Question 1

Explain why patients with HS sometimes suffer from gallstones.

Accepted Answer

Spherocytes in peripheral circ

Question 2

The laboratory professional notes about 20% spherocytes on a peripheral blood smear of a 4-year-old boy.An osmotic fragility test is performed.The control shows initial hemolysis at 0.50% NaCl and complete hemolysis at 0.35% NaCl.The patient sample has initial hemolysis 60% NaCl and complete hemolysis at 0.45% NaCl.What does this indicate?

Accepted Answer

A) The patient is exhibiting increased osmotic fragility. 
B) The patient has AIHA 
C) The patient is not exhibiting decreased osmotic fragility. 
D) The control result is out of control so patient results are invalid. 
A) The patient is exhibiting increased osmotic fragility. 
B) The patient has AIHA 
C) The patient is not exhibiting decreased osmotic fragility. 
D) The control result is out of control so patient results are invalid.

Question 3

Which of the following sets of disorders is characterized by increased osmotic fragility?

Accepted Answer

A) HE and PNH 
B) HS and hereditary overhydrated stomatocytosis 
C) HS and hereditary dehydrated stomatocytosis 
D) HS and thalassemia 
A) HE and PNH 
B) HS and hereditary overhydrated stomatocytosis 
C) HS and hereditary dehydrated stomatocytosis 
D) HS and thalassemia

Question 4

The screening sucrose hemolysis (sugar-water) test is useful in identifying PNH cells by what mechanism?

Accepted Answer

A) Immunophenotyping 
B) Hemoglobin electrophoresis 
C) Erythrocyte survival test 
D) Complement lysis 
A) Immunophenotyping 
B) Hemoglobin electrophoresis 
C) Erythrocyte survival test 
D) Complement lysis

Question 5

In hereditary spherocytosis, the erythrocytes become trapped in the splenic cords and run out of ATP to pump out excessive ions.What is this ion?

Accepted Answer

A) K+ 
B) Chloride 
C) Na+ 
D) Oxygen 
A) K+ 
B) Chloride 
C) Na+ 
D) Oxygen

Question 6

Which of the following tests is used to screen for PNH?

Accepted Answer

A) Immunophenotyping 
B) Sugar-water test 
C) Acidified serum test 
D) All of the above 
A) Immunophenotyping 
B) Sugar-water test 
C) Acidified serum test 
D) All of the above

Question 7

The SAO variant of HE is associated with:

Accepted Answer

A) Defective pectrin tetramer formation. 
B) Defective protein 4.1. 
C) Defective band 3 protein and abnormally tight binding to ankyrin. 
D) All of the above. 
A) Defective pectrin tetramer formation. 
B) Defective protein 4.1. 
C) Defective band 3 protein and abnormally tight binding to ankyrin. 
D) All of the above.

Question 8

What is the principal confirmation test in the diagnosis of HS?

Accepted Answer

A) Erythrocyte indices 
B) Erythrocyte survival test 
C) Osmotic fragility test 
D) Hemoglobin electrophoresis 
A) Erythrocyte indices 
B) Erythrocyte survival test 
C) Osmotic fragility test 
D) Hemoglobin electrophoresis

Question 9

Which of the following statements best describes the function of DAF in the body?

Accepted Answer

A) Facilitates the amplification of the complement cascade by activating C3 convertase. 
B) Prevents the amplification of C3/C5 convertase activity. 
C) Facilitates complement binding on erythrocytes. 
D) Prevents apoptosis of erythrocyte precursors. 
A) Facilitates the amplification of the complement cascade by activating C3 convertase. 
B) Prevents the amplification of C3/C5 convertase activity. 
C) Facilitates complement binding on erythrocytes. 
D) Prevents apoptosis of erythrocyte precursors.

Question 10

Which of the following disorders is characterized by increased erythrocyte membrane sphingomyelin and decreased lecithin?

Accepted Answer

A) HS 
B) HE 
C) Acanthocytosis 
D) HPP 
A) HS 
B) HE 
C) Acanthocytosis 
D) HPP

Question 11

Explain how a somatic mutation in the PIGA gene leads to PNH.

Accepted Answer

The somatic mutation of the PI

Question 12

Which of the following subtypes of PNH is characterized by moderate hemolysis and intermediate GPI expression?

Accepted Answer

A) Type I 
B) Type II 
C) Type III 
D) It cannot be determined based on the information given. 
A) Type I 
B) Type II 
C) Type III 
D) It cannot be determined based on the information given.

Question 13

A 5-year-old boy is admitted to the pediatric unit with a fractured tibia.Routine blood work is ordered.His PB smear shows moderate spherocytosis.An osmotic fragility is also ordered, and the test result indicates an increased osmotic fragility.Based on these findings, what is the most likely defect?

Accepted Answer

A) Hereditary elliptocytosis 
B) Hereditary spherocytosis 
C) Microangiopathic hemolytic anemia due to trauma 
D) Cold autoimmune hemolytic anemia 
A) Hereditary elliptocytosis 
B) Hereditary spherocytosis 
C) Microangiopathic hemolytic anemia due to trauma 
D) Cold autoimmune hemolytic anemia

Question 14

Paroxysmal nocturnal hemoglobinuria has four basic disease mechanisms: hyperhemolysis, venous thrombosis, infection, and bone marrow hypoplasia.Which of these mechanisms is the most severe complication?

Accepted Answer

A) Hyperhemolysis 
B) Infection 
C) Venous thrombosis 
D) Bone marrow hypoplasia 
A) Hyperhemolysis 
B) Infection 
C) Venous thrombosis 
D) Bone marrow hypoplasia

Question 15

Immunophenotyping is performed on a patient suspected of having PNH.What monoclonal antibody specificity should be ordered?

Accepted Answer

A) CD33 and CD13 
B) CD56 and CD4 
C) CD3 and CD8 
D) CD55 and CD59 
A) CD33 and CD13 
B) CD56 and CD4 
C) CD3 and CD8 
D) CD55 and CD59

Question 16

Select the best statement from the choices below to describe the pathogenesis of PNH.

Accepted Answer

A) Increased hemolytic episodes are produced as a result of complement binding to erythrocytes via IgG antibodies in cold temperatures. 
B) Increased hemolytic episodes are produced as a result of missing cell membrane proteins that regulate complement activation. 
C) Increased hemolytic episodes are produced as a result of missing enzymes necessary for ATP production in erythrocytes. 
D) Increased hemolytic episodes are produced as a result of initiation of an immune response against erythrocytes. 
A) Increased hemolytic episodes are produced as a result of complement binding to erythrocytes via IgG antibodies in cold temperatures. 
B) Increased hemolytic episodes are produced as a result of missing cell membrane proteins that regulate complement activation. 
C) Increased hemolytic episodes are produced as a result of missing enzymes necessary for ATP production in erythrocytes. 
D) Increased hemolytic episodes are produced as a result of initiation of an immune response against erythrocytes.

Exam 14: Hemolytic Anemia: Membrane Defects

Explain why patients with HS sometimes suffer from gallstones.

Which of the following sets of disorders is characterized by increased osmotic fragility?

The screening sucrose hemolysis (sugar-water) test is useful in identifying PNH cells by what mechanism?

In hereditary spherocytosis, the erythrocytes become trapped in the splenic cords and run out of ATP to pump out excessive ions.What is this ion?

Which of the following tests is used to screen for PNH?

The SAO variant of HE is associated with:

What is the principal confirmation test in the diagnosis of HS?

Which of the following statements best describes the function of DAF in the body?

Which of the following disorders is characterized by increased erythrocyte membrane sphingomyelin and decreased lecithin?

Explain how a somatic mutation in the PIGA gene leads to PNH.

Which of the following subtypes of PNH is characterized by moderate hemolysis and intermediate GPI expression?

Paroxysmal nocturnal hemoglobinuria has four basic disease mechanisms: hyperhemolysis, venous thrombosis, infection, and bone marrow hypoplasia.Which of these mechanisms is the most severe complication?

Immunophenotyping is performed on a patient suspected of having PNH.What monoclonal antibody specificity should be ordered?

Select the best statement from the choices below to describe the pathogenesis of PNH.

Introduction

Cellular Homeostasis

Hematopoiesis

Structure and Function of Hematopoietic Organs

The Erythrocyte

Hemoglobin

The Leukocyte

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopaties: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Introduction to Hemolytic Anemia

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Granulocyte and Monocyte Disorders

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Disorders

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Acute Lymphoblastic Leukemias

Lymphoid Malignancies: Chronic Lymphoid Leukemias, Lymphomas, and Plasma Cell Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology Laboratory

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hematology Procedures and Hemostasis

Bone Marrow Examination

Automation in Hematology and Hemostasis

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Laboratory Testing in Coagulation

Quality Assessment in the Hematology Laboratory

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