Question 1

What is the typical clinical course for those with G6PD A-?

Accepted Answer

A)  Severe anemia from infancy onward 
B)  Moderate anemia throughout adulthood 
C)  No anemia until exposure to an oxidant drug causes acute hemolysis 
D)  Benign because it does not cause anemia 
A)  Severe anemia from infancy onward 
B)  Moderate anemia throughout adulthood 
C)  No anemia until exposure to an oxidant drug causes acute hemolysis 
D)  Benign because it does not cause anemia

Question 2

A patient in the hospital for a hemolytic crisis after an infection has Heinz bodies when his blood is incubated with crystal violet. The fluorescent enzyme-screening test for G6PD is normal. Select the best course of action.

Accepted Answer

A)  Repeat the red cell examination with new methylene blue stain. 
B)  Repeat the enzyme test several weeks after the hemolytic crisis. 
C)  Perform an osmotic fragility test. 
D)  Perform a haptoglobin test. 
A)  Repeat the red cell examination with new methylene blue stain. 
B)  Repeat the enzyme test several weeks after the hemolytic crisis. 
C)  Perform an osmotic fragility test. 
D)  Perform a haptoglobin test.

Question 3

The most common inherited anemia found in people of Northern European extraction is hereditary:

Accepted Answer

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

Question 4

Which of the following is a typical finding in paroxysmal nocturnal hemoglobinuria?

Accepted Answer

A)  Hemosiderin in urine sediment 
B)  Normal levels of decay accelerating factor (CD55) 
C)  Hemolysis when blood is alkaline 
D)  Elevated white cells and platelets 
A)  Hemosiderin in urine sediment 
B)  Normal levels of decay accelerating factor (CD55) 
C)  Hemolysis when blood is alkaline 
D)  Elevated white cells and platelets

Question 5

What is the best test to confirm the diagnosis of PK?

Accepted Answer

A)  PK enzyme assay 
B)  Reticulocyte count 
C)  Haptoglobin test 
D)  Urine hemosiderin test 
A)  PK enzyme assay 
B)  Reticulocyte count 
C)  Haptoglobin test 
D)  Urine hemosiderin test

Question 6

Which of the following tests can confirm a paroxysmal nocturnal hemoglobinuria diagnosis in a patient?

Accepted Answer

A)  Sucrose hemolysis test (sugar water test) 
B)  Reticulocyte count 
C)  Ham test (acidified serum lysis test) 
D)  Flow cytometry for cell surface markers 
A)  Sucrose hemolysis test (sugar water test) 
B)  Reticulocyte count 
C)  Ham test (acidified serum lysis test) 
D)  Flow cytometry for cell surface markers

Question 7

What red cell morphology is often seen in patients with uremia?

Accepted Answer

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

Question 8

What causes the destruction of red cells in hereditary spherocytosis?

Accepted Answer

A)  Complement activation 
B)  Bound autoantibodies 
C)  Phagocytosis by peripheral monocytes 
D)  Phagocytosis by splenic macrophages 
A)  Complement activation 
B)  Bound autoantibodies 
C)  Phagocytosis by peripheral monocytes 
D)  Phagocytosis by splenic macrophages

Question 9

Hereditary spherocytosis is the result of a defect in:

Accepted Answer

A)  Hemoglobin structure 
B)  Red cell membrane lipid bilayer 
C)  Red cell membrane skeletal proteins 
D)  Rate of synthesis of globin chains 
A)  Hemoglobin structure 
B)  Red cell membrane lipid bilayer 
C)  Red cell membrane skeletal proteins 
D)  Rate of synthesis of globin chains

Exam 23: Intrinsic Defects Leading to Increased Erythrocyte Destruction

What is the typical clinical course for those with G6PD A-?

A patient in the hospital for a hemolytic crisis after an infection has Heinz bodies when his blood is incubated with crystal violet. The fluorescent enzyme-screening test for G6PD is normal. Select the best course of action.

The most common inherited anemia found in people of Northern European extraction is hereditary:

Which of the following is a typical finding in paroxysmal nocturnal hemoglobinuria?

What is the best test to confirm the diagnosis of PK?

Which of the following tests can confirm a paroxysmal nocturnal hemoglobinuria diagnosis in a patient?

What red cell morphology is often seen in patients with uremia?

What causes the destruction of red cells in hereditary spherocytosis?

Hereditary spherocytosis is the result of a defect in:

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 is the typical clinical course for those with G6PD A^-?