A few questions about paroxysmal sleep hemoglobinuria in the diagnosis and management of paroxysmal sleep hemoglobinuria?

1. This disease is quite similar to remittent anemia in terms of clinical manifestations, blood and bone marrow. Especially in the absence of hemoglobinuria, it is often easy to misdiagnose as simple aplastic anemia. Therefore, such patients should be carefully examined and followed up for a long time to distinguish it from simple paroxysmal sleep hemoglobinuria, reocclusion-sleeping hemoglobinuria syndrome or simple aplastic anemia. In addition, this disease needs to be distinguished from other hemolytic diseases, especially paroxysmal cold hemoglobinuria, cold agglutinin syndrome, hemoglobinopathies and so on. Xu Wenjiang, Department of Hematology, Langfang Hospital of Traditional Chinese Medicine 2, paroxysmal sleep hemoglobinuria has a slow onset, but there are also those with a more acute onset. It is not difficult to diagnose typical clinical cases of hemoglobinuria. For those who have no obvious typical hemoglobinuria, it is easy to misdiagnose and miss the diagnosis. Therefore, the following clinical manifestations, should consider the possibility of this disease, and further relevant examinations: such as unexplained anemia, especially the initial diagnosis of hemolytic anemia and the etiology of the unknown; unexplained thrombocytopenia, regardless of myeloproliferative hypoplasia; persistent unexplained iron deficiency, especially with hemolysis; recurrent episodes of venous thrombosis, especially with abdominal pain or headache; unexplained intravascular hemolysis For those with hemoglobinuria. 3.For no hemoglobinuria attack. Bone marrow hyperplasia, moderate to severe hematopoiesis of this disease, commonly used to stimulate hematopoietic drugs, such as androgens, generally oral-based, but in the use of the liver should pay attention to the side effects of liver damage, should be routinely strengthened to protect the liver, such as liver tetrazolium, hepatic get health, etc., should not be used bifidobenzoate, because of its acidic taste, in order to avoid inducing or aggravating hemolysis. 4.For those who have typical hemoglobinuria episodes. For those who have typical hemoglobinuria episodes and active bone marrow proliferation, hemolytic episodes should be actively controlled with corticosteroids, dextrose anhydride, sodium bicarbonate, high-dose vitamin E, etc., and regular and sufficient transfusion of washed erythrocytes can be used to inhibit the generation of type II and type III erythrocytes to alleviate hemolysis; small-dose chemotherapy can be considered if necessary. In addition, iron in the iron deficiency can be given to the regular dose of 1/3 ~ 1/10 discretionary supplementation, attention can not be supplemented according to the regular dose, not to mention in the absence of clear evidence of iron deficiency before the blind supplementation of iron, so as not to stimulate the complement-sensitive cells proliferation induced hemolysis. For those with thrombosis, it is advisable to use dextrose anhydride, it is reported that the molecular weight of 142000 is better; also can use anticoagulants, but heparin should be used with caution, unless extensive thrombosis, otherwise it is best not to use, because there are reports of hemolysis can be induced. For this disease, there are also reports that large doses of 654-II can be used, such as hemoglobinuria episodes of 90mg to 120mg, intravenous drip, after stabilization, it was changed to 20mg, 3 times a day orally. 5, paroxysmal sleep hemoglobinuria belongs to the scope of the motherland medicine jaundice (yin yang), deficiency labor, stasis. Its main pathogenesis is dampness and heat intertwined with steam, and yellowing; or dampness and toxin fire, injuring the camp and seizing the blood; or qi and blood deficiency, blood is not colorful; or chronic disease into the complex, stagnation of qi and blood stasis. The key to the pathogenesis of chronic anemia is deficiency of the spleen, stomach, liver and kidney, and insufficiency of qi and blood; the key to the pathogenesis of acute hemolysis is dampness, heat and toxicity; and the change of the disease is due to deficiency of qi and stagnation of qi, and stagnation of blood and stasis of blood in the collaterals. The disease is characterized by deficiency of spleen and kidney yin, yang, qi and blood, with dampness, heat and blood stasis as the symptoms. Clinical deficiency and evil real often exist at the same time, the symptoms can be converted to each other, and the evidence is often mixed with each other, so it is necessary to grasp the mechanism of the disease, and diagnosis and treatment. Generally speaking, in the chronic anemia period, the method of benefiting qi and nourishing blood, strengthening the spleen and tonifying the kidney, filling in the essence and marrow, nourishing the liver and kidney, etc.; in the acute hemolytic period, the method of clearing away heat and dampness, eliminating blood stasis and removing the yellow coloration is often used; in the case of stasis and blood stasis blocking the orifices and collaterals, the method of benefitting the qi and activating the blood, eliminating blood stasis and clearing the collaterals and opening up the orifices is used. It has been reported that Huangqi Jianzhong Tang, Yin Chen Artemisia Tang, Yin Chen Wu Ling San and other additions and subtractions can prevent hemolytic episodes. There is also a report of single-flavored Chinese herbal medicine Yangmei root bark extract made of “anti-soluble spirit” treatment of this disease, the effective rate of 79.3%, can be used for reference. In addition, any acidic Chinese medicine may also induce or aggravate hemolysis, so it is advisable to be cautious or avoided for this disease, such as schizandra, cornelian cherry, sour jujube nut, umeboshi, Sichuan papaya and so on. Can also be added in the formula squid bone, corrugated seeds, mother of pearl, oysters and other acid-producing drugs. 6.Till now, there is no ideal treatment for this disease. Allogeneic hematopoietic stem cell transplantation is expected to remove the abnormal hematopoietic clones and achieve the goal of eradication, but due to the lack of donors, high cost, side effects and other limitations, it has not been widely and routinely used. Due to the lack of effective therapies, it is more important to avoid triggering factors as much as possible, including actively enhancing physical fitness, preventing and controlling viral infections; avoiding the use of drugs that may induce hemolysis; avoiding the consumption of acidic foods such as oranges, mangoes, vinegar, and leeks, and drinking alcohol; avoiding strenuous exercise, excessive fatigue, and mental stimulation; avoiding or reducing surgeries, traumas, and transfusion of washed or concentrated red blood cells instead of whole blood when necessary; and avoiding the use of red blood cells for patients with this disease, including the use of blood transfusions, which can cause the disease to become chronic. For patients with this disease, it is prudent to carry out vaccination, and minimize acidic diet during pregnancy and menstruation. For patients with frequent hemolytic episodes, alkaline drugs such as baking soda can be added as appropriate.