Question 1

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 A

Question 2

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. A

Question 3

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. C

Question 4

Which of the following disorders describes red cells that acquire a reduced surface area from losing unsupported membrane when defective proteins disrupt vertical interactions between transmembrane proteins and the cytoskeleton?

Accepted Answer

A) spherocytosis 
B) stomatocytosis 
C) pyropoikilocytosis 
D) acanthocytosis 
A) spherocytosis 
B) stomatocytosis 
C) pyropoikilocytosis 
D) acanthocytosis

Question 5

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 6

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 7

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 8

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 9

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 10

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 11

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 12

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 13

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 14

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 15

A patient with a previously compensated hereditary spherocytosis has a severe drop in hemoglobin and reticulocyte count 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

Question 16

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)

Question 17

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 18

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

Accepted Answer

A) elliptocytosis. 
B) stomatocytosis. 
C) spherocytosis. 
D) pyropoikilocytosis. 
A) elliptocytosis. 
B) stomatocytosis. 
C) spherocytosis. 
D) pyropoikilocytosis.

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

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

Exam 24: Intrinsic Defects Leading to Increased Erythrocyte Destruction

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

In most patients with hereditary spherocytosis,the condition is

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

Which of the following disorders describes red cells that acquire a reduced surface area from losing unsupported membrane when defective proteins disrupt vertical interactions between transmembrane proteins and the cytoskeleton?

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

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

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

Paroxysmal nocturnal hemoglobinuria is

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

What destroys the red cells in paroxysmal nocturnal hemoglobinuria?

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

Which of the following is normal in hereditary spherocytosis?

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

What causes the destruction of red cells in hereditary spherocytosis?

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

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

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

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

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

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

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Blood Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Erythrocyte Metabolism and Membrane Structure and Function

Hemoglobin Metabolism

Iron Kinetics and Laboratory Assessment

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Manual, Semiautomated and Point-of-Care Testing in Hematology

Automated Blood Cell Analysis

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

Bone Marrow Examination

Body Fluid Analysis in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron Kinetics and Heme Metabolism

Anemias Caused by Defects of DNA Metabolism

Bone Marrow Failure

Introduction to Increased Destruction of Erythrocytes

Extrinsic Defects Leading to Increased Erythrocyte Destruction Nonimmune Causes

Extrinsic Defects Leading to Increased Erythrocyte Destruction Immune Causes

Hemoglobinopathies (Structural Defects in Hemoglobin)

Thalassemias

Nonmalignant Leukocyte Disorders

Cytogenetics

Molecular Diagnostics in Hematopathology

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Normal Hemostasis and Coagulation

Hemorrhagic Disorders and Laboratory Assessment

Thrombotic Disorders and Laboratory Assessment

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Antithrombotic Therapies and their Laboratory Assessment

Hemostasis and Coagulation Instrumentation

Pediatric and Geriatric Hematology and Hemostasis

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