The clinical manifestations of paroxysmal sleep hemoglobinuria include fatigue, low back pain, carnal hemoglobinuria, thrombosis, and renal impairment.
Paroxysmal sleep hemoglobinuria is due to the human PIG-A gene mutation, resulting in the acquired deficiency of cell membrane anchor chain protein (CD55, CD59 deficiency), the activation of complement can not be inhibited, which leads to intravascular hemolysis, thrombosis and other manifestations, and in severe cases, it can lead to renal function impairment.
1. When intravascular hemolysis occurs in patients with paroxysmal sleep hemoglobinuria, symptoms of hemolytic anemia, such as fatigue, dizziness, low back pain, etc., increased hemoglobin in urine, urine can be red wine-like or soy sauce-like changes, and hemoglobinuria is more obvious after sleep.
2. After intravascular hemolysis occurs in patients with this disease, red blood cells release pro-coagulant substances and complement to act on the platelet membrane at the same time, thus promoting platelet aggregation and increasing the risk of thrombosis. Some patients have different degrees of renal function impairment, manifested by varying degrees of creatinine elevation, caused by microthrombosis and repeated hemolysis leading to renal tubular damage.
It is recommended that the patient should consult the hematology department in time, complete the relevant examinations to assess the severity of the disease, and have the specialist formulate an individualized treatment plan according to the patient’s condition.