Infants and young children need to have a hemoglobin test after birth, so that we can find out whether their hemoglobin index is at a normal level. If your baby's hemoglobin shows 114 after the test, you must pay more attention. Such unstable hemoglobin is most likely to cause anemia symptoms or other blood system diseases in the baby's future life. In addition to jaundice, anemia, and hepatosplenomegaly 3 to 4 months after birth, the patient also has poor development. Organ dysfunction caused by sickle cell obstruction of microcirculation can manifest as abdominal pain, shortness of breath, renal pain and hematuria. Patients often die from aplastic crisis, worsening anemia, and concurrent infections. HbS heterozygous patients do not develop sickle cell disease or anemia except in hypoxic conditions due to the low HbS concentration in their red blood cells. There are no clinical symptoms or only occasional manifestations such as hematuria and splenic infarction. HbC is more common in black Africans, but there are no reports in China. Hemoglobin crystals may also form in homozygotes, and patients may be asymptomatic or have mild hemolytic anemia. The spleen may be enlarged, and the number of target cells in the blood smear may increase. Heterozygotes do not have anemia. 2. Unstable hemoglobin Patients with this disease may have anemia of varying severity, or may have no anemia or other clinical symptoms. 3. Increased oxygen affinity of hemoglobin In severe cases, it can cause tissue hypoxia and compensatory erythrocytosis. Neither white blood cells nor platelets increase, and there are patients with the same disease in the family. 4. Hemoglobin M (familial cyanosis) Patients are often asymptomatic and clinically cyanotic, but methemoglobin generally does not exceed 30 molecules per hundred. Hemolysis is usually not obvious and hyaluronic acid bodies are not formed in red blood cells. 5. Increased red blood cell count can cause hypoxia, so the patient's breathing condition should be checked. [1] 1. Blood picture: Hemoglobin is mostly <60g/L, showing microcytic hypochromic anemia, red blood cells are of different sizes and shapes, target cells are mostly >10%, and red blood cell fragments can be seen. Reticulocytosis. There are inclusion bodies in red blood cells. White blood cells and platelets are normal or decreased. 2. Bone marrow image: active proliferation, obvious proliferation of erythroid cells. Extracellular iron and sideroblasts increased. 3. Hemoglobin electrophoresis: hemoglobin F>30% (severe β-globin production disorder). Hemoglobin Bart>80% (hemoglobin Bart fetal edema syndrome); hemoglobin H band appears on electrophoresis (hemoglobin H disease). 4. If conditions permit, analysis of α and β globin peptide chain synthesis ratio and gene analysis can be performed. |
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