Paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic intravascular hemolysis caused by acquired red blood cell membrane defects. It often worsens during sleep and may be accompanied by paroxysmal hemoglobinuria and pancytopenia. Although this disease is rare, the incidence has been increasing in recent years. It is more common in the north of my country than in the south. More than half of the cases occur in young and middle-aged people aged 20-40, and some are under 10 years old and over 70 years old. There are more men than women. PNH has always been classified as a hemolytic disease, but in addition to anemia, it is often accompanied by a decrease in neutrophils and (or) platelets, and the molecular lesions of PNH involve various blood cells. Therefore, some authors in recent years regard PNH as a hematopoietic stem cell disease. The main cause of death in China is infection and in foreign countries is vascular embolism.
-
Site of disease:
kidney -
Infectious :
Not contagious -
Frequent population:
The peak age of onset is between 20 and 40 years old... -
Related symptoms:
Fatigue palpitations nosebleed loss of appetite dizziness -
Concurrent disease:
Related Drugs
Nephritis recovery tablets
Manufacturer:
Tianjin Tongrentang Group Co., Ltd.
EMPAVELI- pegcetacoplan_injection, solution
Indication: EMPAVELI registered EMPAVELI is a complement inhibitor indicated for the treatment of adult patients with paroxysmal nocturnal hemoglobinuria (PNH).
Manufacturer:
Apellis Pharmaceuticals, Inc.
EPYSQLI- eculizumab-aagh_injection, solution
Indication: EPYSQLI is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. 1 the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. 2 Limitation of Use the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive. 3 EPYSQLI is indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. EPYSQLI is indicated for the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). EPYSQLI is indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive.
Manufacturer:
Samsung Bioepis Co., Ltd.
EPYSQLI- eculizumab-aagh_injection, solution
Indication: EPYSQLI is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. 1 the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. 2 Limitation of Use the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive. 3 EPYSQLI is indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. EPYSQLI is indicated for the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). EPYSQLI is indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive.
Manufacturer:
Samsung Bioepis Co., Ltd.
EMPAVELI- pegcetacoplan_injection, solution
Indication: EMPAVELI registered EMPAVELI is a complement inhibitor indicated for the treatment of adult patients with paroxysmal nocturnal hemoglobinuria (PNH).
Manufacturer:
Apellis Pharmaceuticals, Inc.