2023年7月31日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that presents clinically with a variety of nonspecific symptoms. It causes complications mainly through intravascular hemolysis, thrombosis, and bone marrow failure.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, [1] life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system.

Paroxysmal nocturnal hemoglobinuria (PNH), first described in the 18th century, is characterized by the typical clinical triad of hemolytic anemia, bone marrow failure, and propensity to thromboembolism (Rotoli and Luzzatto 1989; Dunn et al 2000; Rotoli et al 2006; Hill et al 2007a).

When discussing Paroxysmal Nocturnal Hemoglobinuria (PNH), the “classic triad” refers to a set of three key clinical manifestations that have historically been associated with the disease. It’s important to note that while these three features are significant, not every paroxysmal nocturnal hemoglobinuria (PNH) patient will present with ...

2024年6月25日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell (HSC) disease. It is a rare, chronic, debilitating disorder that most frequently presents in early...

2017年2月2日 · Paroxysmal nocturnal hemoglobinuria is a rare, life-threatening, genetic hematological disorder characterized by hemolysis of the red blood cells. The classic manifestation of PNH is dark urine during the night with partial clearing during the day. 7 Its mostly present with hemolytic anemia usually in the form of intravascular hemolysis.

PNH is characterized by the triad of acquired corpuscular hemolysis, thrombophilia, and bone marrow failure. The intravascular destruction of PNH red blood cells causes chronic, often disabling symptoms of fatigue and intermittent episodes of dysphagia, abdominal pain, and hemoglobinuria.

2008年1月1日 · Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia caused by the expansion of a hematopoietic progenitor cell that has acquired a mutation in the X-linked PIGA gene. PNH occurs on the background of bone marrow failure.

2025年1月2日 · The classic triad of PNH is hemolytic anemia, marrow failure, and thrombophilia. Patients may be treated with the monoclonal antibody eculizumab or with stem cell transplantation, in addition to being treated for associated complications. Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza.

2021年11月18日 · Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the triad of intravascular hemolysis (IVH), a tendency to thrombosis, and an element of bone marrow failure.