Paroxysmal nocturnal hemoglobinuria

Diseases are easy to occur, and there are many types of diseases. In the treatment of diseases, the choice of methods is critical. Different diseases have different treatment methods. Therefore, when treating diseases, you must first understand the disease, so that you will know what treatment method to choose. This is also very helpful for the treatment of diseases. What is paroxysmal nocturnal hemoglobinuria?

Many people don't know much about paroxysmal nocturnal hemoglobinuria, so when treating this disease, you must understand its symptoms so that you will know what method to choose when treating your own disease.

Paroxysmal nocturnal hemoglobinuria:

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired benign Crohn's disease caused by mutations in the PIG-A gene of hematopoietic stem cells. The first case reported by Paul Stǔbing in 1882 was hemoglobinuria occurring at night, so it was named "paroxysmal nocturnal hemoglobinuria" (PNH). However, it was later discovered that the onset of hemoglobinuria does not necessarily occur at night, but often after sleep, so it was renamed "paroxysmal nocturnal hemoglobinuria" in my country. 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, in recent years, some authors have regarded PNH as a hematopoietic stem cell disease.

Symptoms and signs

1. Anemia. The vast majority of patients have varying degrees of anemia, which is often moderate or severe. Since anemia usually occurs slowly, patients often have better adaptability, so they can still move around and even work even though their hemoglobin is low. In addition, due to long-term intravascular hemolysis, hemosiderin is deposited in the skin, so the face and skin are often dark brown.

2. Hemoglobinuria. Typical hemoglobinuria is soy sauce or strong tea color. It usually lasts for 2 to 3 days and disappears on its own without treatment. In severe cases, it can last for 1 to 2 weeks or even longer. Some patients have frequent attacks of hemoglobinuria, some have occasional attacks or once every few months, and some patients have urine that is not dark in color but have persistently positive urine occult blood tests. About 25% of patients never have an attack during a long course of illness or observation period.

Factors that can cause hemoglobinuria include colds or other infections, blood transfusions, iron supplements, fatigue, etc. When hemoglobinuria occurs, symptoms such as chills, fever, back pain, and abdominal pain may occur. There is no good explanation as to why some patients experience worsening hemoglobinuria during sleep. Some people have suggested that this may be due to the absorption of more bacterial lipopolysaccharide through the intestines during sleep, which activates complement. Wang Yuzhou's observation in 2000 showed that there was no obvious change in blood pH value during sleep in PNH patients, and the complement 3 activation product C3d increased slightly, which was the same as normal people.

Through the above introduction, we have a good understanding of the symptoms of paroxysmal nocturnal hemoglobinuria. Therefore, when treating such a disease, we must choose a treatment method that suits us. At the same time, when treating this type of disease, we must pay attention to the fact that it may take longer, so we should not be too anxious during treatment.