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.