Question 1

The major components of the red cell membrane skeleton are:

Accepted Answer

A)  Spectrin 
B)  Actin 
C)  Protein 4.1 
D)  All of the above 
E)  None of the above 
A)  Spectrin 
B)  Actin 
C)  Protein 4.1 
D)  All of the above 
E)  None of the above D

Question 2

Which of the following represents the pattern of inheritance of hereditary pyropoikilocytosis (HPP)?

Accepted Answer

A)  Autosomal recessive 
B)  Autosomal dominant 
C)  X-linked 
D)  Any of the above 
E)  Acquired, not inherited 
A)  Autosomal recessive 
B)  Autosomal dominant 
C)  X-linked 
D)  Any of the above 
E)  Acquired, not inherited A

Question 3

Which of the following are classic clinical manifestations of hereditary spherocytosis (HS)?

Accepted Answer

A)  Jaundice 
B)  Anemia 
C)  Enlarged spleen 
D)  Mild uncompensated hemolytic anemia 
E)  All of the above 
A)  Jaundice 
B)  Anemia 
C)  Enlarged spleen 
D)  Mild uncompensated hemolytic anemia 
E)  All of the above E

Question 4

The morphologic hallmark of HS hereditary spherocytosis on the peripheral blood smear is:

Accepted Answer

A)  Polychromasia 
B)  Ovalocytes 
C)  Spherocytes 
D)  Poikilocytes 
E)  Sickle cells 
A)  Polychromasia 
B)  Ovalocytes 
C)  Spherocytes 
D)  Poikilocytes 
E)  Sickle cells

Question 5

What does the osmotic fragility test measure?

Accepted Answer

A)  Surface-to-volume ratio of the RBC 
B)  Structural and metabolic integrity of RBC membrane 
C)  Amount of RBC membrane spectrin 
D)  RBC metabolic pathways 
E)  None of the above 
A)  Surface-to-volume ratio of the RBC 
B)  Structural and metabolic integrity of RBC membrane 
C)  Amount of RBC membrane spectrin 
D)  RBC metabolic pathways 
E)  None of the above

Question 6

Which of the following characterizes hereditary spherocytosis (HS)?

Accepted Answer

A)  Decreased surface-to-volume ratio 
B)  Increased MCHC 
C)  Hemolytic anemia 
D)  Spherocytes on the peripheral smear 
E)  All of the above 
A)  Decreased surface-to-volume ratio 
B)  Increased MCHC 
C)  Hemolytic anemia 
D)  Spherocytes on the peripheral smear 
E)  All of the above

Question 7

Which of the following may be found on the peripheral smear of a patient with HS (hereditary spherocytosis) prior to splenectomy?

Accepted Answer

A)  Spherocytes 
B)  Polychromasia 
C)  Poikilocytes 
D)  Anisocytosis 
E)  All of the above 
A)  Spherocytes 
B)  Polychromasia 
C)  Poikilocytes 
D)  Anisocytosis 
E)  All of the above

Question 8

Which of the following is the most common treatment for most patients with HS?

Accepted Answer

A)  Exchange transfusion 
B)  Chemotherapy 
C)  Splenectomy 
D)  No treatment 
E)  None of the above 
A)  Exchange transfusion 
B)  Chemotherapy 
C)  Splenectomy 
D)  No treatment 
E)  None of the above

Question 9

Which of the following laboratory results is not characteristic of HPP?

Accepted Answer

A)  Low MCV 
B)  Increase in osmotic fragility 
C)  Increase in autohemolysis test 
D)  RBC thermal instability at 45°C leading to fragmentation 
E)  Normal MCV 
A)  Low MCV 
B)  Increase in osmotic fragility 
C)  Increase in autohemolysis test 
D)  RBC thermal instability at 45°C leading to fragmentation 
E)  Normal MCV

Question 10

Which of the following represent the major clinical and morphologic syndromes of HE?

Accepted Answer

A)  Common HE (minimal or no hemolysis) 
B)  Spherocytic HE (hybrid of mild HE and HS) 
C)  Stomatocytic HE (common only in Melanesians) 
D)  All of the above 
E)  None of the above 
A)  Common HE (minimal or no hemolysis) 
B)  Spherocytic HE (hybrid of mild HE and HS) 
C)  Stomatocytic HE (common only in Melanesians) 
D)  All of the above 
E)  None of the above

Question 11

Which of the following represents hereditary intracorpuscular defects?

Accepted Answer

A)  Red blood cell (RB
C)  membrane defects 
B)  Enzyme deficiencies 
C)  Hemoglobinopathies 
D)  Thalassemia 
E)  All of the above 
A)  Red blood cell (RB
C)  membrane defects 
B)  Enzyme deficiencies 
C)  Hemoglobinopathies 
D)  Thalassemia 
E)  All of the above

Question 12

The majority of hereditary RBC membrane defects are inherited by what pattern?

Accepted Answer

A)  Autosomal dominant 
B)  Autosomal recessive 
C)  Sex-linked 
D)  Codominant 
E)  None of the above 
A)  Autosomal dominant 
B)  Autosomal recessive 
C)  Sex-linked 
D)  Codominant 
E)  None of the above

Question 13

Which of the following represent extracorpuscular defects?

Accepted Answer

A)  Immune hemolytic anemias 
B)  Infections 
C)  Microangiopathic hemolytic anemia 
D)  Hypersplenism 
E)  All of the above 
A)  Immune hemolytic anemias 
B)  Infections 
C)  Microangiopathic hemolytic anemia 
D)  Hypersplenism 
E)  All of the above

Question 14

An RPI of greater than __________ is generally regarded as indicative of a hemolytic state.

Accepted Answer

A)  2.5-3.0 
B)  1-1.5 
C)  0-1 
D)  4-5 
E)  5-6 
A)  2.5-3.0 
B)  1-1.5 
C)  0-1 
D)  4-5 
E)  5-6

Question 15

What does the autohemolysis test measure?

Accepted Answer

A)  Surface-to-volume ratio of the RBC 
B)  Structural and metabolic integrity of RBC membrane 
C)  Amount of RBC membrane spectrin 
D)  RBC metabolic pathways 
E)  None of the above 
A)  Surface-to-volume ratio of the RBC 
B)  Structural and metabolic integrity of RBC membrane 
C)  Amount of RBC membrane spectrin 
D)  RBC metabolic pathways 
E)  None of the above

Question 16

Which of the following membrane defects is the most common hereditary hemolytic anemia in Caucasians?

Accepted Answer

A)  Hereditary elliptocytosis 
B)  Hereditary spherocytosis 
C)  Thalassemia 
D)  Hereditary pyropoikilocytosis 
E)  None of the above 
A)  Hereditary elliptocytosis 
B)  Hereditary spherocytosis 
C)  Thalassemia 
D)  Hereditary pyropoikilocytosis 
E)  None of the above

Question 17

Which of the following RPI results will suggest an inadequate response of erythropoiesis by bone marrow in response to anemia?

Accepted Answer

A) >3.0 B) 2.0-3.0 C) <2.0 D) 2.5 E) None of the above A) >3.0 B) 2.0-3.0 C) <2.0 D) 2.5 E) None of the above

Question 18

Which of the following is not characteristic of hereditary hydrocytosis (stomatocytosis)?

Accepted Answer

A)  Inherited autosomal dominant 
B)  Presence of stomatocytes on the peripheral smear 
C)  Increased passive permeability of sodium into red cell and potassium out of red cell 
D)  Increased MCV and decreased MCHC 
E)  Decreased MCV and increased MCHC 
A)  Inherited autosomal dominant 
B)  Presence of stomatocytes on the peripheral smear 
C)  Increased passive permeability of sodium into red cell and potassium out of red cell 
D)  Increased MCV and decreased MCHC 
E)  Decreased MCV and increased MCHC

Question 19

Which of the following laboratory results are characteristic of HS?

Accepted Answer

A)  Increased MCHC 
B)  Abnormal osmotic fragility test 
C)  Abnormal autohemolysis test 
D)  Normal MCV (mean corpuscular volume) and MCH (mean corpuscular hemoglobin) 
E)  All of the above 
A)  Increased MCHC 
B)  Abnormal osmotic fragility test 
C)  Abnormal autohemolysis test 
D)  Normal MCV (mean corpuscular volume) and MCH (mean corpuscular hemoglobin) 
E)  All of the above

Question 20

A 40-year-old patient was admitted to the hospital with acute cholecystitis and a palpable spleen. A CBC was performed: Hemoglobin, 13.0 g/dL; MCV, 80 fL; Hct, 38%; MCH, 28 pg; Retic, 7%; MCHC, 36%; RPI, 3.9; Coombs' test negative; spherocytes, 2+; anisocytosis, 2+; poikilocytosis, 2+. Which of the following disorders is consistent with the laboratory profile?

Accepted Answer

A)  Autoimmune hemolytic anemia 
B)  Thalassemia 
C)  Hereditary elliptocytosis (H
E)  
D)  Hereditary spherocytosis (HS) 
E)  Hereditary pyropoikilocytosis (HPP) 
A)  Autoimmune hemolytic anemia 
B)  Thalassemia 
C)  Hereditary elliptocytosis (H
E)  
D)  Hereditary spherocytosis (HS) 
E)  Hereditary pyropoikilocytosis (HPP)

Exam 9: Introduction to Hemolytic Anemias: Intracorpuscular Defects: I Hereditary Defects of the Red Cell Membrane

The major components of the red cell membrane skeleton are:

Which of the following represents the pattern of inheritance of hereditary pyropoikilocytosis (HPP)?

Which of the following are classic clinical manifestations of hereditary spherocytosis (HS)?

The morphologic hallmark of HS hereditary spherocytosis on the peripheral blood smear is:

What does the osmotic fragility test measure?

Which of the following characterizes hereditary spherocytosis (HS)?

Which of the following may be found on the peripheral smear of a patient with HS (hereditary spherocytosis) prior to splenectomy?

Which of the following is the most common treatment for most patients with HS?

Which of the following laboratory results is not characteristic of HPP?

Which of the following represent the major clinical and morphologic syndromes of HE?

Which of the following represents hereditary intracorpuscular defects?

The majority of hereditary RBC membrane defects are inherited by what pattern?

Which of the following represent extracorpuscular defects?

An RPI of greater than __________ is generally regarded as indicative of a hemolytic state.

What does the autohemolysis test measure?

Which of the following membrane defects is the most common hereditary hemolytic anemia in Caucasians?

Which of the following RPI results will suggest an inadequate response of erythropoiesis by bone marrow in response to anemia?

Which of the following is not characteristic of hereditary hydrocytosis (stomatocytosis)?

Which of the following laboratory results are characteristic of HS?

Morphology of Human Blood and Marrow Cells: Hematopoiesis

Bone Marrow

The Red Blood Cell: Structure and Function

Anemia: Diagnosis and Clinical Considerations

Evaluation of Red Blood Cell Morphology and Introduction to Platelet and White Blood Cell Morphology

Iron Metabolism and Hypochromic Anemias

Megaloblastic Anemias

Aplastic Anemia Including Pure Red Cell Aplasia and Congenital Dyserythropoietic Anemia and Paroxysmal Nocturnal Hemoglobinuria

Hemolytic Anemias: Intracorpuscular Defects: II Hereditary Enzyme Deficiencies

Hemolytic Anemias: Intracorpuscular Defects: III the Hemoglobinopathies

Hemolytic Anemias: Intracorpuscular Defects: Iv Thalassemia

Hemolytic Anemias: Extracorpuscular Defects

Hypoproliferative Anemia: Anemia Associated With Systemic Diseases

Cell Biology, Disorders of Neutrophils, Infectious Mononucleosis, and Reactive Lymphocytosis

Introduction to Leukemia and the Acute Leukemias

Chronic Myeloproliferative Disorders I: Chronic Myelogenous Leukemia

Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

Myelodysplastic Syndromes

Chronic Lymphocytic Leukemia and Related Lymphoproliferative Disorders

The Lymphomas

Multiple Myeloma and Related Plasma Cell Disorders

Lipid Lysosomal Storage Diseases and Histiocytosis

Introduction to Hemostasis

Disorders of Primary Hemostasis: Quantitative and Qualitative Platelet Disorders and Vascular Disorders

Disorders of Plasma Clotting Factors

Interaction of the Fibrinolytic, Coagulation, and Kinin Systems; Disseminated Intravascular Coagulation; and Related Pathology

Introduction to Thrombosis and Anticoagulant Therapy

Quality Control in the Hematology Laboratory

Body Fluid Examination: the Qualitative, Quantitative, and Morphologic Analysis of Serous, Cerebrospinal, and Synovial Fluids

Hematology Methods

Principles of Automated Differential Analysis

Coagulation Procedures

Applications of Flow Cytometry to Hematology and Hemostasis

Molecular Diagnostic Techniques in Hematopathology

Special Stainscytochemistry

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