Paroxysmal nocturnal hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria
Pathology
Anemias
Autoimmune hemolytic anemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hemolytic disease of the newborn
Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
Pyruvate kinase deficiency
Sickle cell disease (NORD)
Fanconi anemia
Folate (Vitamin B9) deficiency
Megaloblastic anemia
Vitamin B12 deficiency
Alpha-thalassemia
Anemia of chronic disease
Beta-thalassemia
Iron deficiency anemia
Lead poisoning
Sideroblastic anemia
Anemia of chronic disease
Aplastic anemia
Diamond-Blackfan anemia
Fanconi anemia
Dysplastic and proliferative disorders
Langerhans cell histiocytosis
Mastocytosis (NORD)
Essential thrombocythemia (NORD)
Myelodysplastic syndromes
Myelofibrosis (NORD)
Polycythemia vera (NORD)
Leukemoid reaction
Leukemoid reaction
Mixed platelet and coagulation disorders
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Von Willebrand disease
Thrombosis syndromes (hypercoagulability)
Antiphospholipid syndrome
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Hematological system pathology review
Coagulation disorders: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Leukemias: Pathology review
Lymphomas: Pathology review
Macrocytic anemia: Pathology review
Microcytic anemia: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Myeloproliferative disorders: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Plasma cell disorders: Pathology review
Platelet disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Assessments
Paroxysmal nocturnal hemoglobinuria
Flashcards
0 / 15 complete
USMLE® Step 1 questions
0 / 2 complete
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?
External References
First Aid
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Eculizumab p. 120
for paroxysmal nocturnal hemoglobinuria p. 417
Pancytopenia p. 429
paroxysmal nocturnal hemoglobinuria p. 417
Paroxysmal nocturnal hemoglobinuria p. 417
in anemia taxonomy p. 425
CD53 deficiency p. 105
eculizumab for p. 120
flow cytometry diagnosis p. 52
intravascular hemolysis in p. 429
presentation p. 718
Venous thrombosis p. 417
paroxysmal nocturnal hemoglobinuria p. 417
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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