Question 1

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

Accepted Answer

The somatic mutation of the PI

Question 2

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 3

The function of the MIRL is to:

Accepted Answer

A)  Induce red cell agglutination 
B)  Prevent activation of C3b converting enzyme 
C)  Interfere with C8/C9 activation 
D)  All of the above 
A)  Induce red cell agglutination 
B)  Prevent activation of C3b converting enzyme 
C)  Interfere with C8/C9 activation 
D)  All of the above

Question 4

A sample has the following test results: hemoglobin 9 g/dL, autohemolysis increased after 48 hours, and increased hemolysis observed with the Ham test and sucrose hemolysis test. Based on these findings, what is the patient most likely suffering from?

Accepted Answer

A)  HS 
B)  HE 
C)  PNH 
D)  PCH 
A)  HS 
B)  HE 
C)  PNH 
D)  PCH

Question 5

PNH is currently confirmed in the lab by which of the following?

Accepted Answer

A)  Immunophenotyping 
B)  Ham and sucrose lysis tests 
C)  Donath-Landsteiner test 
D)  Osmotic fragility 
A)  Immunophenotyping 
B)  Ham and sucrose lysis tests 
C)  Donath-Landsteiner test 
D)  Osmotic fragility

Question 6

Hereditary stomatocytosis is a condition resulting from defects in:

Accepted Answer

A)  Cation permeability 
B)  Spectrin 
C)  Protein 4.1 
D)  Spectrin dimer-dimer association 
A)  Cation permeability 
B)  Spectrin 
C)  Protein 4.1 
D)  Spectrin dimer-dimer association

Question 7

Which of the following disorders is caused by abnormal permeability of the RBC membrane?

Accepted Answer

A)  Hereditary elliptocytosis 
B)  Hereditary spherocytosis 
C)  PNH 
D)  Hereditary dehydrated stomatocytosis 
A)  Hereditary elliptocytosis 
B)  Hereditary spherocytosis 
C)  PNH 
D)  Hereditary dehydrated stomatocytosis

Question 8

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 9

Hereditary pyropoikilocytosis differs in red cell morphology from other erythrocyte membrane defects by what characteristic cell shape?

Accepted Answer

A)  Spherocyte 
B)  Elliptocyte 
C)  Schistocyte 
D)  Target cell 
A)  Spherocyte 
B)  Elliptocyte 
C)  Schistocyte 
D)  Target cell

Question 10

A defect in ankyrin is described as:

Accepted Answer

A)  A vertical interaction defect 
B)  A horizontal interaction defect 
C)  Both vertical and horizontal interaction defects 
D)  Cannot be determined 
A)  A vertical interaction defect 
B)  A horizontal interaction defect 
C)  Both vertical and horizontal interaction defects 
D)  Cannot be determined

Question 11

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 12

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 13

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 14

Explain why patients with HS sometimes suffer from gallstones.

Accepted Answer

Spherocytes in peripheral circ

Question 15

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 16

Explain why patients with HS typically have an MCHC >36%.

Accepted Answer

Spherocytes have decreased sur

Exam 17: Hemolytic Anemia: Membrane Defects

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

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

The function of the MIRL is to:

A sample has the following test results: hemoglobin 9 g/dL, autohemolysis increased after 48 hours, and increased hemolysis observed with the Ham test and sucrose hemolysis test. Based on these findings, what is the patient most likely suffering from?

PNH is currently confirmed in the lab by which of the following?

Hereditary stomatocytosis is a condition resulting from defects in:

Which of the following disorders is caused by abnormal permeability of the RBC membrane?

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

Hereditary pyropoikilocytosis differs in red cell morphology from other erythrocyte membrane defects by what characteristic cell shape?

A defect in ankyrin is described as:

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

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?

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

Explain why patients with HS sometimes suffer from gallstones.

The SAO variant of HE is associated with:

Explain why patients with HS typically have an MCHC >36%.

Introduction

Cellular Homeostasis

Structure and Function of Hematopoietic Organs

Hematopoiesis

The Erythrocyte

Hemoglobin

Granulocytes and Monocytes

Lymphocytes

The Platelet

The Complete Blood Count and Peripheral Blood Smear Evaluation

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopathies: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Hypoproliferative Anemias

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Disorders of Leukocytes: Granulocytes and Monocytes

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Precursor Lymphoid Neoplasms

Mature Lymphoid Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hemostasis: Laboratory Testing and Instrumentation

Hematology Procedures

Bone Marrow Examination

Automation in Hematology

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Quality Assessment in the Hematology Laboratory

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