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The following are compounds formed in the synthesis of heme:
1 coproporphyrinogen
2 porphobilinogen
3 uroporphyrinogen
3 protoporphyrinogen
Which of the following responses lists these compounds in the order in which they are formed?
a. 4, 3, 1, 2,
b. 2, 3, 1, 4
c. 4, 2, 3, 1
d. 2, 1, 3, 4
b
The majority of the iron in an adult is found as a constituent of:
a. hemoglobin
b. hemosiderin
c. myoglobin
d. transferrin
a
In order for hemoglobin to combine reversibly with oxygen, the iron must be:
a complexed with haptoglobin
b. freely circulating in the cytoplasm
c. attached to transferrin
d. in the ferrous state
d
Which description best fits the Donath-Landsteiner antibody?
a. IgM cold agglutinin
b. Biphasic IgM hemolysin
c. IgG biphasic hemolysin
d. IgG warm agglutinin
c
Which of the following ions is bound to hemoglobin in methemoglobin?
a. Ca2+
b. Fe3+
c. Fe3+
d. Mg2+
b
An increased amount of cytoplasmic basophilia in a blood cell indicates:
a. increased cytoplasmic maturation
b. decreased cytoplasmic maturation
c. reduction in size of the cell
d. decreased nuclear maturation
b
Specific (secondary) granules of the neutrophilic granulocyte:
a. appear first at the myelocyte stage
b. contain esterases
c. are formed on the mitochondria
d. are derived from azurophil (primary) granules
a
In normal adult bone marrow, the most common granulocyte is the?
a. basophil
b. myeloblast
c. eosinophil
d. metamyelocyte
d
Pluripotent hematopoietic stem cells are capable of producing:
a. daughter cells of only one cell line
b. only T lymphocytes and B lymphocytes
c. erytropoietin, thrombopoietin, and leukopoietin
d. lymphoid and myeloid stem cells
d
Which of the following cytokines is most responsible for eosinophil differentiation and release from the bone marrow?
a. IL-1
b. IL-2
c. IL-4
d. IL-5
d
Auer rods are characterized as:
a. fused primary granules
b. DNA precipitates
c. denatured hemoglobin
d. large cytoplasmic granules
b
Which of the following is characteristic of cellular changes as megakaryoblasts mature into megakaryocytes with the bone marrow?
a. progressive decrease in overall cell size
b. increasing basophilia of cytoplasm
c. nuclear division without cytoplasmic division
d. fusion of the nuclear lobes
c
Which of the following cells is the largest cell in the bone marrow:
a. megakaryocyte
b. histiocyte
c. osteoblast
d. mast cell
a
Which one of the following is a true statement about megakaryocytes in a bone marrow aspirate?
a. an average of 2-10 should be found in each low power field (10x)
b. the majority of forms are the MK1 stage
c. morphology must be determined from the biopsy section
d. quantitative estimation is done using the 100x oil immersion lens
a
After the removal of red blood cells from the circulation, hemoglobin is broken down into:
a. iron, porphyrin, and amino acids
b. iron, protoporphyrin, and globin
c. heme, protoporphyrin, and amino acids
d. heme, hemosiderin, and globin
b
The main function of the hexose monophosphate shunt in the erythrocyte is to:
a. regulate the level of 2,3-DPG
b. provide reduced glutatione to prevent hemoglobin oxidation
c. prevent the reduction of heme to iron
d. provide energy for membrane maintenance
b
In the normal adult, the spleen acts as a site for:
A. storage of RBCs
B. production of RBCs
C. synthesis of erythropoietin
D. removal of imperfect and aging cells
d
Cells for the transport of O2 and CO2 are:
a. erythrocytes
b. granulocytes
c. lymphocytes
d. thrombocytes
a
Erythropoietin acts to:
a. shorten the replication time of the granules
b. stimulates RNA synthesis of erythroid cells
c. increase colony-stimulating factors produced by the B lymphocytes
d. decrease the release of marrow reticulocytes
b
Cells that produce antibodies and lymphokines are:
a. erythrocytes
b. granulocytes
c. lymphocytes
d. thrombocytes
c
Phagocytosis is a function of"
a. a. erythrocytes
b. granulocytes
c. lymphocytes
d. thrombocytes
b
Which cells are involved in immediate hypersensitivity reactions?
a. eosinophils
b. basophils
c. plasma cells
d. reactive lymphocytes
b
Alpha granules are found on the platelet in the:
a. peripheral zone
b. sol gel zone
c. organelle zone
d. membranes
c
Which of the following best represents the 3 steps of normal hemostasis (in order)?
a. decreased heart rate, adhesion of platelets, plug formation
b. platelet aggregation, formation of FXII, fibrin plug
c. vasoconstriction, platelet agregation, fibrin formation
d. vascular damage, stasis, endothelial injury
C
Which of the following platelet antigens is the receptor for collagen?
a. GPib/V/IX complex
b. GPIIb/IIa complex
c. GPIa/lla complex
d. GPIc/lla complex
C
Which platelet surface antigen acts as the receptor for fibrinogen?
a. GPib/V/IX complex
b. GPIIb/IIa complex
c. GPIa/lla complex
d. GPIc/lla complex
B
How does GPib become activated in vivo and in vitro respectively?
a. shear force, ristocetin
b. ristocetin, compression
c. activation of ADP receptor, restocetin
d. binding vWF, epinephrine
A
The characteristic erythrocyte found in pernicious anemia is:
a. microcytic
b. spherocytic
c. hypochromic
d. macrocytic
D
Hemolysis in paroxsymal nocturnal hemoglobinuria (PNH) is:
a. temperature dependent
b. complement dependent
c. antibody-mediated
d. caused by a red cell membrane defect
D
Which of the following is most closely associated with idiopathic hemochromatosis?
a. iron overload in tissue
b. target cells
c. basophilic stippling
d. ringed sideroblasts
A
A patient with polycythemia vera who is treated by phlebotomy is most likely to develop a deficiency of:
a. iron
b. vitamin B12
c. folic acid
d. erythropoeitin
A
the direct antiglobulin test can help distinguish:
a. inherited from acquired spherocytosis
b. intravascular from extravascular hemolysis
c. heterozygous from homozygous thalassemia
d. sickle cell trait from sickle cell disease
A
The anemia of chronic infection is characterized by:
a. decreased iron stores in the reticuloendothelial system
b. decreased serum iron levels
c. macrocytic erythrocytes
d. increased serum iron binding capacity
B
Factors commonly involved in producing anemia in patients with chronic renal disease include:
a. marrow hypoplasia
b. inadequate erythropoiesis
c. vitamin B12 deficiency
d. increased erythropoietin production
B
A 20 year old woman with sickle cell anemia whose usual hemoglobin concentration is 8 g/dL develops fever, increased weakness and malaise. The hemoglobin concentration is 4 g/dL and the reticulocyte count is 0.1%. The most likely explanation for her clinical picture is:
a. increased hemolysis due to hypersplenism
b. aplastic crisis
c. thrombotic crisis
d. occult blood loss
b
The hypoproliferative red cell population in the bone marrow of uremic patients is caused by:
a. infiltration of bone marrow by toxic waste products
b. decreased levels of circulating of erythropoietin
c. defective globin synthesis
d. overcrowding of bone marrow space by increased myeloid precursors
b
Which of the following characteristics are common to hereditary sperocytosis, hereditary elliptocytosis, hereditary stomatocytosis, and paroxysmal nocturnal hemoglobinuria
a. autosomal dominant inheritance
b. red cell membrane defects
c. positive direct antiglobulin test
d. measure platelet count
b
Which of the following is most closely associated with iron deficiency anemia?
a. iron overload in tissue
b. macrocytes
c. basophilic stippling
d. chronic blood loss
d
Which one of the following hypochromic anemias is usually associated with a normal free erythrocyte protoporphyrin level?
a. anemia of chronic disease
b. iron deficiency
c. lead poisoning
d. thalassemia minor
D
Evidence indicates that the genetic defect in thalassemia usually results in:
a. the production of abnormal globin chains
b. a quantitative deficiency in RNA resulting in decreased globin chain production
c. a structural change in the heme portion of the hemoglobin
d. an abnormality in the alpha or beta chain binding or affinity
b
A 20 year old African American man has peripheral blood changes suggesting thalassemia minor. The quantitative hemoglobin A2 level is normal but the hemoglobin level is 5% (normal <2%). This is most consistent with:
a. alpha thalassemia minor
b. beta thalassemia minor
c. delta beta thalassemia minor
d. heriditary persistence of fetal hemoglobin
c
Anemia secondary to uremia characteristically is:
a. microcytic, hypochromic
b. hemolytic
c. normocytic, normochromic
d. macrocytic
c
Which of the following sets of laboratory findings is consistent with hemolytic anemia?
a. normal or slightly increased erythrocyte survival; normal reticulocyte count
b. decreased erythrocyte survival: increased catabolism of heme
c. decreased serum lactate dehydrogenase activity; normal catabolism of heme
d. normal concentration of haptoglobin; marked hemoglobinuriab
b
An enzyme deficiency associated with a moderate to severe hemolytic anemia after the patient is exposed to certain drugs and characterized by red cell inclusions formed by denatured hemoglobin is?
a. lactate dehydrogenase deficiency
b. G6PD deficiency
c. pyruvate kinase deficiency
d. hexokinase deficiency
b
Patients with G6PD deficiency are least likely to have hemolytic episodes in which of the following situations?
a. following the administration of oxidizing drugs
b. following the engestion of fava beans
c. during infections
d. spontaneously
d
A patient has a congenital nonspherocytic hemolytic anemia. After exposure to anti-malaria drugs the patient experiences a severe hemolytic episode. This episode is characterized by red cells inclusions caused by hemoglobic denaturation. Which of the following conditions is most consistent with these findings?
a. G6PD deficiency
b. thalassemia major
c. pyruvate kinase deficiency
d. paroxysmal nocturnal hemoglobinuria
a
All of the findings listed below may be seen in acquired hemolytic anemias of the autoimmune variety. The one considered to be the MOST characteristic is:
a. increased reticulocyte count
b. leukopenia and thrombocytopenia
c. peripheral spherocytosis
d. positive direct antiglobulin test
d
Peripheral blood smears from patients with untreated pernicious anemia are characterized by:
a. pancytopenia and macrocytosis
b. leukocytosis and elliptocytosis
c. leukocytosis and ovalocytosis
d. pancytopenia and microcytosis
a
The laboratory tests performed on a patient indicate macrocytosis, anemia, leukopenia, and thrombocytopenia. Which of the following disorders is the patient most likely to have?
a. anemia of chronic disease
b. vitamin B12 deficiency
c. iron deficiency
d. acute hemorrhage
B
The characteristic morphologic feature in folic acid deficiency is:
a. macrocytosis
b. target cells
c. basophilic stippling
d. rouleaux formation
A
Megaloblastic asynchronus development in the bone marrow indicates which one of the following?
a. proliferation of erythrocyte precursors
b. impaired synthesis of DNA
c. inadequate production of erythropoietin
d. deficiency in G6PD
B
Which of the following are found in association with megaloblastic anemia?
a. neutropenia and thrombocytopenia
b. decreased LD activity
c. increased erythrocyte folate levels
d. decreased plasma bilirubin levels
a
A characteristic morphologic feature in hemoglobin C disease is:
a. macrocytosis
b. sperocytosis
c. rouleaux formation
d. target cells
d
Thalassemias are characterized by:
a. structural abnormalities in the hemoglobin molecule
b. absence of iron in hemoglobin
c. decreased rate of heme synthesis
d. decreased rate of globin synthesis
d
Laboratory findings in hereditary spherocytosis include:
a. decreased WBCs
b. decreased RBC band 3 protein
c. reticulocytopenia
d. positive direct antiglobulin test
b
Which of the following types of polycythemia is a severely burned patient most likely to have?
a. polycythemia vera
b. polycythemia secondary to hypoxia
c. relative polycythemia associated with dehydration
d. polycythemia associated with renal disease
c
The characteristic morphological feature in lead poisoning is:
a. macrocytosis
b. target cells (codocytes)
c. basophilic stippling
d. rouleaux formation
c
Which of the following is characteristic of polycythemia vera?
a. elevated urine erythropoietin levels
b. increase oxygen affinity of hemoglobin
c. decreased hematocrit
d. decreased or absent bone marrow iron stores
d
The white cell feature most characteristic of pernicious anemia is:
a. eosinophilia
b. toxic granulation
c. hypersegmentation
d. reactive lymphocytes
c
Which paremeter is most constistently abnormal in cases of hereditary spherocytosis?
a. RBC count
b. MCV
c. hypersegmentation
d. MCHC
d
What protein is commonly defective in hereditary elliptocytosis?
a. ankyrin
b. spectrin
c. band 4.1
d. elliptocin
b
What is the most common mechanism resulting in hereditary stomatocytosis?
a. abnormal Na/K permeability
b. deficient cytoskeletal structural proteins
c. inability to repair oxidative stress damage
d. ATP depletion due to glycolytic enzyme deficiency
a
What accounts for the majority of cases of sideroblastic anemia?
a. clonal stem cell defect
b. medications
c. alcohol
d. irradiation
a
To what class of disorders does Fancooni anemia belong?
a. hypoproliferative
b. myeloproliferative
c. myelodysplastic
d. mitochondrial
a
Two common causes of acquired pure red cell aplasia are:
a. cytomegalovirus and parvovirus infection
b. thymoma and parvovirus infection
c. parvovirus infection and squamous cell carcinoma
d. squamous cell carcinoma and thymoma
b
Which of the following features of G6PD deficiency are typically present on a Wright Giemsa stained peripheral blood smear?
a. Cabot rings
b. microcytosis
c. bite cells
d. Heinz bodies
c
Which abnormal RBC morphology is associated with pyruvate kinase deficiency?
a. acanthocytes
b. dacrocytes
c. bite cells
d. drepanocytes
c
Which of the following hemoglobinopathies is associated with rod shaped crystals?
a. HgbS
b. HgbC
c. HgbSC
d. HgbD
b
Which of the following statements about hemoglobin D and G is true?
a. they are clinically abnormal
b. they both migrate with HgbS on alkaline gel
c. they are both caused by mutations in the beta-globin gene
d. they cannot be separated on citrate gels
b
This hemoglobinopathy results from a fusion product of the delta and beta gene:
a. HgbD
b. HgbG
c. Hgb Lepore
d. Hgb Constant Spring
c
Which of the following is consistent with the diagnosis of heterozygous beta-thalassemia?
a. increased red blood cell count
b. high MCV
c. decreased HgbA2
d. decreased iron stores
a
Hereditary persistence of fetal hemoglobin (HPFH) is due to a loss of expression of this globin chain:
a. alpha
b. beta
c. gamma
d. delta
c
What is the specificity of cold agglutinin disease?
a. anti-i
B. anti-H
c. anti-Pr
d. anti-I
d
What is the most common presentation of paroxysmal cold hemoglobinuria?
a. older people with Raynaud syndrome
b. children following a viral illness
c. neonates with congenital syphilis
d. alcoholics with advanced cirrhosis
b
Which of the following is the most common cause of anemia in hospitalized patients?
a. inadequate iron intake
b. inadequate folate intake
c. hemolytic anemia
d. anemia of chronic disease
d
In a patient with an increased red cell mass into the 99th percentile and serum erythropoietin level below reference range for normal, which of the following criteria confirms a diagnosis of polycythemia vera?
a. bone marrow panmyelosis
b. inv(16) mutation
c. JAK2 V617 mutation
d. BCR-ABL1 transolcation
c
A medical technologist is examining a peripheral smear and notices 7 large segmented neutrophils with between 5 and 7 lobes. Everything else about the CBC is otherwise normal. This observed morphologic change might develop months ahead of which of the following changes:
a. an increase in MCV, MCH and RDW
b. a decrease in MCV, MCH, RDW
c. an increase in metamyelocytes and bands
d. a bone marrow showing aplasia
a
Which red cell morphology may be expected in a treated polycythemia vera patient?
a. microcytic cells
b. teardrop cells
c. helmet cells
d. oval macrocytes
a
Hemoglobin H disease results from:
a. absence of 3 of 4 alpha genes
b. absence of 2 of 4 alpha genes
c. absence of 1 of 1 alpha genes
d. absence of all 4 alpha genes
a
The M:E ratio in polycythemia vera is usally:
a. normal
b. high
c. low
d. variable
a
A patient has a tumor that concentrates erythropoietin. He is most likely to have which of the following types of polycythemia?
a. polycythemia vera
b. polycythemia, secondary to hypoxia
c. benign familial polycythemia
d. polycythemia associated with renal disease
d
Which of the following types of polycythemia is most often associated with lung disease?
a. polycythemia vera
b. polycythemia, secondary to hypoxia
c. relative polycythemia associated with dehydration
d. polycythemia associated with renal disease
b
A patient diagnosed with polycythemia vera 5 years previously now has a decreased hemoglobin and microcytic, hypochromic red cells. What is the most probable cause for the current peripheral blood findings?
a. phlebotomy
b. myelofibrosis
c. preleukemia
d. aplastic anemia
a
A patient has been treated for polycythemia vera for several years. His blood smear now shows
oval macrocytes
Howell-Jolly bodies
hypersegmented neutrophils
large, agranular platelets
The most probaly cause of this blood picture is:
a. iron deficiency
b. alcoholism
c. dietary B12 deficiency
d. chemotherapy
d
The most likely cause of the macrocytosis that often accompanies primary myelofibrosis is:
a. folic acid deficiency
b. increased reticulocyte count
c. inadequate B12 absorption
d. pyridoxine dificiency
a
Giant, vacuolated, multinucleated erythroid precursors are present in which of the following:
a. chronic myelocytic leukemia
b. primary myelofibrosis
c. erythroleukemia
d. acute myelocytic leukemia
c
Which of the following is a significant feature of dsyserythropiesis?
a. persistently increased M:E ration
b. megaloblastoid erythropoieisis
c. marked thrombocytosis
d. decreased ferritin levels
b
The M:E ratio in erythroleukemia is usually:
a. normal
b. high
c. low
d. variable
c
Autoimmune hemolytic anemia is often a complication of:
a. PV
b. CML
c. CLL
d. HCL
c
Elevation of the total granulocyte count above 7.0 x 10^3/µL (7.0 x 10^9/L) is termed:
A. relative lymphocytosis
B. leukocytosis
C. relative neurtophilic leukocytosis
D. absolute neutrophilic lekocytosis
d
Elevation of the total white count above 11 x 10^3/µL (11 x 10^9/L) is termed:
A. relative lymphocytosis
B. absolute lymphocytosis
C. leukocytosis
D. relative neutrophilic leukocytosis
c
Elevation of the lymphocyte percentage above 45% is termed:
a. relative lymphocytosis
b. absolute lymphocytosis
c. leukocytosis
d. absolute neutrophilic leukocytosis
a
The Philadelphia chromosome is formed by a translocation between:
a. chromosome 22 and chromosome 9
b. chromosome 21 and chromosome 9
c. chromosome 21 and chromosome 6
d. chromosome 22 and chromosome 6
a
The mechanism causing catecholamine induced neutrophilia includes:
a. a shift in granulocytes from the marginating pool to the circulating pool
b. an increased exit of granulocytes from the circulation
c. a decrease exit of granulocytes from the bone marrow
d. granulocytes return from the tissues to the circulating pool
a
What acounts for the frequent smudge cells in CLL?
a. increased in vivo cell lysis
b. apoptosis related changes
c. artifact due to fragile cells
d. artifact due to heparin
c
The M:E ration in chronic myelocytic leukemia is usually:
a. normal
b. high
c. low
d. variable
b
In the World Health Organization (WHO) classification, myelomonocytic leukemia would be acute myeloid leukemia (AML)
a. with myelodysplastic-related changes
b. with recurrent cytogenic changes
c. not otherwise specified
d. therapy-related
c
Abnormalities found in erythroleukemia include:
a. rapid DNA synthesis
b. marrow firbosis
c. megaloblastic development
d. increased erythrocyte survival
c
Neutropenia is usually associated with:
a. bacteria infections
b. viral infections
c. inflammatory processes
d. myeloproliferative neoplasms
b
Auer rods are most likely present in which of the following?
a. CML
b. primary myelofibrosis
c. erythroleukemia
d. acute myelocytic leukemia
d
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