Question 1

Gaucher's disease is due to a deficiency of the enzyme __________.

Accepted Answer

A)  Sphingomyelinase 
B)  Hexosaminidase A 
C)  β-Glucocerebrosidase 
D)  Sulfaminidase 
E)  None of the above 
A)  Sphingomyelinase 
B)  Hexosaminidase A 
C)  β-Glucocerebrosidase 
D)  Sulfaminidase 
E)  None of the above C

Question 2

The mature histiocytes present in eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease are called __________.

Accepted Answer

A)  Eosinophils 
B)  Langerhans' cells 
C)  Plasma cells 
D)  Lymphocytes 
E)  Giant cells 
A)  Eosinophils 
B)  Langerhans' cells 
C)  Plasma cells 
D)  Lymphocytes 
E)  Giant cells B

Question 3

Which organ(s) is/are affected by products of the mucopolysaccharidoses?

Accepted Answer

A)  Spleen 
B)  Bone marrow 
C)  Liver 
D)  Lymph nodes 
E)  All of the above 
A)  Spleen 
B)  Bone marrow 
C)  Liver 
D)  Lymph nodes 
E)  All of the above E

Question 4

The mucopolysaccharidoses are characterized by accumulations of unmetabolized __________ within lysosomes.

Accepted Answer

A)  Sphingomyelin 
B)  Sphingolipids 
C)  Mucopolysaccharides 
D)  Hexosaminidase A 
E)  None of the above 
A)  Sphingomyelin 
B)  Sphingolipids 
C)  Mucopolysaccharides 
D)  Hexosaminidase A 
E)  None of the above

Question 5

Histiocytes produced in sea-blue histiocyte syndrome will stain positive with all the following except:

Accepted Answer

A)  Periodic acid-Schiff (PAS) 
B)  Toluidine blue 
C)  Sudan Black B 
D)  Acid fast 
E)  None of the above 
A)  Periodic acid-Schiff (PAS) 
B)  Toluidine blue 
C)  Sudan Black B 
D)  Acid fast 
E)  None of the above

Question 6

Which stain is used to aid in confirmation of Alder-Reilly bodies?

Accepted Answer

A)  Wright's stain 
B)  New methylene blue 
C)  Giemsa stain 
D)  Toluidine blue 
E)  Prussian blue 
A)  Wright's stain 
B)  New methylene blue 
C)  Giemsa stain 
D)  Toluidine blue 
E)  Prussian blue

Question 7

Which of the following lipid storage diseases has increased incidence in the Jewish ethnic group?

Accepted Answer

A)  Gaucher's 
B)  Tay-Sachs 
C)  Niemann-Pick 
D)  All of the above 
E)  None of the above 
A)  Gaucher's 
B)  Tay-Sachs 
C)  Niemann-Pick 
D)  All of the above 
E)  None of the above

Question 8

What is the most common type of Gaucher's disease?

Accepted Answer

A)  Adult 
B)  Infantile 
C)  Juvenile 
D)  Subacute 
E)  None of the above 
A)  Adult 
B)  Infantile 
C)  Juvenile 
D)  Subacute 
E)  None of the above

Question 9

The presence of __________ may be seen with Niemann-Pick cells in the bone marrow of some adult patients.

Accepted Answer

A)  Sea-blue histiocytes 
B)  Mast cells 
C)  Macrophages 
D)  Osteoclasts 
E)  None of the above 
A)  Sea-blue histiocytes 
B)  Mast cells 
C)  Macrophages 
D)  Osteoclasts 
E)  None of the above

Question 10

Clinical manifestations of sea-blue histocyte syndrome include all of the following except:

Accepted Answer

A)  Splenomegaly 
B)  Hepatomegaly 
C)  Thrombocytopenia 
D)  Epistaxis 
E)  Enlarged lymph nodes 
A)  Splenomegaly 
B)  Hepatomegaly 
C)  Thrombocytopenia 
D)  Epistaxis 
E)  Enlarged lymph nodes

Question 11

What is the most common form of Niemann-Pick disease?

Accepted Answer

A)  Adult 
B)  Infantile 
C)  Adolescent 
D)  Juvenile 
E)  None of the above 
A)  Adult 
B)  Infantile 
C)  Adolescent 
D)  Juvenile 
E)  None of the above

Question 12

In Niemann-Pick disease there is an accumulation of __________ in histiocytes as well as cholesterol.

Accepted Answer

A)  Unmetabolized triglycerides 
B)  Metabolized triglycerides 
C)  Unmetabolized lipid sphingomyelin 
D)  Metabolized chylomicrons 
E)  None of the above 
A)  Unmetabolized triglycerides 
B)  Metabolized triglycerides 
C)  Unmetabolized lipid sphingomyelin 
D)  Metabolized chylomicrons 
E)  None of the above

Question 13

Gaucher cells are which type of cells?

Accepted Answer

A)  Megakaryocytes 
B)  Erythrocytes 
C)  Histiocytes 
D)  Macrophages 
E)  None of the above 
A)  Megakaryocytes 
B)  Erythrocytes 
C)  Histiocytes 
D)  Macrophages 
E)  None of the above

Question 14

The major site of pathology in Tay-Sachs disease is the __________.

Accepted Answer

A)  Spleen 
B)  Central nervous system 
C)  Lymph nodes 
D)  Bone marrow 
E)  Liver 
A)  Spleen 
B)  Central nervous system 
C)  Lymph nodes 
D)  Bone marrow 
E)  Liver

Question 15

Which type of Gaucher's disease has the longest survival rate?

Accepted Answer

A)  Adult 
B)  Infantile 
C)  Juvenile 
D)  Subacute 
E)  None of the above 
A)  Adult 
B)  Infantile 
C)  Juvenile 
D)  Subacute 
E)  None of the above

Question 16

What substance is present in excess in Gaucher cells?

Accepted Answer

A)  Metabolized glucocerebroside 
B)  Unmetabolized glucocerebroside 
C)  Metabolized hexosaminidase 
D)  Unmetabolized hexosaminidase 
E)  None of the above 
A)  Metabolized glucocerebroside 
B)  Unmetabolized glucocerebroside 
C)  Metabolized hexosaminidase 
D)  Unmetabolized hexosaminidase 
E)  None of the above

Question 17

The aim of control in the lipid storage diseases is directed at:

Accepted Answer

A)  Controlling anemia 
B)  Prenatal detection 
C)  Correcting bone malformation 
D)  Dietary nutrition to lower cholesterol 
E)  Hyperlipidemia 
A)  Controlling anemia 
B)  Prenatal detection 
C)  Correcting bone malformation 
D)  Dietary nutrition to lower cholesterol 
E)  Hyperlipidemia

Question 18

Which of the following are hematologic findings in Gaucher's disease?

Accepted Answer

A)  Normocytic normochromic anemia 
B)  Decreased iron in erythroid precursors 
C)  Leukopenia 
D)  Thrombocytopenia 
E)  All of the above 
A)  Normocytic normochromic anemia 
B)  Decreased iron in erythroid precursors 
C)  Leukopenia 
D)  Thrombocytopenia 
E)  All of the above

Question 19

Which of the following is/are general characteristic(s) of sea-blue histiocytosis?

Accepted Answer

A)  Striking blue histiocytes with Wright's stain 
B)  Splenomegaly 
C)  Hepatomegaly 
D)  Thrombocytopenia 
E)  All of the above 
A)  Striking blue histiocytes with Wright's stain 
B)  Splenomegaly 
C)  Hepatomegaly 
D)  Thrombocytopenia 
E)  All of the above

Question 20

In Tay-Sachs disease, the number and size of vacuoles present in __________ in the peripheral blood is related to the duration of disease.

Accepted Answer

A)  Myelocytes 
B)  Lymphocytes 
C)  Monocytes 
D)  Megakaryocytes 
E)  None of the above 
A)  Myelocytes 
B)  Lymphocytes 
C)  Monocytes 
D)  Megakaryocytes 
E)  None of the above

Exam 23: Lipid Lysosomal Storage Diseases and Histiocytosis

Gaucher's disease is due to a deficiency of the enzyme __________.

The mature histiocytes present in eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease are called __________.

Which organ(s) is/are affected by products of the mucopolysaccharidoses?

The mucopolysaccharidoses are characterized by accumulations of unmetabolized __________ within lysosomes.

Histiocytes produced in sea-blue histiocyte syndrome will stain positive with all the following except:

Which stain is used to aid in confirmation of Alder-Reilly bodies?

Which of the following lipid storage diseases has increased incidence in the Jewish ethnic group?

What is the most common type of Gaucher's disease?

The presence of __________ may be seen with Niemann-Pick cells in the bone marrow of some adult patients.

Clinical manifestations of sea-blue histocyte syndrome include all of the following except:

What is the most common form of Niemann-Pick disease?

In Niemann-Pick disease there is an accumulation of __________ in histiocytes as well as cholesterol.

Gaucher cells are which type of cells?

The major site of pathology in Tay-Sachs disease is the __________.

Which type of Gaucher's disease has the longest survival rate?

What substance is present in excess in Gaucher cells?

The aim of control in the lipid storage diseases is directed at:

Which of the following are hematologic findings in Gaucher's disease?

Which of the following is/are general characteristic(s) of sea-blue histiocytosis?

In Tay-Sachs disease, the number and size of vacuoles present in __________ in the peripheral blood is related to the duration of disease.

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

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

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

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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