Paroxysmal nocturnal hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria
Hematological system
Anemias
Iron deficiency anemia
Beta-thalassemia
Alpha-thalassemia
Sideroblastic anemia
Anemia of chronic disease
Lead poisoning
Hemolytic disease of the newborn
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Autoimmune hemolytic anemia
Pyruvate kinase deficiency
Paroxysmal nocturnal hemoglobinuria
Sickle cell disease (NORD)
Hereditary spherocytosis
Anemia of chronic disease
Aplastic anemia
Fanconi anemia
Megaloblastic anemia
Folate (Vitamin B9) deficiency
Vitamin B12 deficiency
Fanconi anemia
Diamond-Blackfan anemia
Heme synthesis disorders
Acute intermittent porphyria
Porphyria cutanea tarda
Lead poisoning
Coagulation disorders
Hemophilia
Vitamin K deficiency
Platelet disorders
Bernard-Soulier syndrome
Glanzmann's thrombasthenia
Hemolytic-uremic syndrome
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Mixed platelet and coagulation disorders
Von Willebrand disease
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Thrombosis syndromes (hypercoagulability)
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Antiphospholipid syndrome
Lymphomas
Hodgkin lymphoma
Non-Hodgkin lymphoma
Leukemias
Chronic leukemia
Acute leukemia
Leukemoid reaction
Leukemoid reaction
Dysplastic and proliferative disorders
Myelodysplastic syndromes
Polycythemia vera (NORD)
Myelofibrosis (NORD)
Essential thrombocythemia (NORD)
Langerhans cell histiocytosis
Mastocytosis (NORD)
Plasma cell dyscrasias
Multiple myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom macroglobulinemia
Hematological system pathology review
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Coagulation disorders: Pathology review
Platelet disorders: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Lymphomas: Pathology review
Leukemias: Pathology review
Plasma cell disorders: Pathology review
Myeloproliferative disorders: Pathology review
Assessments
Paroxysmal nocturnal hemoglobinuria
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USMLE® Step 1 questions
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High Yield Notes
13 pages
Flashcards
Paroxysmal nocturnal hemoglobinuria
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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?
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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
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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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