Advanced Hematology Set

A microcyte is defined as having a diameter less than which value?

7 µm

A patient with a vitamin B12 deficiency might have an anemia classified as:

Macrocytic/normochromic

A normal size red cell with a slit-like area of central pallor is called a:

Stomatocyte

A 4+ grading for anisocytosis would indicate what percentage of red blood cells (RBCs) would differ from the normal red cells?

>75%

What is the normal range for MCHC?

32-36%

An acanthocyte can be described as containing:

3-12 spicules of uneven length

A person diagnosed with lead poisoning may demonstrate:

Basophilic stippling

Which distinctive property is characteristic of a spherocyte?

Decreased surface area-to-volume ratio

What cellular remnants in a reticulocyte are visible when stained with new methylene blue?

RNA

A lack of intrinsic factor is diagnostic for which of the following?

Pernicious Anemia

Two-thirds of the total body iron is found as _________.

Heme in hemoglobin

Calculate the MCHC given the following: Hb = 9.0 g/dL, Hct = 27.4%:

33%

What is the mean corpuscular volume (MCV) used for?

All of the following

A patient is diagnosed with an associated aplastic anemia syndrome. His bone marrow examination reveals normal cellularity with absence of erythroid precursors. What is the most likely condition?

pure red cell aplasia

Heinz bodies may be seen in what hematologic disease?

All of the above

A patient diagnosed with autoimmune hemolytic anemia may have a blood smear demonstrating:

Spherocytes

Approximately how many milligrams (mg) of iron is lost during pregnancy?

1000 mg

An elderly white male of Scandinavian descent presents with the following hematopoietic studies: MCV = 115 fL, 3+ oval macrocytes, 20% hypersegmented neutrophils. What hematologic condition is suspected?

Megaloblastic anemia

A 2+ grading for poikilocytosis would indicate what percentage of RBCs would differ from normal red cells?

25-50%

A patient whose RBC indices are MCV = 80 fL, MCH = 28 pg, and mean corpuscular hemoglobin concentration (MCHC) = 34% would be classified as:

Normocytic/ Normochromic

In megaloblastic anemia, the absolute reticulocyte count is decreased as a result of:

Ineffective erythropoiesis

What factor affects the red cell's tendency to sickle in sickle cell anemia?

Low oxygen tension

To carry oxygen iron must be in what state in the heme of the hemoglobin molecule?

Ferrous (Fe2+)

The reversed myeloid-erythroid ratio in the bone marrow of a person with megaloblastic anemia is defined as:

Hypocellularity

What is the morphological classification of megaloblastic anemia?

Mactrocytic normochromic

The storage form of iron is called __________.

Ferritin

Iron absorption is influenced by which of the following?

All of the above

A patient with pernicious anemia has the following indices: mean corpuscular volume (MCV) >100 fL, mean corpuscular hemoglobin (MCH) = 31 pg, mean corpuscular hemoglobin concentration (MCHC) = 33% What might be seen on the peripheral smear?

Macrocytes

What hematologic condition is associated with the appearance of Heinz bodies?

Glucose 6-phosphate dehydrogenase (G6PD) deficiency

In PNH, treatment is directed at controlling complications that arise, such as:

All of the above

What is the mean corpuscular hemoglobin (MCH) used to measure?

Hemoglobin content

Bone marrow aspiration of a patient with aplastic anemia often results in:

Dry tap

The adult hemoglobin reference range for men and women, respectively, is:

16.0 ± 2 g/dL to 14.0 ± 2 g/dL

A patient diagnosis with hereditary spherocytosis would have the following indices:

Increased MCHC

What is most striking when viewing the peripheral smear of a severely anemic patient with megaloblastic anemia?

All of the above

Heinz bodies are a result of:

Denatured hemoglobin

A tear-drop cell is formed as a result of:

None of the above

Iron-deficiency anemia is characterized by:

Decreased serum iron, decreased ferritin, and increased total iron-binding capacity (TIBC)

What hematologic parameter on an automated instrument would be increased in megaloblastic anemia?

MCV

Calculate the MCH based on the following parameters: hemoglobin = 12 g/dL, RBC count = 4.4 × 1012/ L:

27 pg

What protein is responsible for vitamin B12 transport to the bone marrow?

Transcobalamin II

Calculate the MCV, given the following parameters: hematocrit = 36%, RBC count = 4.0 × 1012/L

90 fL

What is the average diameter of a mature erythrocyte?

7-8 µm

What is the primary cause of vitamin B12 deficiency?

Malabsorption

Which is a congenital disease associated with aplastic anemia?

Fanconi's anemia

The most common cause of death in a PNH patient is:

Thromboembolism

As cholesterol accumulates in the plasma, as in the case of patients with liver dysfunction, the cells that are observed in the blood smear are the:

Target cells

The reference range for a reticulocyte count in a normal adult is:

0.5-2.0%

One-third of the total body iron may be found in storage pools such as:

All of the following

What hemoglobin value would support the diagnosis of severe anemia?

Which of the following are common causes of iron deficiency anemia worldwide?

All of the above

The grading of "slight" anisocytosis and poikilocytosis would indicate what percentage of RBCs would differ from normal red cells?

5-10%

What is the diameter of a macrocyte?

9-12 µm

Iron-deficiency anemia is characterized by:

Microcytic, hypochromic RBCs

Codocyte is another name for which type of red cell?

Target cells

Polychromasia on the peripheral smear represents an increase in what cell and is a measure of the bone marrow's ability to replace red cells which have been destroyed?

Reticulocyte

A patient with microcytic anemia might have which of the following diseases?

All of the above

Which of the following can lead to a folic acid deficiency?

All of the above

A patient who is diagnosed with aplastic anemia and has no history of exposure to drugs, radiation, or chemicals can be said to have:

Idiopathic aplastic anemia

A patient's hemoglobin is 12 g/dL. What would you expect the hematocrit to be?

36%

Hypochromia is defined as any red cell having a central area of pallor of greater than which value?

3 µm

How many cells are usually counted in the manual reticulocyte count?

1000

Target cells may be found in which of the following conditions?

All of the above

Hemosiderosis is the accumulation of excess __________ in the macrophages of various tissues.

Iron

Approximately what percent of ingested iron is absorbed?

5-10%

A person living in a high-altitude environment reaching elevations as high as 10,000 feet will have a hemoglobin value that is:

Increased

One of the major complications of PNH is:

Venous thrombosis

In megaloblastic anemia, the band and metamyelocyte may exhibit:

Giantism

What is the normal range for MCV?

80-100 fL

Rouleaux formation is the result of:

Increased fibrinogen

What hemoglobin range will suggest a newborn patient?

Newborn HGB range 17-23 g/dL (ask why was this marked incorrect!) 20+/-2

A drepanocyte is another name for which red cell?

Sickel cell

The mature erythrocyte, upon maturity, passes from a:

Nucleated to non-nucleated state

What protein is responsible for vitamin B12 absorption?

Intrinsic factor

Leukocytosis commonly refers to an increase in peripheral blood white blood cell (WBC) counts of greater than what value?

10,000 cells/μL

1. What is another name for thalassemia major?

A) Cooley's anemia

B) Fanconi's anemia

C) Alpha thalassemia

D) Schilling's syndrome

E) None of the above

A

2. In contrast to beta thalassemia, alpha thalassemia usually manifests:

A) After age 40

B) At the onset of puberty

C) Immediately at birth

D) Only in postmenopausal women

E) After age 10

C

3. Gene deletions are the cause of the majority of alpha thalassemia cases because:

A) There are no areas of homology between the two closely linked alpha genes globin.

B) The a2 globin gene is expressed at two to three times the rate of a1 globin gene.

C) Great homology in the gene blocks render the areas more susceptible to deletion error during crossover.

D) Alpha genes are juxtapositioned to numerous transcriptional mutants.

E) None of the above

C

4. Hemoglobin A consists of:

A) Two alpha (a) chains and two gamma (g) chains

B) Two alpha chains and two delta (d) chains

C) Two alpha chains and two beta (b) chains

D) Two alpha chains and two epsilon (e) chains

E) None of the above

C

5. It has been hypothesized that a person who is heterozygous for the thalassemia gene is resistant to malaria caused by __________.

A) Plasmodium ovale

B) Plasmodium falciparum

C) Plasmodium vivax

D) Plasmodium malariae

E) None of the above

B

6. A person native to Thailand would have more probability of presenting with:

A) Thalassemia major

B) Alpha thalassemia

C) Beta thalassemia

D) Gamma-beta thalassemia

E) None of the above

B

7. What hemoglobin structures make up hemoglobin A2?

A) Zeta 2 epsilon 2

B) Alpha 2 beta 2

C) Alpha 2 gamma 2

D) Alpha 2 delta 2

E) None of the above

D

8. What percentage of normal hemoglobin consists of hemoglobin A?

A) Gene addition

B) Gene fusion

C) Point mutation

D) Point elongation

E) None of the above

C

9. The anemia of thalassemia is morphologically characterized as __________ anemia.

A) Macrocytic/hyperchromic

B) Microcytic/hypochromic

C) Normocytic/normochromic

D) Microcytic/normochromic

E) None of the above

B

10. Which of the following findings would not be indicative of heterozygous b thalassemia?

A) Hemoglobin A2 level of 3.5% to 7%

B) Hemoglobin F level of 2% to 5%

C) Hemoglobin A level of 65% to 85%

D) Hemoglobin A level of 90% to 95%

E) D and A are correct

D

11. Thalassemia is found:

A) Only in the Mediterranean Sea area

B) Only in Australia and South Pacific Islands

C) Only in Southeast Asia

D) Only on the African continent

E) Worldwide

E

12. Which type of bb+ thalassemia produces the least amount of beta chains?

A) Type 1

B) Type 2

C) Type 3

D) Type 4

E) None of the above

A

13. Homozygosity for the b0 or b+ thalassemia gene results in:

A) Hemoglobin H disease

B) Alpha thalassemia

C) Thalassemia major

D) Thalassemia minor

E) None of the above

C

14. The characteristic facial changes in a patient diagnosed with thalassemia major include hypertrophy of maxilla, widely spared eyes, and prominent cheekbones. This is the result of:

A) Expansion of marrow space

B) Extramedullary hematopoiesis

C) Vitamin B12 deficiency

D) Increase in hemoglobins

E) None of the above

A

15. What hemoglobin consists of four gamma chains?

A) Hemoglobin H

B) Hemoglobin Bart's

C) Hemoglobin F

D) Hemoglobin A2

E) None of the above

B

16. Decreased or absent alpha-chain production will result in:

A) Decreased gamma chain production during fetal life and adult life

B) Excess gamma chain production during fetal life and at birth

C) No gamma chain production during fetal life

D) No gamma chain production during adult life

E) None of the above

B

17. Which non-alpha thalassemias may be expected to have similar if not identical hemoglobin A, A2, and F levels?

A) Homozygous hereditary persistence of fetal hemoglobin (HPFH) and homozygous delta-beta thalassemia

B) Homozygous Hgb Lepare and thalassemia intermedia

C) Thalassemia major and thalassemia minor

D) Heterozygous HPFH and homozygous HPFH

E) None of the above

A

18. What lethal disease is characterized by homozygosity of the a0-thalassemia gene haplotype in utero?

A) Hydrops fetalis

B) Hemolytic disease of the newborn (HDN)

C) Hemoglobin Portland disease

D) Hemoglobin H disease

E) Hemoglobin Zurich

A

19. The distinguishing feature of both alpha thalassemia-one-trait and alpha thalassemia-two-trait conditions compared to Bart's hydrops fetalis or hemoglobin H disease is:

A) Lack of hemoglobin Bart's at birth

B) Disappearance of hemoglobin Bart's with development

C) Persistence of high levels of fetal hemoglobin into adulthood.

D) Excessive amounts of hemoglobin Portland at birth

E) None of the above

B

20. All of the following comprise the clinical categories of alpha thalassemia except:

A) Bart's hydrops fetalis syndrome

B) Hemoglobin H disease

C) Hereditary persistence of fetal hemoglobin

D) Alpha thalassemia 1

E) Alpha thalassemia 2

C

21. Which abnormal hemoglobin is produced from a fusion of delta and beta chains?

A) Hemoglobin Lepore

B) Hemoglobin Portland

C) Hemoglobin Constant Spring

D) Hemoglobin Bart's

E) Hemoglobin Zurich

A

22. Which form of HPFH is characterized by uniform distribution among red cells when stained by the Kleinhauer-Betke method?

A) Hypocellular

B) Pancellular

C) Heterocellular

D) Hypercellular

E) Acellular

B

23. What thalassemia-associated variant will result in sickling crisis in severe hypoxic conditions?

A) Beta thalassemia/hemoglobin E

B) Beta thalassemia/hemoglobin C

C) Beta thalassemia/hemoglobin S

D) Alpha thalassemia/sickle cell

E) Hemoglobin SC

C

24. Which clinical expression of thalassemia is associated with a healthy silent carrier showing no clinical symptoms and minimal to no hematologic abnormalities?

A) Thalassemia major

B) Thalassemia minor

C) Thalassemia intermedia

D) Bart's hydrops fetalis

E) None of the above

D

25. Therapy for thalassemia major patients may include:

A) High blood transfusion program

B) Splenectomy

C) Bone marrow transplant

D) All of the above

E) None of the above

D