Paraoxysmal Nocturnal Hemoglobinuria

Paroxysmal nocturnal hemoglobinuria is an acquired disease that leads to premature death and impaired blood cell production. The disease affects red blood cells (erythrocytes) transferring oxygen; white blood cells (leukocytes) protecting an organism from an infection, and platelets (platelets) involved in blood coagulation. Paroxysmal nocturnal hemoglobinuria equally affects both sexes and can occur at any age, although it is most often diagnosed in young adulthood. The disease develops slowly and a person may not immediately notice symptoms of pathology. A qualified specialist will conduct the necessary research and select the optimal therapy scheme.

It is important to contact the haematologist at the first signs, such as unnatural paleness of the skin. In the early stages, complications can be identified with such advanced research technologies and modern equipment as the Israeli clinic Tel Aviv Medical Clinic. Our doctors have considerable experience in prescribing the optimal course of therapy to patients even with a neglected form of leukaemia. They manage to achieve remission, and in many cases, the recovery of the patient without the threat of relapse.

Symptoms of the disease

If you notice the occurrence of the following signs of the disease, you should immediately consult a doctor:

• Pale skin.
• Slight temperature rise.
• Malaise and weakness.
• Frequent infectious diseases due to loss of body protective immunity.
• Increased bleeding.
• Severity in the underbelly.
• Itching.
• Strong night sweating.
• Pathological enlargement of liver and spleen is observed.
• Urine colour change.

A qualified specialist will conduct the necessary examination and select the optimal therapy scheme taking into account the characteristics of the development of the disease and the general condition of the patient.

Treatment of the disease

Treatment of paroxysmal nocturnal hemoglobinuria is symptomatic as no specific therapy exists. The main method of therapy for patients with paroxysmal nocturnal hemoglobinuria is transfusion of washed (at least 5 times) or thawed red blood cells, which, as a rule, are well tolerated by patients for a long time and do not cause is sensitization. Transfusions of freshly prepared whole blood or red blood cell mass with a shelf life of fewer than 7 days are contraindicated due to the possibility of increasing hemolysis, developing hemoglobinuria crises due to the presence of leukocytes in these transfusion media, which leads to the formation of anti-leukocyte antibodies and complement activation.