Question 1

The deletion of two α -globin chains of hemoglobin is the cause of
A) α -thalassemia trait
B) α -thalassemia minor
C) salient carrier state
D) hemoglobin H disease
E) hydrops fetalis

Accepted Answer

Deletion of two a-globin genes is found in patients who have a-thalassemia trait. These patients, like those who have ß-thalassemia minor, are asymptomatic.

Question 2

The most common cause of myelophthisic anemia is
A) metastatic carcinoma to the bones
B) multiple myeloma
C) lymphoma
D) leukemia
E) osteosclerosis

Accepted Answer

Myelophthisic anemia, resulting from the destruction and replacement of the hematopoietic bone marrow by other cells, is most often caused by metastatic carcinomas. These tumors usually originate from primary cancers in the breast, lung, or prostate.

Question 3

Severe hemophilia develops in patients who have factor VIII reduced below
A) 1%
B) 5%
C) 10%
D) 20%
E) 25%

Accepted Answer

Severe hemophilia occurs only in patients who have less than 1% of the normal concentration of factor VIII. Levels in the range of 2% to 5% are associated with moderate hemophilia, whereas patients who have 6% to 15% of the normal levels have mild hemophilia.

Question 4

The most common form of autosomal dominant hereditary spherocytosis is caused by a mutation of the gene encoding which of the following proteins?
A) Ankyrin
B) Spectrin
C) Protein 4.1
D) Hemoglobin A
E) Transferrin

Accepted Answer

The most common form of autosomal dominant hereditary spherocytosis is caused by a mutation of the ankyrin gene. However, a deficiency of spectrin, which may be primary or secondary due to another defect, is found in most forms of hereditary spherocytosis, making it the most common biochemical abnormality in this disease.

Question 5

Hemolytic uremic syndrome (HUS) shares many features with thrombotic thrombocytopenic purpura (TTP), but in contrast with patients with TTP, those with HUS do NOT have
A) anemia
B) renal failure
C) neurologic deficits
D) fever
E) thrombocytopenia

Accepted Answer

Hemolytic uremic syndrome (HUS) most commonly develops following a gastrointestinal infection caused by verotoxin-producing Escherichia coli O157:H7. It resembles thrombotic thrombocytopenic purpura (TTP), but in contrast to TTP, HUS does not cause neurologic deficits.

Question 6

Which is the most common histologic form of Hodgkin disease?
A) Lymphocyte predominance
B) Nodular sclerosis
C) Mixed cellularity
D) Lymphocyte depletion
E) Nodular lymphocyte predominance

Accepted Answer

Nodular sclerosis is the most common histologic form of Hodgkin disease and accounts for approximately 70% of all cases.

Question 7

Which is the most common cause of microcytic hypochromic anemia in American women aged 20 to 50 years? A) Autoimmune hemolysis B) Iron deficiency C) Vitamin B₁₂ deficiency D) Thalassemia minor E) Viral infection

Accepted Answer

Iron deficiency resulting from the monthly loss of iron in menstrual blood is the most common cause of microcytic hypochromic anemia.

Question 8

Terminal deoxynucleotidyltransferase is a marker of
A) B cells
B) T cells
C) Precursors of B and T lymphocytes
D) Plasma cells
E) Natural killer cells

Accepted Answer

Terminal deoxynucleotidyltransferase, a specialized DNA polymerase, is expressed on undifferentiated pre-B and pre-T lymphoblasts. It is typically found in more than 95% of cases of acute lymphoblastic leukemia of the precursor B-cell and T-cell type.

Question 9

The monoclonal immunoglobulin, the so-called M protein of multiple myeloma, belongs to which class of immunoglobulins?
A) IgA
B) IgD
C) IgE
D) IgG
E) IgM

Accepted Answer

The M protein, which is found in 55% of patients with multiple myeloma, belongs to the IgG class.

Question 10

Which of the following bleeding tests is abnormal in patients with von Willebrand disease?
A) Prolonged bleeding time
B) Thrombocytopenia
C) Prolonged prothrombin time
D) Shortened partial thromboplastin time
E) Increased levels of factor VIII in plasma

Accepted Answer

A deficiency of the von Willebrand factor (vWF) in this disease, which carries the same name, affects the ability of platelets to participate in hemostasis. The bleeding time, which was designed to measure the function of platelets and blood vessels, is thus prolonged. Because vWF interacts with factor VIII, partial thromboplastin time is also prolonged and the concentration of factor VIII in blood is reduced.

Question 11

Transformation of chronic lymphocytic leukemia or small cell lymphocytic lymphoma into a large B-cell lymphoma is called
A) Burkitt lymphoma
B) Waldenstrom macroglobulinemia
C) Richter syndrome
D) myelodysplastic syndrome
E) mycosis fungoides

Accepted Answer

Terminal transformation of low-grade lymphoma/leukemia into high-grade lymphoblastic lymphoma is called Richter syndrome. It occurs in 10% of patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL).

Question 12

Which of the following is the most common aggressive form of non-Hodgkin lymphoma in the United States?
A) Follicular lymphoma
B) Diffuse large B-cell lymphoma
C) Mantle cell lymphoma
D) Peripheral T-cell lymphoma
E) Angiocentric lymphoma

Accepted Answer

Diffuse large B-cell lymphomas account for 60% to 70% of all aggressive forms of lymphoma. These tumors thus form the largest group of high-grade malignant lymphomas in the United States.

Question 13

Aplastic crisis, which can be encountered in patients with hereditary spherocytosis or sickle cell anemia, is most often precipitated by
A) deficiency of erythropoietin
B) negative feedback inhibition by bilirubin
C) adverse effect of iron released from hemolyzed erythrocytes
D) autoimmune mechanisms
E) parvovirus infection

Accepted Answer

Aplastic crisis in hereditary spherocytosis or sickle cell anemia is usually triggered by parvovirus infection. The virus infects and destroys the red blood cell precursors in the bone marrow.

Question 14

Which test is abnormal in hemophilia A?
A) Bleeding
B) Platelet count
C) Prothrombin time
D) Partial thromboplastin time
E) Clot retraction

Accepted Answer

Hemophilia A results from a deficiency of clotting factor VIII, which participates in the intrinsic coagulation pathway. Partial thromboplastin time provides information about the intrinsic pathway components and is thus prolonged in hemophilia A and hemophilia B.

Question 15

Which form of lymphoma tends to arise in the gastrointestinal tract at the site of chronic inflammation, such as chronic sialadenitis in Sjögren disease or chronic gastritis caused by Helicobacter pylori?
A) Pre-B, pre-T lymphoblastic lymphoma
B) Mantle cell lymphoma
C) Marginal cell lymphoma
D) Peripheral T-cell lymphoma
E) Burkitt lymphoma

Accepted Answer

Marginal cell lymphomas, also known as mantle cell lymphomas, are low-grade lymphomas that occur at the site of chronic inflammation in the gastrointestinal tract.

Question 16

What is the most common form of non-Hodgkin lymphoma in the United States?
A) Follicular lymphoma
B) Diffuse large B-cell lymphoma
C) Mantle cell lymphoma
D) Peripheral T-cell lymphoma
E) Angiocentric lymphoma

Accepted Answer

Follicular lymphoma accounts for 45% of all non-Hodgkin lymphomas and is the most common lymphoma in the United States.

Question 17

Normocytic normochromic anemia, associated with low serum iron, reduced total-iron binding capacity, and abundant iron stores, as seen in the bone marrow biopsy, are laboratory findings typically found in anemia of A) iron deficiency B) chronic disease C) thalassemia minor D) thalassemia trait E) vitamin B₁₂ deficiency

Accepted Answer

Anemia of chronic disease is typically normocytic, hypochromic, and associated with hypoutilization of iron, reflected in abundant iron stores and reduced total iron-binding capacity.

Question 18

What are the mechanisms of thrombocytopenia in patients who have giant hemangiomas?
A) Autoimmune destruction of platelets
B) Inhibition of bone marrow
C) Mechanical destruction of platelets
D) Increased consumption of platelets in peripheral blood vessels
E) Disseminated intravascular hemolysis

Accepted Answer

The turbulent blood flow through the giant hemangiomas promotes mechanical destruction of platelets.

Question 19

A 15-year-old boy developed generalized lymphadenopathy, accompanied by fever and malaise. A lymph node biopsy specimen showed that the architecture of the lymph nodes was erased and the nodes were infiltrated with moderately large lymphoid cells. There were numerous mitoses and apoptotic bodies. Between the B lymphocytes, there were phagocytic macrophages, imparting a "starry-sky" appearance to the lymph node. Which of the following is the most likely diagnosis?
A) Follicular lymphoma
B) Large B-cell lymphoma
C) Chronic lymphocytic leukemia/small lymphocytic lymphoma
D) Burkitt lymphoma
E) Multiple myeloma

Accepted Answer

Burkitt lymphoma presents with diffuse infiltration of the lymph nodes. There are many mitoses, apoptotic bodies, and macrophages that impart a starry-sky appearance to the lymph node.

Question 20

A 20-year-old woman was diagnosed with Hodgkin disease involving the mediastinum. Which would be the most common histologic form of Hodgkin disease to occur at this site?
A) Lymphocyte predominance
B) Nodular sclerosis
C) Mixed cellularity
D) Lymphocyte depletion
E) Nodular lymphoid predominance

Accepted Answer

Nodular sclerosis most often affects young women and presents with mediastinal involvement.

Quiz 11: Hematopoietic and Lymphoid Systems

The deletion of two α -globin chains of hemoglobin is the cause of

The most common cause of myelophthisic anemia is

Severe hemophilia develops in patients who have factor VIII reduced below

The most common form of autosomal dominant hereditary spherocytosis is caused by a mutation of the gene encoding which of the following proteins?

Hemolytic uremic syndrome (HUS) shares many features with thrombotic thrombocytopenic purpura (TTP) , but in contrast with patients with TTP, those with HUS do NOT have

Which is the most common histologic form of Hodgkin disease?

Which is the most common cause of microcytic hypochromic anemia in American women aged 20 to 50 years?

Terminal deoxynucleotidyltransferase is a marker of

The monoclonal immunoglobulin, the so-called M protein of multiple myeloma, belongs to which class of immunoglobulins?

Which of the following bleeding tests is abnormal in patients with von Willebrand disease?

Transformation of chronic lymphocytic leukemia or small cell lymphocytic lymphoma into a large B-cell lymphoma is called

Which of the following is the most common aggressive form of non-Hodgkin lymphoma in the United States?

Aplastic crisis, which can be encountered in patients with hereditary spherocytosis or sickle cell anemia, is most often precipitated by

Which test is abnormal in hemophilia A?

Which form of lymphoma tends to arise in the gastrointestinal tract at the site of chronic inflammation, such as chronic sialadenitis in Sjögren disease or chronic gastritis caused by Helicobacter pylori?

What is the most common form of non-Hodgkin lymphoma in the United States?

Normocytic normochromic anemia, associated with low serum iron, reduced total-iron binding capacity, and abundant iron stores, as seen in the bone marrow biopsy, are laboratory findings typically found in anemia of

What are the mechanisms of thrombocytopenia in patients who have giant hemangiomas?

A 20-year-old woman was diagnosed with Hodgkin disease involving the mediastinum. Which would be the most common histologic form of Hodgkin disease to occur at this site?