PNH Study Guide – Questions With Solutions
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Practice questions for this set
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End result of hemolysis-induced NO depletion
Choose an answer
1 PNH stands for? 2 PHT cause in PNH
Which bone marrow disorder causes the
3 PNH results from... 4
highest risk for PNH?
Don't know?
Terms in this set (110)
PNH stands for? Paroxysmal Nocturnal Hemoglobinuria
Is PNH paroxysmal? NO - ongoing hemolysis
Is PNH Nocturnal? NO - Subtle and constant
Does PNH develop on its own or does it Both - arises with another bone marrow disorder
arise in the presence of another disorder?
Which bone marrow disorder causes the Aplastic anemia
highest risk for PNH?
% of AA pts. that have PNH clones? 26%
Why the correlation between AA and PNH? AA creates an environment that promotes the survival of
stems cells with PIG-A
, Are AA and PNH the same disease? NO - separate disease states requiring separate treatments
Demographics - Age Prime of life - early 30s
Demographics - gender Affects equally
Incidence 1.3/million per year
Prevalence 15.9/million over 15 years
PNH results from... Somatic Mutation on the X-linked PIG-A gene
Loss of PIG-A results in... Lack of GPI anchors
Lack of GPI anchors results in... Loss of cell surface proteins
CD55 DAF - Decay accelerating factor
Inhibits the complement cascade at C3 and C5 convertase
steps
CD59 MIRL - Membrane inhibitor of reactive lysis
Blocks the formation of MAC on cell surface
PNH clonal expansion -Immunologic selection is not the sole contributor to clonal
expansion
-Additional genetic abnormalities may cause the PNH clones
to develop a growth pattern similar to that of benign tumors
Hemoglobin and NO -Hemoglobin is released during hemolysis
-Free Hb binds to NO with high affinity
Consequence of lack of NO -NO is an important regulator of vascular activity
-Maintains normal vessel wall tone and limits platelet
activation
-Inhibits platelet activation and aggregation
Most notable consequence of PNH Thrombosis
What is a PNH clone? -A blood cell derived from a hematopoietic stem cell with the
PIG-A mutation that lacks GPI anchors
Median survival rate of PNH patient? 10 years
What is the 5 year death rate after 35%
diagnosis? (%)
2 leading causes of death in PNH? 1) Thrombosis - majority
2) Renal failure
PNH thrombosis deaths? (%) 40-67%
Where does thrombosis occur in PNH? Any site
Why are anticoagulants ineffective in PNH? It does not address the underlying cause
% thrombosis in anticoagulant patients? 46%
Save
Students also studied
WSC 2026: Are we there yet? Social Science Nationalism Vocab T... WSC 2026 Next Year
Teacher 156 terms 21 terms Teacher 50 terms
Amy_Woodall4 Preview Keiya_Raja Preview Sandywu1205
Practice questions for this set
Learn 1 /7 Study using Learn
End result of hemolysis-induced NO depletion
Choose an answer
1 PNH stands for? 2 PHT cause in PNH
Which bone marrow disorder causes the
3 PNH results from... 4
highest risk for PNH?
Don't know?
Terms in this set (110)
PNH stands for? Paroxysmal Nocturnal Hemoglobinuria
Is PNH paroxysmal? NO - ongoing hemolysis
Is PNH Nocturnal? NO - Subtle and constant
Does PNH develop on its own or does it Both - arises with another bone marrow disorder
arise in the presence of another disorder?
Which bone marrow disorder causes the Aplastic anemia
highest risk for PNH?
% of AA pts. that have PNH clones? 26%
Why the correlation between AA and PNH? AA creates an environment that promotes the survival of
stems cells with PIG-A
, Are AA and PNH the same disease? NO - separate disease states requiring separate treatments
Demographics - Age Prime of life - early 30s
Demographics - gender Affects equally
Incidence 1.3/million per year
Prevalence 15.9/million over 15 years
PNH results from... Somatic Mutation on the X-linked PIG-A gene
Loss of PIG-A results in... Lack of GPI anchors
Lack of GPI anchors results in... Loss of cell surface proteins
CD55 DAF - Decay accelerating factor
Inhibits the complement cascade at C3 and C5 convertase
steps
CD59 MIRL - Membrane inhibitor of reactive lysis
Blocks the formation of MAC on cell surface
PNH clonal expansion -Immunologic selection is not the sole contributor to clonal
expansion
-Additional genetic abnormalities may cause the PNH clones
to develop a growth pattern similar to that of benign tumors
Hemoglobin and NO -Hemoglobin is released during hemolysis
-Free Hb binds to NO with high affinity
Consequence of lack of NO -NO is an important regulator of vascular activity
-Maintains normal vessel wall tone and limits platelet
activation
-Inhibits platelet activation and aggregation
Most notable consequence of PNH Thrombosis
What is a PNH clone? -A blood cell derived from a hematopoietic stem cell with the
PIG-A mutation that lacks GPI anchors
Median survival rate of PNH patient? 10 years
What is the 5 year death rate after 35%
diagnosis? (%)
2 leading causes of death in PNH? 1) Thrombosis - majority
2) Renal failure
PNH thrombosis deaths? (%) 40-67%
Where does thrombosis occur in PNH? Any site
Why are anticoagulants ineffective in PNH? It does not address the underlying cause
% thrombosis in anticoagulant patients? 46%
