Heme 1.2 Normocytic and Normochromic Anemias Flashcards
- Hypersplenism is characterized by:
A. Polycythemia
B. Pancytosis
C. Leukopenia
D. Myelodysplasia
C. Leukopenia
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.
- Which of the following organs is responsible for the “pitting process” in RBCs?
A. Liver
B. Spleen
C. Kidney
D. Lymph nodes
B. Spleen
- 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
D. Increased deformability
- Which of the following is not associated with HS?
A. Increased osmotic fragility
B. MCHC greater than 36%
C. Intravascular hemolysis
D. Extravascular hemolysis
C. Intravascular hemolysis
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
- 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)
D. Hereditary spherocytosis (HS)
Spherocytic cells have decreased tolerance to swelling and, therefore, hemolyse at a higher concentration of sodium salt compared with normal RBCs.
- The anemia seen in sickle cell disease is usually:
A. Microcytic, normochromic
B. Microcytic, hypochromic
C. Normocytic, normochromic
D. Normocytic, hypochromic
C. Normocytic, normochromic
- 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
D. Hgb A
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.
- 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
- 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%
- 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.
- 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 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.
- 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
- 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
- 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
There are 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.
