Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hemolytic anemia caused by somatic mutations in the phosphatidylinositol glycan-complementation class A gene and the resulting absence of a key complement regulatory protein, CD59. Affected red blood cells in patients with PNH undergo int
Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome
✍ Scribed by A. C. Hauser; A. Brichta; I. Pabinger-Fasching; U. Jäger
- Publisher
- Springer
- Year
- 2003
- Tongue
- English
- Weight
- 168 KB
- Volume
- 82
- Category
- Article
- ISSN
- 0939-5555
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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic hemolytic anemia associated with an unusual susceptibility to hemolytic crisis, infection, and venous thrombosis which would be aggravated by a number of factors including surgery. We report a case of PNH undergoing percutaneous transl
A patient with paroxysmal nocturnal hemoglobinuria developed lactic acidosis associated with severe anemia. The lactic acidosis corrected after blood transfusion. In the absence of shock, sepsis, or other identifiable causes of lactic acidosis, the severe anemia (hemoglobin 1.2 g/dl) appeared to be
Budd-Chiari syndrome (BCS) is the end result of a number of disease states resulting in hepatic venous outflow obstruction. We report a Janus kinase 2-homozygous patient with BCS who thrombosed a transjugular intrahepatic portosystemic shunt (TIPS) despite treatment with warfarin (international norm