- Hypersplenism is characterized by:
A. Polycythemia
B. Pancytosis
C. Leukopenia
D. Myelodysplasia
Hematology/Correlate clinical and laboratory data/WBCs/Hypersplenism/2 - Which of the following organs is responsible for the “pitting process” in RBCs?
A. Liver
B. Spleen
C. Kidney
D. Lymph nodes
Hematology/Apply knowledge of fundamental biological characteristics/Physiology/1 - Spherocytes differ from normal RBCs in all of the following except:
A. Decreased surface to volume
B. No central pallor
C. Decreased resistance to hypotonic saline
D. Increased deformability
Hematology/Apply knowledge of fundamental biological characteristics/RBC microscopic
morphology/2 - Which of the following is not associated with HS?
A. Increased osmotic fragility
B. MCHC greater than 36%
C. Intravascular hemolysis
D. Extravascular hemolysis
Hematology/Correlate clinical and laboratory data/Hereditary spherocytosis/2 - Which of the following disorders has an increase in osmotic fragility?
A. Iron deficiency anemia (IDA)
B. Hereditary elliptocytosis (HE)
C. Hereditary stomatocytosis
D. Hereditary spherocytosis (HS)
Hematology/Evaluate laboratory data to recognize health and disease states/Special
tests/Osmotic fragility/2 - The anemia seen in sickle cell disease is usually:
A. Microcytic, normochromic
B. Microcytic, hypochromic
C. Normocytic, normochromic
D. Normocytic, hypochromic
Hematology/Apply knowledge of fundamental biological characteristics/RBC microscopic
morphology/Hemoglobinopathy/1 - Which is the major Hgb found in the RBCs of patients with the sickle cell trait?
A. Hgb S
B. Hgb F
C. Hgb A2
D. Hgb A
Hematology/Apply knowledge of fundamental biological
characteristics/Anemia/Hemoglobinopathy/1 - Select the amino acid substitution that is responsible for sickle cell anemia.
A. Lysine is substituted for glutamic acid at the sixth position of the α-chain
B. Valine is substituted for glutamic acid at the sixth position of the β-chain
C. Valine is substituted for glutamic acid at the sixth position of the α-chain
D. Glutamine is substituted for glutamic acid at the sixth position of the β-chain
Hematology/Apply knowledge of fundamental biological
characteristics/Hemoglobinopathy/1 - All of the following are usually found in Hgb C disease except:
A. Hgb C crystals
B. Target cells
C. Lysine substituted for glutamic acid at the sixth position of the β–chain
D. Fast mobility of Hgb C at pH 8.6
Hematology/Apply knowledge of fundamental biological
characteristics/Anemia/Hemoglobinopathy/1 - Which of the following Hgbs migrates to the same position as Hgb A2 at pH 8.6?
A. Hgb H
B. Hgb F
C. Hgb C
D. Hgb S
Hematology/Correlate clinical and laboratory data/Hemoglobin electrophoresis/1 - Which of the following electrophoretic results is consistent with a diagnosis of the sickle
cell trait?
A. Hgb A: 40% Hgb S: 35% Hgb F: 5%
B. Hgb A: 60% Hgb S: 40% Hgb A2: 2%
C. Hgb A: 0% Hgb A2: 5% Hgb F: 95%
D. Hgb A: 80% Hgb S: 10% Hgb A2: 10%
Hematology/Evaluate laboratory data to recognize health and disease/Special
tests/Electrophoresis/2 - In which of the following conditions will autosplenectomy most likely occur?
A. Thalassemia major
B. Hgb C disease
C. Hgb SC disease
D. Sickle cell disease
Hematology/Apply knowledge of fundamental biological
characteristics/Anemia/Hemoglobinopathy/1 - Which of the following is most true of paroxysmal nocturnal hemoglobinuria (PNH)?
A. It is a rare acquired stem cell disorder that results in hemolysis
B. It is inherited as a sex-linked trait
C. It is inherited as an autosomal dominant trait
D. It is inherited as an autosomal recessive trait
Hematology/Apply knowledge of fundamental biological characteristics/PNH/1 - Hemolytic uremic syndrome (HUS) is characterized by all of the following except:
A. Hemorrhage
B. Thrombocytopenia
C. Hemoglobinuria
D. Reticulocytopenia
Hematology/Correlate clinical and laboratory data/HUS/2 - The autohemolysis test result is positive in all of the following conditions except:
A. Glucose-6-phosphate dehydrogenase (G6PD) deficiency
B. HS
C. Pyruvate kinase (PK) deficiency
D. PNH
Hematology/Correlate clinical and laboratory tests/Special tests/2 - Which antibody is associated with paroxysmal cold hemoglobinuria (PCH)?
A. Anti-I
B. Anti-i
C. Anti-M
D. Anti-P
Hematology/Apply knowledge of fundamental biological characteristics/Anemia/PCH/1 - All of the following are associated with intravascular hemolysis except:
A. Methemoglobinemia
B. Hemoglobinuria
C. Hemoglobinemia
D. Decreased haptoglobin
Hematology/Correlate clinical and laboratory data/Anemia/Hemolytic/2 - Autoimmune hemolytic anemia (AIHA) is best characterized by which of the following?
A. Increased levels of plasma C3
B. Spherocytic RBCs
C. Decreased osmotic fragility
D. Decreased unconjugated bilirubin
Hematology/Correlate clinical and laboratory data/Anemia/Hemolytic/2 - “Bite cells” are usually seen in patients with:
A. Rh null trait
B. Chronic granulomatous disease
C. G6PD deficiency
D. PK deficiency
Hematology/Correlate clinical and laboratory data/RBC microscopic morphology/1 - The morphological classification of anemias is based on which of the following?
A. Myeloid:erythroid (M:E) ratio
B. Prussian blue stain
C. RBC indices
D. Reticulocyte count
Hematology/Correlate clinical and laboratory diseases/RBC microscopic morphology/1 - Which of the following is a common finding in aplastic anemia?
A. A monoclonal disorder
B. Tumor infiltration
C. Peripheral blood pancytopenia
D. Defective deoxyribonucleic acid (DNA) synthesis
Hematology/Apply knowledge of fundamental biological characteristics/Aplastic anemia/1 - Congenital dyserythropoietic anemias (CDAs) are characterized by:
A. Bizarre multinucleated erythroblasts
B. Cytogenetic disorders
C. Megaloblastic erythropoiesis
D. An elevated M:E ratio
Hematology/Apply knowledge of fundamental biological
characteristics/Anemia/Characteristics/2 - Microangiopathic hemolytic anemia is characterized by:
A. Target cells and Cabot rings
B. Toxic granulation and Döhle bodies
C. Pappenheimer bodies and basophilic stippling
D. Schistocytes and NRBCs
Hematology/Correlate clinical and laboratory data/RBC microscopic morphology/Anemia/2 - Which antibiotic(s) is (are) most often implicated in the development of aplastic
anemia?
A. Sulfonamides
B. Penicillin
C. Tetracycline
D. Chloramphenicol
Hematology/Correlate clinical and laboratory data/Aplastic anemia/1 - Sickle cell disorders are:
A. Hereditary, intracorpuscular RBC defects
B. Hereditary, extracorpuscular RBC defects
C. Acquired, intracorpuscular RBC defects
D. Acquired, extracorpuscular RBC defects
Hematology/Apply knowledge of fundamental biological concepts/2 - Which of the following conditions may produce spherocytes in a peripheral blood
smear?
A. Pelger–Huët anomaly
B. Pernicious anemia
C. AIHA
D. Sideroblastic anemia
Hematology/Evaluate laboratory data to recognize health and disease states/Morphology/2 - A patient’s peripheral blood smear reveals numerous NRBCs, marked variation of
RBC morphology, and pronounced polychromasia. In addition to decreased Hgb and
decreased Hct values, what other CBC parameters may be anticipated?
A. Reduced PLTs
B. Increased MCHC
C. Increased MCV
D. Decreased RDW
Hematology/Correlate lab data with clinical picture/Complete blood counts/3 - What RBC inclusion may be seen in the peripheral blood smear from a patient
postsplenectomy?
A. Toxic granulation
B. Howell–Jolly bodies
C. Malarial parasites
D. Siderotic granules
Hematology/Correlate clinical laboratory data/Inclusions/1 - Reticulocytosis usually indicates:
A. Response to inflammation
B. Neoplastic process
C. Aplastic anemia
D. RBC regeneration
Hematology/Correlate laboratory data for clinical conditions/Morphology/2 - Hereditary pyropoikilocytosis (HP) is an RBC membrane defect characterized by:
A. Increased pencil-shaped cells
B. Increased oval macrocytes
C. Misshapen budding fragmented cells
D. Bite cells
Hematology/Evaluate laboratory data to recognize health and disease states/Red cell
membrane/2
Answers key
- C Hypersplenic conditions are generally described by the following four criteria: (1)
cytopenias of one or more peripheral cell lines, (2) splenomegaly, (3) bone marrow
hyperplasia, and (4) resolution of cytopenia by splenectomy. - B The spleen is the supreme filter of the body, pitting imperfections from the erythrocyte
without destroying the integrity of the membrane. - D Spherocytes lose their deformability because of a defect in spectrin, a membrane
protein, and are therefore prone to splenic sequestration and hemolysis. - C Classic features of intravascular hemolysis, such as hemoglobinemia, hemoglobinuria,
or hemosiderinuria, do not occur in HS. The hemolysis seen in HS is an extravascular
process, rather than an intravascular process. - D Spherocytic cells have decreased tolerance to swelling and, therefore, hemolyse at a
higher concentration of sodium salt compared with normal RBCs. - C Sickle cell disease is a chronic hemolytic anemia classified as a normocytic,
normochromic anemia. - D The major Hgb in sickle cell trait is Hgb A, which constitutes 50% to 70% of the total.
Hgb S comprises 20% to 40%, and Hgb A2 and Hgb F are present in normal amounts. - B The structural mutation for Hgb S is the substitution of valine for glutamic acid at the
sixth position of the β-chain. Because glutamic acid is negatively charged, this
decreases its rate of migration toward the anode at pH 8.6. - D Substitution of a positively charged amino acid for a negatively charged amino acid in
Hgb C disease results in a slower electrophoretic mobility at pH 8.6. - C At pH 8.6, several Hgbs migrate together. These include Hgb A2, Hgb C, Hgb E, Hgb
0Arab, and Hgb CHarlem. These are located nearest the cathode at pH 8.6. - B Electrophoresis at alkaline pH usually shows 50% to 70% Hgb A, 20% to 40% Hgb S,
and normal levels of Hgb A2 in a patient with the sickle cell trait. - D Autosplenectomy occurs in sickle cell anemia as a result of repeated infarcts to the
spleen caused by an overwhelming sickling phenomenon. - A PNH is a rare acquired stem cell disorder that results in abnormalities of the RBC
membrane. This causes the RBCs to become highly sensitive to complement-mediated
hemolysis. Because this is a stem cell disorder, abnormalities are seen in leukocytes
and PLTs, as well as in RBCs. PNH is characterized by recurrent, episodic
intravascular hemolysis, hemoglobinuria, and venous thrombosis. - D Hemolytic anemia of HUS is associated with reticulocytosis. The anemia seen in
HUS is multifactorial, with characteristic thrombocytopenia, schistocytes, and
polychromasia commensurate with the anemia. - D The autohemolysis test result is positive in G6PD and PK deficiencies and in HS but
is normal in PNH because lysis in PNH requires sucrose to enhance complement
binding. The addition of glucose, sucrose, or adenosine triphosphate (ATP) corrects
autohemolysis of HS. Autohemolysis of PK can be corrected by ATP. - D PCH is caused by the anti-P antibody, a cold autoantibody that binds to the patient’s
RBCs at low temperatures and fixes complement. In the classic Donath–Landsteiner
test, hemolysis is demonstrated in a sample placed at 4°C then warmed to 37°C. - A Methemoglobin occurs when iron is oxidized to the ferric state. Normally, iron is
predominantly in the ferrous state in the Hgb that circulates. During intravascular
hemolysis, the RBCs rupture, releasing Hgb directly into the bloodstream. Haptoglobin
is a protein that binds to free Hgb. The increased free Hgb in intravascular hemolysis
causes depletion of haptoglobin. As haptoglobin is depleted, unbound Hgb dimers
appear in the plasma (hemoglobinemia) and are filtered through the kidneys and
reabsorbed by the renal tubular cells. The renal tubular uptake capacity is
approximately 5 g/day of filtered Hgb. Beyond this level, free Hgb appears in urine
(hemoglobinuria). Hemoglobinuria is associated with hemoglobinemia. - B Spherocytes are characteristic of AIHA and cause increased osmotic fragility. In
AIHAs, production of autoantibodies against one’s own RBCs causes hemolysis or
phagocytic destruction of RBCs. A positive direct antiglobulin (DAT or Coombs’) test
identifies in vivo antibody-coated and complement-coated RBCs. A positive DAT
result distinguishes AIHA from other types of hemolytic anemia that produce
spherocytes. - C In patients with G6PD deficiency, the RBCs are unable to reduce nicotinamide
adenine dinucleotide phosphate (NADP); consequently, Hgb is denatured, and Heinz
bodies are formed. “Bite cells” appear in the peripheral circulation as a result of splenic
pitting of Heinz bodies. - C RBC indices classify the anemia morphologically. Anemias can be classified
morphologically by using laboratory data; physiologically, based on the mechanism;
and clinically, based on an assessment of symptoms. - C Aplastic anemia has many causes, such as chemical, drug, or radiation poisoning;
congenital aplasia; and Fanconi syndrome. All result in depletion of hematopoietic
precursors of all cell lines, leading to peripheral blood pancytopenia. - A There are four classifications of CDAs, each characterized by ineffective
erythropoiesis, increased unconjugated bilirubin, and bizarre multinucleated erythroid
precursors. - D Microangiopathic hemolytic anemia is a condition resulting from shear stress to the
erythrocytes. Fibrin strands are laid down within the microcirculation, and RBCs
become fragmented as they contact fibrin through the circulation process, forming
schistocytes. - D Chloramphenicol is the drug most often implicated in acquired aplastic anemia.
About half the cases occur within 30 days after therapy, and about half are reversible.
Penicillin, tetracycline, and sulfonamides have been implicated in a small number of
cases. - A Sickle cell disorders are intracorpuscular RBC defects that are hereditary and result in
defective Hgbs being produced. The gene for sickle cell can be inherited either
homozygously or heterozygously. - C Spherocytes are produced in AIHA. Spherocytes may be produced by one of three
mechanisms. First, they are a natural morphological phase of normal RBC senescence.
Second, they are produced when the cell surface:volume ratio is decreased, as seen in
HS. And, third, they may be produced as a result of antibody coating of RBCs. As the
antibody-coated RBCs travel through the spleen, the antibodies and portions of the
RBC membrane are removed by macrophages. The membrane repairs itself; hence, the
RBC’s morphology changes from a biconcave disk to a spherocyte. - C This patient’s abnormal peripheral blood smear indicates marked RBC regeneration,
causing many reticulocytes to be released from bone marrow. Because reticulocytes
are larger than mature RBCs, MCV will be slightly elevated. - B As a result of splenectomy, Howell–Jolly bodies may be seen in great numbers. One
of the main functions of the spleen is pitting, which allows inclusions to be removed
from the RBC without destroying the cell membrane. - D Reticulocytes are polychromatophilic macrocytes, and the presence of reticulocytes
indicates RBC regeneration. Bone marrow’s appropriate response to anemia is to
deliver RBCs prematurely to the peripheral circulation. In this way, reticulocytes and
possibly NRBCs may be seen in the peripheral blood smear. - C HP is a membrane defect characterized by a spectrin abnormality and thermal
instability. MCV is decreased, and RBCs appear to be budding and fragmented.
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