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Paroxysmal nocturnal hemoglobinuria (PNH) is a serious blood disease in which patients experience the destruction of red blood cells and the release of hemoglobin in the urine at night.1. Diagnosis of paroxysmal nocturnal hemoglobinuria
1.1. Clinical symptoms
Urine: Dark color due to the presence of hemoglobin (about 25% in this case). Thrombosis: Venous thrombosis in places such as: intra-abdominal veins, cerebral veins and pulmonary infarction. Anemia, reduced reticulocytes, possibly decreased white blood cells and platelets. Esophageal contractility Erectile dysfunction Liver and spleen enlargement: uncommon
Bệnh tiểu huyết sắc tố kịch phát về đêm gây thiếu máu ở người bệnh
1.2. Test
Ham Dacie test and positive sugar test (low specificity) Flow cytometry: CD55 and CD59 deficiency in peripheral red blood cells (this is the basis for dividing the disease). Deficiency of CD55, CD59 in granulocytes: >50% risk of thrombosis. In addition, there may be a deficiency of CD16, CD59 in granulocytes and CD14, CD24 in monocytes
1.3.Determined diagnosis
Deficiency of CD55 and CD59 in erythrocytes and leukocytes Total blood cell analysis: red blood cells decreased, possibly decreased red blood cells, white blood cells, and reticulocytes. Bilirubin, serum LDH increased Myelogram, bone marrow biopsies: images of hypoplasia or myeloproliferative disorders. Cytogenetic analysis: Possible damage in the case of myeloproliferative disorders.
2. How to classify paroxysmal nocturnal hemoglobinuria
3. Treatment of paroxysmal nocturnal hemoglobinuria
3.1. Treatment of anemia
Transfusion of red blood cells Add iron if iron is low
3.2. Hemolysis treatment
Methylprednisolone: short-term treatment in case of acute hemolysis. Prolonged treatment in case of prevention of erythrocyte destruction. Androgen Eculizumab: Initial dose: once a week 600mg/day x 4 weeks. After the initial dose of 900mg/day x 1 week. Maintenance dose: 2 times 900mg/day.
3.3. Thrombosis prevention and treatment of thrombosis
3.3.1. Thrombosis Prophylaxis
Deficiency of CD55 and CD59 in granulocytes > 50%: prophylaxis with warfarin. In case of contraindications to prophylactic anticoagulation: The disease has a long course without thrombosis. Patients at risk of bleeding related to myelosuppression
3.3.2. Thrombosis treatment
Acute embolism: Heparin Budd-Chiari syndrome with thrombosis: anticoagulants and vascular interventions. Patients with a history of thrombosis: prolonged maintenance anticoagulation therapy.
3.4. Treatment of bone marrow damage
Use of immunosuppressive drugs
Tổn thương tuỷ xương cần điều trị bằng thuốc
3.5.Hematopoietic stem cell transplantation: in cases
Marrow damage: bone marrow failure Paroxysmal nocturnal hemoglobinuria Complications: Dangerous, life-threatening thrombosis, repeated many times Hemolytic anemia supplementing blood transfusion All questions should be consulted by a specialist Answering department as well as customers wishing to be examined and treated at Vinmec International General Hospital, you can contact Vinmec Health System nationwide or register online HERE.
