Metabolism

Iron Metabolism

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Routine and Point-Of-Care Testing in Hematology: Manual and Semiautomated Methods

Examination of the Peripheral Blood Film and Correlation With the Complete Blood Count

Bone Marrow Examination

Body Fluids in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron and Heme Metabolism

Anemias Caused by Defects of Dna Metabolism

Bone Marrow Failure

Introduction to Increased Destruction of Erythrocytes

Extrinsic Defects Leading to Increased Erythrocytedestructionnonimmune Causes

Extrinsic Defects Leading to Increased Erythrocytedestructionimmune Causes

Hemoglobinopathies Structural Defects in Hemoglobin

Thalassemias

Nonmalignant Leukocyte Disorders

Introduction to Leukocyte Neoplasms

Cytochemistry

Cytogenetics

Molecular Diagnostics in the Clinical Laboratory

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Pediatric and Geriatric Hematology

Automated Cell-Counting Instrumentation

Normal Hemostasis and Coagulation

Hemorrhagic Coagulation Disorders

Thrombosis Risk Testing

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Monitoring Antithrombotic Therapies

Coagulation Instrumentation

Filters

What ethnic group most often has glucose-6-phosphate dehydrogenase (G6PD) A^- in the United States?