What are the clinical manifestations of paroxysmal sleep hemoglobinuria?

1 Symptoms: The onset of the disease is slow, and the first symptom is anemia, which accounts for 60.3% of the early manifestations. There are also a few patients with acute hemolysis, and the sudden appearance of soy sauce-colored urine is detected, the conscious symptoms include weakness, dizziness, pallor, palpitations, shortness of breath, tinnitus, eye blossoms, etc. Paroxysmal aggravation or episodes of hemoglobinuria is the typical symptom of the disease, 35% of patients hemoglobinuria is related to sleep, hemolysis episodes can be seen after sleep brown urine and soy sauce color, a few patients may have back pain, limb pain, loss of appetite, fever, nausea and vomiting, incomplete urination, pain in the urethra, etc, When the attack is serious, a few patients may have back pain, limb pain, loss of appetite, fever, nausea and vomiting, incomplete urination, urethral pain, etc. 2. Characteristics: (1) Anemia: pale face, pale lips, pale auricles and pale nail bed. Some patients have iron-containing heme deposits in the skin, pale with dark brown, and pigmentation is more obvious in those with long disease duration. (2) Jaundice: due to hemolysis, 47% of patients have jaundice in the course of the disease, and jaundice as the first manifestation accounts for 4%, jaundice is mostly mild or moderate. (3) Bleeding: A few cases have a tendency to bleed, with bleeding as the initial manifestation in 12% of cases, manifesting as mild to moderate bleeding such as bleeding from the gums, bleeding from the nose and bleeding spots on the skin. Female patients may also present with excessive menstruation. Individual patients may have massive epistaxis, postoperative bleeding that is not explained by local causes, post-abortion bleeding, tarry stool blood and fundus bleeding. (4) Hepatosplenomegaly: 25% have enlargement, 13% have splenomegaly, and 12.5% have enlargement of both liver and spleen (5) Other: compensatory enlargement of the heart is seen in long-term anemia.