Paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria
Sangre y sistema linforreticular
Anemia, citopenias y anemias por policitemia
Trastornos de la coagulación (condiciones hipercoaguables e hipocoagulables)
Trastornos infecciosos e inmunológicos
Neoplasias
Trastornos traumáticos, mecánicos y vasculares
Revisión de la patología sanguínea y del sistema linfarreticular
Flashcards
Paroxysmal nocturnal hemoglobinuria
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Preguntas
Preguntas del estilo USMLE® Step 1
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| Laboratory value | Result | Reference Range |
| Hematologic | ||
| Hemoglobin | 8.3 g/dL | 13.5-17.5 g/dL |
| Mean corpuscular volume (MCV) | 84 fL | 80-100 fL |
| Platelet count | 90,000/mm3 | 150,000-400,000/mm3 |
| Leukocyte count | 2,300/mm3 | 4,500-11,000/mm3 |
Acidified serum lysis test is positive. Which of the following is a potential complication of this patient’s disease?
Aspectos destacados
en inglés
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.