Hemolytic Anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)

What is Hemolytic Anemia associated with a deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)?

Hemolytic anemia associated with a deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG) is a hereditary hemolytic anemia associated with a deficiency of enzyme activity.

In contrast to microspherocytosis, it is characterized by a normal form of red blood cells with a tendency to macroplanocytosis, normal or increased osmotic resistance of red blood cells, a recessive mode of inheritance, and the absence of a splenectomy effect.

Causes of Hemolytic Anemia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency (G-6-FDG)

According to WHO, there are about 100 million people in the world who are deficient in G-6-FDG activity. Most often this anomaly is found in the countries of the Mediterranean coast (Italy, Greece), in some countries of Latin America and Africa. In the CIS, the deficit of G-6-FDG is most common among the residents of Azerbaijan. In addition, the carriage of the pathological gene is described in Tajiks, Georgians, and Russians. Children with G-6-FDG deficiency may show favism. The deficiency of G-6-FDG is inherited by the recessive type, coupled with the floor, and therefore the clinical manifestations of this pathology are observed mainly in men. With low G-6-FDG activity in erythrocytes, the reduction of nicotinamidine nucleotide phosphate (NADP) and the conversion of oxidized glutathione into reduced, protecting the erythrocyte from the destructive action of potential hemolytic agents (phenylhydrazine, some medicines, legumes, etc.) are disturbed. Hemolysis occurs predominantly intravascular. Skin and internal organs are jaundiced. There is an increase and a plethora of the liver and spleen, a moderate increase and swelling of the kidneys. Microscopically, hemoglobin-containing cylinders are detected in the renal tubules. In the liver and spleen, a macrophage reaction is observed with the presence of hemosiderin in macrophages.

Pathogenesis during Hemolytic Anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)

The basis of the pathogenesis of nonsferocytic hemolytic anemia is the lack of activity of certain erythrocyte enzymes, as a result of which erythrocytes become sensitive to the effects of various substances of plant origin and drugs.

Symptoms of Hemolytic Anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)

As a rule, G-6-FDG deficiency does not manifest clinically without exposure to various hemolytic agents. Anti-malarial drugs, sulfonamides, analgesics, some chemotherapy drugs (furadonin, PAS), vitamin K, herbal products (legumes, chillies) can provoke a hemolytic crisis. The severity of the hemolytic process depends on the degree of G-6-FDG deficiency and on the dose of the drug taken. Hemolysis does not occur immediately, but 2 to 3 days after taking the drugs. In severe cases, patients develop a high temperature, severe weakness, pain in the abdomen and back, and abundant vomiting. There is marked shortness of breath, palpitations, often the development of a collaptoid state. A characteristic symptom is the discharge of dark urine, which is sometimes black in color, which is associated with the intravascular breakdown of red blood cells and the release of hemosiderin with urine. In some cases, due to blockage of the renal tubules by the breakdown products of hemoglobin and a sharp decrease in glomerular filtration, the development of acute renal failure is possible. An objective study noted icteric staining of the skin and mucous membranes, an enlarged spleen, less often the liver. A week later, hemolysis stops, regardless of whether the drug is continued or not.

Diagnosis of Hemolytic Anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)

During the first two days of hemolytic crisis, patients develop severe normochromic anemia with a drop in hemoglobin of up to 30 g / l and below. There is a high reticulocytosis, the presence of normocytes in the blood. A feature of the red blood cells is the presence of Heinz bodies in them, which are denatured hemoglobin and are detected with supravital coloration. Osmotic resistance of erythrocytes is normal or increased. From the side of white blood during a crisis, leukocytosis is noted with a shift to the left to myelocytes and younger forms. Hyperplasia of an erythroid sprout and the phenomenon of erythrophagocytosis is observed in the bone marrow. The diagnosis of acute hemolytic anemia associated with G-6-FDG deficiency is made on the basis of the typical clinical and hematological picture of acute intravascular hemolysis, the association of the disease with medication and laboratory data revealing a decrease in G-6-FDG activity in erythrocytes of patients, and sometimes their relatives. When diagnosing, it is necessary to take into account the geographical prevalence of G-6-FDG deficiency.

Treatment of Hemolytic Anemia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency (G-6-FDG)

The main method of treating acute hemolytic anemia with a pronounced drop in the hemoglobin content is repeated transfusions of fresh-citric single-group blood in 250–500 ml 1–2 times a week intravenous fluids of large amounts of saline or 5% glucose solution. As anti-shock drugs used morphine, prednisone, promedol. From vascular means use kordiamin, camphor. With the development of acute renal failure, the usual complex of therapeutic measures is carried out; in the absence of effect, hemodialysis is indicated. In case of mild hemolytic crises, ervit intramuscularly in doses of 2 ml, 2 times a day, is prescribed as an antioxidant drug.

Prevention of Hemolytic Anemia associated with deficiency of glucose-6-phosphate dehydrogenase (G-6-FDG)

Prevention of hemolytic crises is the careful collection of anamnesis before the appointment of funds that can provoke hemolytic crisis with a deficiency of G-6-FDG. If necessary, the use of these drugs in individuals with G-6-FDG deficiency is recommended to use funds to restore glutathione. For this purpose, xylitol is used in a daily dose of 30 g in combination with riboflavin at a dose of 0.03 g for 1 to 2 months. The prognosis is unfavorable for the development of anuria and renal failure. With fulminant forms of the disease, death occurs from shock or acute anoxia.

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