Paroxysmal nocturnal hemoglobinuria

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Flashcards

Paroxysmal nocturnal hemoglobinuria

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Questions

USMLE® Step 1 style questions USMLE

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A 33-year-old man comes to the primary care office because of fatigue, shortness of breath, pallor, and increased respiratory infections for the past six months. Temperature is 37.3°C (99.1°F), pulse is 86/min, respirations are 20/min, and blood pressure is 108/68 mmHg. Physical examination shows scleral icterus. Laboratory tests are obtained, and the results are shown below.
Acidified serum lysis test is positive. Which of the following is a potential complication of this patient’s disease?

External References

First Aid

2024

2023

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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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