Pnh - High Yield Topic Series
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Single answer only !
- 1.
Pancytopenia with cellular marrow is seen in
- A.
G6PD deficiency
- B.
PNH
- C.
Acquired aplastic anemia
- D.
Thalassemia
Correct Answer
B. PNHExplanation
Pancytopenia with cellular marrow is seen in PNH (Paroxysmal Nocturnal Hemoglobinuria). PNH is a rare acquired genetic disorder characterized by the destruction of red blood cells, white blood cells, and platelets. This leads to a decrease in all three cell types in the blood, resulting in pancytopenia. The bone marrow, however, shows normal or increased cellularity, indicating that the problem lies in the destruction of cells rather than a decrease in production.Rate this question:
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- 2.
The Diagnostic test for PNH
- A.
Sugar Water Test
- B.
Hams test
- C.
FISH
- D.
Flowcytometry
Correct Answer
D. FlowcytometryExplanation
Flow cytometry is a diagnostic test for PNH (Paroxysmal Nocturnal Hemoglobinuria). It is a laboratory technique that uses laser technology to analyze and measure the physical and chemical characteristics of cells in a fluid sample. In the case of PNH, flow cytometry is used to detect and quantify the presence of abnormal red blood cells that lack certain proteins on their surface, which is a characteristic feature of PNH. This test is highly sensitive and specific, making it a reliable method for diagnosing PNH. Other tests mentioned, such as the Sugar Water Test, Hams test, and FISH, may have different applications but are not specifically used for diagnosing PNH.Rate this question:
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- 3.
PNH patients are at increased risk of developing
- A.
AML
- B.
ALL
- C.
CML
- D.
CLL
Correct Answer
A. AMLExplanation
Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) have an increased risk of developing Acute Myeloid Leukemia (AML). PNH is a rare blood disorder characterized by the destruction of red blood cells, leading to symptoms such as anemia, fatigue, and blood clots. PNH is caused by a mutation in the PIG-A gene, which results in the deficiency of certain proteins on the surface of blood cells. This deficiency increases the risk of abnormal cell growth and the development of AML, a type of cancer that affects the bone marrow and blood. Therefore, AML is the correct answer.Rate this question:
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- 4.
Gene defective in PNH is
- A.
GIGA
- B.
PIGA
- C.
GPIA
- D.
PGIA
Correct Answer
B. PIGAExplanation
phosphatidylinositol glycan A (PIGA) is needed to make glycosylphosphatidylinositol (GPI) anchor.Rate this question:
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- 5.
Decay-accelerating factor (DAF) is
- A.
CD55
- B.
CD59
- C.
CD5b
- D.
CD35
Correct Answer
A. CD55Explanation
CD55 is the correct answer because Decay-accelerating factor (DAF) is also known as CD55. CD55 is a protein that is found on the surface of cells and helps to protect them from being attacked by the body's immune system. It does this by preventing the complement system, which is part of the immune system, from damaging the cells. CD59, CD5b, and CD35 are not alternative names for Decay-accelerating factor (DAF).Rate this question:
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- 6.
Mode of Inheritance of PNH is
- A.
Autosomal Dominant
- B.
Autosomal recessive
- C.
X- linked recessive
- D.
X- linked dominant
- E.
None
Correct Answer
E. NoneExplanation
PNH is an ACQUIRED STEM CELL DISORDER !!Rate this question:
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- 7.
PNH causes Extra vascular Hemolysis
- A.
True
- B.
False
Correct Answer
B. FalseExplanation
PNH causes INTRA vascular HemolysisRate this question:
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- 8.
FALSE regarding PNH
- A.
Thrombotic episodes
- B.
Impotence
- C.
Coomb's test positive
- D.
Eculizumab is used for treatment
- E.
Refractory anemia/Aplastic anemia
Correct Answer
C. Coomb's test positiveExplanation
The correct answer is "coomb's test positive". This means that in the case of PNH, the Coombs test, which is used to detect antibodies against red blood cells, is positive. This indicates that there is an immune-mediated destruction of red blood cells in PNH. The other statements mentioned, such as thrombotic episodes, impotence, eculizumab treatment, and refractory/aplastic anemia, are not associated with PNH.Rate this question:
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- 9.
Eculizumab is a monoclonal antibody directed against
- A.
C5
- B.
C6
- C.
C7
- D.
C3b
Correct Answer
A. C5Explanation
Eculizumab is a monoclonal antibody that specifically targets the C5 protein. By binding to C5, eculizumab prevents its cleavage into C5a and C5b, which are important components of the complement system. This inhibition of C5 prevents the formation of the membrane attack complex (MAC) and the release of pro-inflammatory mediators, ultimately reducing the destruction of red blood cells in conditions such as paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS).Rate this question:
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- 10.
Lab findings of PNH are all of the following, EXCEPT
- A.
Increased reticulocyte count
- B.
Increased LDH
- C.
Increased serum haptoglobin
- D.
Decreased serum haptoglobin
Correct Answer
C. Increased serum haptoglobinExplanation
These are the features of all Hemolytic anemiasRate this question:
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Jul 22, 2024Quiz Edited by
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May 10, 2012Quiz Created by
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