Treatment of sideroblastic anemias

Treatment of sideroachrestic anemias

Treatment of patients with hereditary sideroachrestic anemia

1. Vitamin B _{6 in large doses - 4-8 ml of 5% solution per day intramuscularly. If there is no effect, the coenzyme of vitamin B12} - pyridoxal phosphate is indicated. The daily dose of the drug is 80-120 mg when taken orally.
2. Desferal (to bind and remove iron from the body) - 10 mg/kg/day in monthly courses 3-6 times a year.

Treatment of patients with acquired sideroachrestic anemia caused by lead intoxication

1. Identify and eliminate the source of lead. Until the source of lead is completely eliminated, the child should not be in the house. The risk of toxic exposure increases even if the child only sleeps at home. Wet cleaning and vacuuming are necessary to remove lead dust.
2. To compensate for iron deficiency and reduce lead absorption, iron preparations (6 mg/kg/day of elemental iron) are prescribed orally. The course duration is 1 month or until the level of erythrocyte protoporphyrin is normalized.
3. Therapy with complexing agents - EDTA, dimercaprol, penicillamine and succimer.

The goal of treatment is to reduce lead levels to safe levels (blood levels less than 15 mcg%) and erythrocyte protoporphyrin levels to normal levels (less than 35 mcg%).

Indications for therapy with complexing agents.

Therapy with complexing agents is indicated in the presence of at least one of three conditions:

1. lead level in venous blood 50 mcg% in 2 consecutive samples;
2. the level of lead in venous blood is 25-49 mcg%, and the level of erythrocyte protoporphyrin is 125 mcg%;
3. positive EDTA test.

Mild lead poisoning (blood lead level 20-35 mcg%)

Penicillamine is prescribed at a dose of 900 mg/ ^m2 /day in 2 doses. Penicillamine should not be taken together with dairy products and iron preparations; the drug is contraindicated in case of allergy to penicillins.

Moderate lead poisoning (blood lead level 35-45 mcg%)

An EDTA test is performed; if the test results are positive, calcium-disodium EDTA is prescribed at 1000 mg/m2 ^/ day intramuscularly together with procaine for 3-5 days. The break between treatment courses should be at least 48-72 hours. The drug is completely discontinued when the daily excretion of lead in urine is less than 1 μg of lead per 1 mg of EDTA.

Severe lead poisoning without encephalopathy (blood lead level greater than 45 mcg%)

• For lead levels less than 80 mcg%: Succimer: 30 mg/kg/day in 3 doses orally for 5 days, then 20 mg/kg/day in 2 doses for 14 days.
• At lead levels over 80 mcg%: Infusion therapy in a volume exceeding 1.5 times the physiological fluid requirement. Dimercaprol at a dose of 300 mg/m2 ^{intramuscularly}, the dose is divided into 3 injections and administered over 1-3 days. EDTA at a dose of 1500 mg/m2 ^/ day intravenously as a long-term infusion or intramuscularly (single or the dose is divided and administered 2 times a day).

Severe lead poisoning withencephalopathy

1. Hospitalization in the intensive care unit.
2. Infusion therapy.

Dimercaprol 600 mg/ ^m2 /day intramuscularly, divided dose administered 6 times daily. EDTA 1500 mg/kg/day as intravenous infusion, divided dose administered 3 times daily.

3. Anticonvulsants.

After a 5-day course of treatment, take a break for 48 hours, after which treatment is resumed.

Monitoring during treatment with complexing agents

To assess the effectiveness of treatment, daily urinary lead excretion is measured, since its concentration in the blood may be low in the presence of complexing agents. Blood lead concentration is measured every 48-72 hours in hospitalized patients and every 2-4 weeks in outpatients.

During EDTA therapy, monitoring of blood urea and calcium levels, blood and urine lead levels, and periodic urine tests are necessary. If signs of hypocalcemia or renal dysfunction appear, the EDTA dose is reduced or the drug is discontinued, after which renal function normalizes.

Before and during therapy with succimer, biochemical parameters of liver function, urea and creatinine levels in the blood are examined every 5-7 days.

On the 14th and 28th days after completion of therapy with complexing agents, the level of lead in the blood is measured.

Consequences of lead poisoning

All children exposed to lead toxicity should have a physical examination at age 5 to 6 years that includes assessment of auditory and visual perception, gross and fine motor skills, speech comprehension, and language ability.

Prevention of lead poisoning

To avoid lead poisoning, precautions should be taken when renovating old homes, such as temporarily relocating children. Burning and burying lead paint is especially dangerous; it should be scraped off or removed chemically. Monitoring the condition of living spaces and tightening sanitary and building codes reduces the incidence of poisoning.