Hemoglobinuria Paroxysmal Nocturnal

Hemoglobinuria Paroxysmal Nocturnal: symptoms, causes and treatment

Hemoglobinuria Paroxysmal Nocturnal (PHN) is a rare hematological disease characterized by the destruction of red blood cells and the appearance of hemoglobin in the urine. This disease is also known as Harley's disease or Paroxysmal nocturnal hemoglobinuria.

With FPG, complement activation occurs, which leads to the destruction of red blood cells and the release of hemoglobin into the blood. This can happen at any time of the day or night, but most often occurs at night when the person is at rest. Symptoms of FPG may include paleness, fatigue, difficulty breathing, dark urine, and abdominal pain.

The causes of FPG are not fully understood, but the disease is known to be associated with mutations in the PIG-A gene, which encodes a protein needed to create a protective coating on the surface of blood cells. As a result, red blood cells do not receive this protection and become susceptible to destruction.

Treatment for FPG may include blood transfusions to replace destroyed red blood cells, as well as immunotherapy, which aims to suppress complement activation. Some patients may require a bone marrow transplant to correct a genetic defect.

Although FPG is a rare disease, its diagnosis and treatment can be complex and require a specialized approach. If you notice symptoms of FPG, contact your hematologist for further advice and treatment.

**Paroxysmal nocturnal hemoglobinuria** (HPN) is a rare genetic disease characterized by nocturnal attacks of severe abdominal pain, accompanied by the appearance of blood in the urine and profuse hematuric diarrhea. The type of inheritance of the disease is usually autosomal recessive. In some cases, bleeding can occur quite easily, while others are accompanied by critical losses of hemoglobin and hemoglobinuria, leading to hemorrhagic shock. Today, GPN is one of the 25 most severe disorders associated with decreased blood clotting and the likelihood of developing bleeding wounds due to external damage to the skin. Patient management tactics and treatment are decided after a full examination in the hospital, taking into account the identified forms of the disease.\n

Diagnosis is made after symptoms appear. Blood can only be detected in dark sediment in quantities ranging from a few drops to one milliliter. Blood in urine sediment may be present in chronic pyelonephritis and cystine crises due to the presence of hyaline casts and admixtures of fresh blood, so it is considered only in the case of good hemocoelomia of patients. A biop is taken for histology