Paroxysmal nocturnal hemoglobinuria
High Yield Notes
13 pages
Flashcards
Paroxysmal nocturnal hemoglobinuria
of complete
Questions
USMLE® Step 1 style questions USMLE
of complete
Acidified serum lysis test is positive. Which of the following is a potential complication of this patient’s disease?
Memory Anchors and Partner Content
External References
First Aid
2024
2023
2022
2021
Eculizumab p. 120
for paroxysmal nocturnal hemoglobinuria p. 415
Pancytopenia p. 427
paroxysmal nocturnal hemoglobinuria p. 415
Paroxysmal nocturnal hemoglobinuria p. 415
in anemia taxonomy p. 423
CD53 deficiency p. 105
eculizumab for p. 120
flow cytometry diagnosis p. 52
intravascular hemolysis in p. 427
presentation p. 724
Venous thrombosis p. 415
paroxysmal nocturnal hemoglobinuria p. 415
Summary
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening condition characterized by the destruction of red blood cells by the complement portion of the immune system.
This destructive process is a result of a defect in the formation of surface proteins on the red blood cell, which normally functions to inhibit such immune reactions. Common symptoms of PNH are fatigue due to anemia, abdominal pain, and thrombosis, which is the major cause of death. There can also be jaundice from the accumulation of bilirubin, and characteristic dark-colored urine.
The management of PNH involves drugs like eculizumab, a monoclonal antibody that blocks the activity of the complement system, anticoagulation therapy in case of thrombosis, blood transfusion, and supportive management as needed.
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