1.2 Normocytic and Normochromic Anemias Flashcards
Hypersplenism is characterized by:
A. Polycythemia
B. Pancytosis
C. Leukopenia
D. Myelodysplasia
- C. Leukopenia
Hypersplenism: (1) cytopenia one or more peripheral cell lines, (2) BM hyperplasia, (3) splenomegaly; (4) resolution of cytoprnia by splenectomy
Which of the following organs is responsible for the pitting process?
A. Liver
B. Spleen
C. Kidney
D. Lymph nodes
- B. Spleen
Spleen: supreme filter of the body, pitting imperfections from RBC w/o destroying the integrity of the membrane
Spherocytes differ from normal RBCs in all of the following, except:
A. Decreased sufrace to volume ration
B. No central pallor
C. Decreased resistance to hypotonic solution
D. Increased deformability
- D. Increased deformability
spherocytes lose deformability d/t a defect in spectrin
spectrin: membrane protein, w/o = prone to sequestration & hemolysis
Which of the following is not associated with HS?
A. Increased OFT
B. MCHC >36%
C. Intravascular hemolysis
D. Extravascular hemolysis
- C. Intravascular hemolysis
IV hemolysis: hemoglobinemia, hemoglobinuria, hemosiderinuria, ( NO HS)
HS: extravascular process rather than IV process
Which of the following disorders has an increase in OFT?
A. IDA
B. Hereditary elliptocytosis
C. Hereditary stomatocytosis
D. HS
- C. HS
spherocytes: dec. tolerance to swelling, hemolyse at a HIGHER conc NaCl
The anemia seen in sickle cell disease is usually:
A. Microcytic, normochromic
B. Microcytic, hypochromic
C. Normocytic, normochromic
D. Normocytic, hypochromic
- C. Normocytic, normochromic
Sickle cell disease: a chronic hemolytic anemia classified as normocytic, normochromic
What 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
- D. Hgb A
Sickle Cell Trait Hgb: Hgb A (50-70%), Hgb S (20-40%), Hgb F & A2 (normal conc)
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
- B. Valine is substituted for glutamic acid at the sixth position of the β-chain
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.
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
- D. Fast mobility of Hgb C at pH 8.6
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.
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
- C. Hgb 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.
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%
- B. Hgb A: 60% Hgb S: 40% Hgb A2 : 2%
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.
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
- D. Sickle cell disease
Autosplenectomy occurs in sickle cell anemia as a result of repeated infarcts to the spleen caused by an overwhelming sickling phenomenon.
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
- A. It is a rare acquired stem cell disorder that results in hemolysis
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.
Hemolytic uremic syndrome (HUS) is characterized by all of the following except:
A. Hemorrhage
B. Thrombocytopenia
C. Hemoglobinuria
D. Reticulocytopenia
- D. Reticulocytopenia
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.
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
- D. PNH
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.
Which antibody is associated with paroxysmal cold hemoglobinuria (PCH)?
A. Anti-I
B. Anti-i
C. Anti-M
D. Anti-P
- D. Anti-P
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.
All of the following are associated with intravascular hemolysis except:
A. Methemoglobinemia
B. Hemoglobinuria
C. Hemoglobinemia
D. Decreased haptoglobin
- A. Methemoglobinemia
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.
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
- B. Spherocytic RBCs
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.
“Bite cells” are usually seen in patients with:
A. Rh null trait
B. Chronic granulomatous disease
C. G6PD deficiency
D. PK deficiency
- C. G6PD deficiency
In patients with G6PD deficiency, the RBCs are unable to reduce 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.
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
- C. RBC indices
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.
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
- C. Peripheral blood pancytopenia
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.
Congenital dyserythropoietic anemias (CDAs) are characterized by:
A. Bizarre multinucleated erythroblasts
B. Cytogenetic disorders
C. Megaloblastic erythropoiesis
D. An elevated M:E ratio
- A. Bizarre multinucleated erythroblasts
four classifications of CDAs, each characterized by ineffective erythropoiesis, increased unconjugated bilirubin, and bizarre multinucleated erythroid precursors.
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
- D. Schistocytes and NRBCs
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.
Which antibiotic(s) is (are) most often implicated in the development of aplastic anemia?
A. Sulfonamides
B. Penicillin
C. Tetracycline
D. Chloramphenicol
- D. Chloramphenicol
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.
Sickle cell disorders are:
A. Hereditary, intracorpuscular RBC defects
B. Hereditary, extracorpuscular RBC defects
C. Acquired, intracorpuscular RBC defects
D. Acquired, extracorpuscular RBC defects
- A. Hereditary, intracorpuscular RBC defects
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.
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
- C. AIHA
Spherocytes are produced in AIHA. Spherocytes may be produced by one of three mechanisms.
1. a natural morphological phase of normal RBC senescence
2. decreased cell surface:volume ratio
3. result of antibody coating of RBCs; 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.
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
- C. Increased MCV
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.
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
- B. Howell–Jolly bodies
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.
Reticulocytosis usually indicates:
A. Response to inflammation
B. Neoplastic process
C. Aplastic anemia
D. RBC regeneration
- D. RBC regeneration
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.
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
- C. Misshapen budding fragmented cells
HP is a membrane defect characterized by a spectrin abnormality and thermal instability. MCV is decreased, and RBCs appear to be budding and fragmented.
