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December 26, 2016 00:02

sideroblastic anemia

Anemia associated with impaired synthesis or utilization of porphyrins (sideroahrestical, sideroblastic anemia) - a heterogeneous group of diseases, hereditary and acquired, is associated with impaired activity of enzymes involved in the synthesis of porphyrins and heme.The term "anemia sideroahrestical" introduced Heylmeyer (1957).When sideroahrestical anemia serum iron levels increased.In the bone marrow show ringed sideroblasts - nucleated red blood cells with perinuclear halo consisting of coarse granules gemoside-Rina and representing a filled iron mitochondria.

reasons sideroahrestical anemia

Hereditary forms

are transmitted by recessive coupled to the X-chromosome (sick man) or autosomal dominant manner (sick men and women).Metabolic

block can be on the level of formation of delta-aminolevulinic acid from glycine and succinyl CoA.For this reaction requires pyridoxal phosphate - the active coenzyme pyridoxine and aminolevulinic acid synthetase.

Acquired forms

Acquired forms of anemia as

sociated with impaired porphyrin synthesis may be due to lead poisoning.

Household lead poisoning in pediatric patients are quite common.They occur by eating foods, tinned or stored in pottery with a glaze handicraft production.Lead poisoning is most often caused by entering into of lead in paint, plaster and other materials, saturated with lead pigments (paper, plaster, rubble, lead content exceeds 0.06%) and house dust and soil particles (lead content of 500 mg / kg).From the atmosphere lead supplied not only by inhalation;often it is deposited and ingested dust and soil particles.In infants, lead poisoning occurs when using contaminated water to prepare infant formula.By intoxication can result if the necessary precautions in the smelting of lead in the home.

sideroblastic anemia Causes

Diagnostics sideroahrestical anemia

diagnosis of hereditary anemia is confirmed by the study of the content of porphyrins in erythrocytes.It was established that these inherited forms of anemia decreased sideroahrestical protoporphyrin content of erythrocytes.Content coproporphyrin red blood cells and is increased and decreased.Normally, the average erythrocyte protoporphyrin in whole blood - 18 g%, and the upper limit in the absence of anemia - 35 g%.To study the content of iron stores and confirm hemosiderosis applied desferalovaya sample.After intramuscular administration of 500 mg of Desferal with normal urine output of 0.6-1.2 mg of iron per day, and in patients with sideroblastic anemia - 5-10 mg / day.

To diagnose lead poisoning determine the level of lead in the venous blood;protoporphyria-on level of erythrocytes in whole blood - the level above 100 mg% are usually indicative of a toxic action of lead.

Diagnostics sideroblastic anemia

sideroahrestical Treatment of anemia

Treatment of patients with hereditary anemia sideroahrestical

  1. Vitamin B6 in large doses - 4-8 ml of 5% solution per day intramuscularly.If no effect is shown the appointment of a coenzyme of vitamin B12 - pyridoxal phosphate.The daily dose is 80-120 mg when taken orally.
  2. Desferal (for binding and excretion of iron the body) - 10 mg / kg / day monthly rates of 3-6 times a year.

Treatment sideroblastic anemia

Preventing lead poisoning

avoid lead poisoning in the reconstruction of old houses should take precautions by temporarily displacing children.It is especially dangerous to burn and bury in the ground lead paint, they should be scraped or removed by chemical methods.Control of the premises, the tightening of sanitary and building codes reduces the incidence of poisoning.