What is pernicious anemia. Pernicious anemia is a fatal disease that can be cured with a vitamin. Prevention of pernicious anemia

Pernicious (malignant anemia, Addison-Birmer disease) was first described by Addison and Birmer (1855-1871). Pernicious anemia was considered incurable until 1926, but then became treatable and therefore not “malignant.”

It was once believed that the root cause of this disease was intoxication. However, the source of intoxication remained unknown; For differential diagnosis, we relied on the most characteristic points and pathological anatomy of the disease.

Some attached great importance to the state of the bone marrow in malignant anemia, for example Ehrlich, who called this condition “a return to the embryonic state.” Others attached particular importance to the increased activity of the blood-destructive system (based on determining the daily amount of urobilin in feces and bilirubin in bile).

It seemed to many that it was significantly interested in the pathogenesis of the disease, although it did not always turn out to be increased. Proof of the participation of the spleen in the pathogenesis of the disease was the long remissions that occurred after. At the same time, Decastello attributed the positive effect of splenectomy to the loss of physiological hemolysis; others argued that the function of an organ with increased hemolytic activity was lost, while others believed that splenectomy removed an organ that inhibited the erythropoietic activity of the bone marrow.

The onset of remission after other methods of treatment, as well as the return of the disease after splenectomy, proved in the best possible way that not only hemolysis is to blame for the pathogenesis of malignant anemia.

Currently, the cause of Addison-Biermer disease is considered to be a lack of hematopoietic vitamin B12 and folic acid. Deficiency of this vitamin develops due to functional or anatomical disorders of the fundic glands of the stomach of a neurotrophic nature; the same vitamin deficiency occurs with Achilles gastritis (syphilis, polyposis or) or agastria (gastrectomy or exclusion of the stomach by others), with helminthic infestation (wide tapeworm), pregnancy, sprue (protracted enteritis), with resection or exclusion of the small intestine. These are all secondary forms; in some of them, the disease occurs without nervous phenomena, achylia, and is cured with the elimination of the etiological factor. And finally, vitamin B12 deficiency can be of exogenous origin - a lack of vitamin B12 in food products. Pathogenetically, we are talking about a violation of bone marrow hematopoiesis (impaired maturation of the formed elements of the erythroid germ according to the type of return to embryonic hematopoiesis).

The hemolysis that accompanies this form of anemia does not place it among the hemolytic anemias, since we are talking about the destruction of red blood cells in the bone marrow itself due to impaired hematopoiesis.

Symptoms of pernicious anemia

The disease affects people after 40 years of age (before 25 years of age it was extremely rare), equally often in men and women. It all starts with achylia and changes in the nervous system. Weakness, dizziness appear, and then signs of anemia - shortness of breath, palpitations with minor physical exertion. Along with anemia, glossitis occurs (pain and burning in the tongue) - a pathognomonic symptom of pernicious anemia. Subsequently, the inflammation is replaced by atrophy of the papillae and the tongue becomes as if varnished.

Patients are pale, with a lemon-yellow skin tone. There is some puffiness of the face, swelling of the feet and a tendency towards obesity. The liver is enlarged, the spleen is not. If occasionally the spleen turns out to be enlarged, then this should not be considered an indication for splenectomy, as was the case before the 20s of our century due to misconceptions about the essence of the disease.

The course of pernicious anemia is cyclical - deterioration is replaced by remissions, which can occur spontaneously even after very prolonged deterioration. During deterioration, hyperchromic anemia is especially pronounced due to hemoglobin-rich macrocytes-megalocytes (a product of megaloblastic hematopoiesis) without central clearing; there are few polychromatophils and reticulocytes (their appearance portends remission).

Until now, hyperchromic anemia with phenomena of the embryonic type of hematopoiesis (megalocytes from megaloblasts) is the main characteristic feature that distinguishes true pernicious anemia from other anemias. The rest of the blood picture is characterized by a significant decrease in the number of red blood cells, a decrease in hemoglobin, aniso- and poikilocytosis; erythro- and normoblasts do not at all represent characteristic features of a blood smear and are found in significant numbers only on the eve of remissions. As a rule, there is also leukopenia with relative lymphocytosis; hypersegmented forms are found among neutrophils; sometimes myelocytes are detected (a sign of special irritation of the bone marrow). There are always few platelets. Blood serum is dark yellow, especially in moments of deterioration (hemolysis); In parallel with bilirubinemia, urobilinuria is observed. These phenomena directly depend on the severity of pernicious anemia, and therefore may be completely absent during the period of remission. Significant deterioration may be combined with symptoms of hemorrhagic diathesis. Malnutrition of the heart muscle (due to hypoxia associated with anemia) affects the negative T wave on the electrocardiogram.

In the pathological picture, along with severe anemia of all internal organs, degenerative fatty infiltration and siderosis (deposition of iron-containing pigment), especially a lot of iron is found in the liver, spleen, bone marrow, and lymph nodes. In the spleen, iron is found mainly intracellularly, and this distinguishes pernicious anemia from aplastic anemia, in which siderosis is extracellular. Intracellular hemolysis is a normal type of hemolysis, which maintains the balance of iron metabolism in the body, while extracellular hemolysis disrupts it. That is why in pernicious anemia there is hyperchromemia, and in aplastic anemia there is hypochromemia.

Pathoanatomical changes in the spleen with pernicious anemia appear macroscopically in the form of a particularly strong overflow of blood; the histological picture shows a significant overflow of the spleen pulp with red blood cells located more around the trabeculae and follicles; the latter are almost always preserved, and in some cases their number is even increased. Sometimes extramarrow hematopoiesis is observed in the form of the appearance of scattered myeloid foci located near the vascular adventitia. According to some, there are also vascular changes, especially in the form of thickening of the walls of the central arteries and deposition of hyaline in the intimal area. Hyaline degeneration of the small vessels of the spleen is a very common occurrence in people between 10 and 40 years of age, and even more common in older people. At the same time, in small vessels the entire intima is degenerated.

Other changes in the hematopoietic system include the appearance of red bone marrow in long tubular bones and the presence of a large number of megaloblasts in the microscopic picture of the bone marrow; Extramarrow hematopoiesis is also sometimes observed in the lymph nodes and liver.

Along with the hematopoietic system, there are changes in the digestive tract; they come down to inflammation and atrophy of the mucous membrane of the stomach and intestines. Changes in the adrenal glands are detected in the form of a decrease in lipoids and chromaffin substance.

Agastric anemia - anemia due to loss of the anti-anemic function of the stomach (after removal of the stomach or in some of its diseases). Anemia develops as pernicious anemia, but more often as achilic chloranemia. The fears that such anemia always entails are exaggerated. Severe anemia of the malignant type sometimes occurs only after total gastrectomies (about 8%); treatment in these cases is not unsuccessful, and postponing surgery to prevent anemia is dangerous from an oncological point of view. Conventional gastrectomy, especially in cases of peptic ulcer, is sometimes (15-20% of cases) accompanied by slight anemia of the chlorine-anemic order, which responds well to treatment with iron.

Treatment of pernicious anemia

Currently, pernicious anemia is cured with organotherapy and vitamin therapy. It is recommended to take 100 g of raw veal liver (passed through a meat grinder) twice a day (washed down with diluted hydrochloric acid - 25 drops in half a glass of water) 2 hours before regular meals for 5-6 weeks. Liver extracts for internal and parenteral use. The effectiveness of all of these treatment methods depends on the content of hematopoietic vitamin B12 in them. Liver extract has been successfully used recently.

To prevent recurrence of pernicious anemia, liver therapy (liver extract or liver itself, as indicated above) must be carried out regularly every other day. Recently, antianemin (liver concentrate in combination with cobalt) has been successfully used in the form of injections into muscles of 2-4 ml daily. You can also use intramuscular injection of pure (crystalline) vitamin B12, 15-30 mcg.

For functional myelosis, raw liver (rich in vitamins B1 and B12) is most effective.

Anemia in pregnant women is curable with vigorous parenteral liver therapy, Campolon (termination of pregnancy is allowed only in case of ineffective treatment).

Attempts to influence pernicious anemia through a combined intervention in the form of removal of the spleen and simultaneous transplantation of the adrenal gland (the latter measure was based on changes in the adrenal glands of the patients) were also unsuccessful.

Prognosis for pernicious anemia

The prognosis is favorable. With systematic treatment, remission continues for years. The prediction is even better when the cause of the disease is syphilis and tapeworm; expulsion of the latter or specific anti-syphilitic treatment leads to complete recovery.

Pernicious anemia shares symptoms with other types of anemia, but the diagnosis is based on very precise clinical data. Let's consider the features and methods of treatment of this type of anemia.

Features of pernicious anemia

Pernicious anemia is a progressive disease, i.e. it gradually intensifies. Associated with deficiency of a specific vitamin B12 or cobalamin, important for the formation and maturation of red blood cells in the blood.

In addition, the accelerated destruction of red blood cells provokes increased bilirubin levels, a substance that is formed as a result of the catabolism of hemoglobin.

Pernicious anemia is quite common in old age, due to the reduced functionality of the gastrointestinal tract, which loses the ability to effectively absorb many vitamins, including B12.

More rarely, megaloblastic anemia affects children, in which, as we will see later, it is most often associated with genetic causes or nutritional disorders.

Symptoms of megaloblastic anemia

The symptoms of pernicious anemia are caused by low efficiency in transporting oxygen to the tissues and are therefore common to all other types of anemia.

In particular:

• pallor associated with reduced blood flow to the skin
• fatigue and apathy caused by muscle hypoxia
• tachycardia, due to the heart's attempt to compensate for the reduction in oxygen flow
• dizziness, confusion and memory loss, due to the fact that vitamin B12 is a cofactor important for the functioning of nerve cells, and the deficiency of which leads to neurological changes.

How is pernicious anemia diagnosed?

A diagnostic test for pernicious anemia includes, first of all, a complete blood count, which confirms the diagnosis if:

• red blood cell count less than 3 million per mm3
• there is a decrease in serum iron levels
• test for vitamin B12 below control values ​​– 200 - 900 pg/ml
• Ferritin level is reduced, i.e. iron reserves
• the average volume of erythrocytes is increased, since unripe erythrocytes are large

Other tests used for the diagnosis of pernicious anemia:

• indirect bilirubin level, which increases in pernicious anemia
• gastrin level, is a hormone that is present in the gastric mucosa and is necessary for the absorption of vitamin B12
• alkaline phosphatase level, which allows you to evaluate the activity of white blood cells to identify autoimmune causes of the disease

Decreased levels of red blood cells and iron have a negative prognosis if not corrected, as they can lead to significant complications, especially when the nervous system is stressed: cobalamin deficiency over a long period of time can lead to irreversible nerve damage.

Causes of pernicious anemia

Typically, vitamin B12, present in animal products, binds to Castle factor, which is secreted by the gastric mucosa and has the task of promoting the absorption of cobalamin in the intestine.

Thus, vitamin B12 deficiency may result from:

• insufficient consumption of animal foods (vegetarian diet): Vitamin B12 occurs naturally only in animal products, so a vegetarian diet (without the use of supplements) inevitably leads to vitamin B12 deficiency.
• Intrinsic factor deficiency caused by damage to the gastric mucosa. Gastric walls can be damaged by stomach cancer, alcohol, infection of the stomach by helycobacter bacteria, or Biermer's disease (an autoimmune disease in which antibodies attack and destroy the stomach's own mucosa, causing it to atrophy).
• Malabsorption in the intestines, as in the case of Crohn's disease, in which intestinal cells lose the ability to absorb vitamin B12.
• Congenital intrinsic factor deficiency, a rare genetic pathology in which the synthesis of Castle factor is disrupted. The disease is present from birth and manifests itself in children up to the age of five.

Therapy for macrocytic anemia: nutritional supplements and diets

In the case of pernicious anemia, therapy consists of administering to the patient:

• vitamin B12 tablets to compensate for its deficiency;
• gland to restore the level of this mineral in the blood;
• folic acid, which stimulates the synthesis and production of red blood cells.

In case of malabsorption of vitamin B12 or gastric atrophy, vitamin B12 is administered by injection or nasal spray.

Important role in the prevention and treatment of pernicious anemia food plays. Cobalamin can be found in foods such as:

• liver
• egg yolk

and to a lesser extent in:

• mature cheeses
• spirulina algae

A varied and balanced diet provides the right amount of vitamin B12, but vegetarian diets, and especially vegan ones, require appropriate integration of this important vitamin so as not to risk the consequences of pernicious anemia.

Endogenous B12 vitamin deficiency caused by atrophy of the glands of the fundus of the stomach that produce gastromucoprotein. This leads to impaired absorption of vitamin B12, necessary for normal hematopoiesis, and the development of pathological megaloblastic hematopoiesis, resulting in “pernicious” type anemia. People over the age of 50 get sick.

Symptoms of pernicious anemia

Characterized by disorders of the cardiovascular, nervous, digestive and hematopoietic systems.

Patients' complaints are varied:

• general weakness,
• dyspnea,
• heartbeat,
• pain in the heart area,
• swelling of the legs,
• crawling sensation in the hands and feet,
• gait disorder,
• burning pain in the tongue,
• periodic diarrhea.

The patient's appearance is characterized by pale skin with a lemon-yellow tint. The sclera is subicteric. The patients are not exhausted. When examining the cardiovascular system, anemic murmurs are typical, associated with a decrease in blood viscosity and acceleration of blood flow.

On the part of the digestive organs, the so-called Gunter's glossitis (the tongue is bright red, the papillae are smoothed), histamine-resistant achilia (lack of free hydrochloric acid and pepsin in the gastric contents) are detected. The liver and spleen are enlarged.

With a significant decrease in the number of red blood cells (below 2 million), a fever of the wrong type is observed. Changes in the nervous system are associated with degeneration and sclerosis of the posterior and lateral columns of the spinal cord (funicular myelosis).

Blood picture:

• hyperchromic type anemia,
• macrocytes,
• megalocytes,
• red blood cells with Jolly bodies,
• Cabot's rings,
• leukopenia,
• thrombocytopenia (during exacerbation).

Descriptions of symptoms of pernicious anemia

Which doctors should I consult for pernicious anemia?

Treatment of pernicious anemia

Treatment is carried out with vitamin B12-100-200 mcg intramuscularly daily or every other day until remission occurs. If an anemic coma occurs, urgent hospitalization, blood transfusion, preferably red blood cells (150-200 ml). Maintenance therapy with vitamin B12 is necessary to prevent relapses.

Systematic monitoring of blood composition in people with persistent achylia, as well as those who have undergone gastrectomy, is indicated. Patients suffering from pernicious anemia should be under medical supervision (stomach cancer may occur).

Pernicious anemia
Chronic diseases/anemia due to insufficient absorption of vitamin B 12

It occurs in adults as a result of gastric atrophy (cannot absorb vitamin B12). Wall cells in the stomach that produce intrinsic factor necessary for the absorption of vitamin B12 and, if destroyed, lead to a lack of this very important vitamin.

The name pernicious anemia is a holdover from the times when this type of anemia was fatal and retained the name for historical reasons.

Due to a lack of vitamin B12, a number of diseases and conditions can be caused, but anemia only includes those caused by atrophic gastritis and loss of parietal cell function.
Dr. Addison was the first to describe the disease and until 1920, people died from the disease within 1 to 3 years of diagnosis. Doctors have been researching this anemia so that patients consume more raw liver and juice in large quantities. And therefore they deservedly received the Nobel Prize in 1934, incurable diseases!

We are very grateful for the progress, as well as the fact that we do not eat raw liver and have solved the deficiency of this vitamin with tablets or injections!
Vitamin B 12 cannot be created/synthesized by the human or animal body so it must be obtained from food. It is extremely important for the proper functioning of the brain and nervous system; it is involved in the metabolism of every cell in the body.

Most people get vitamin B12 from meat (especially liver), fish, shellfish and dairy products.

The genetic variant of pernicious anemia is an autoimmune disease, with a definitive genetic predisposition. Antibodies occur in 90% of people with pernicious anemia, but currently only 5% of people in the general population.
Classic pernicious anemia, caused by a lack of intrinsic factors synthesized in the parietal cells of the stomach, does not absorb vitamin B12, the result: megaloblastic anemia.
Any disease or condition can lead to malabsorption of vitamin B12 deficiency and anemia is observed (though not always), a neurological condition.

Periodicity:
It is most widespread among the Northern European population. English, Scandinavians, Irish, Scots, aged 40-70 years of life.

Problem:
Weight loss, temperature
Anemia is quite tolerable, even when hemoglobin is very low (40-50), MCV (mean corpuscular volume) is high: more than 100 UGL
About 50% of patients have a very smooth tongue, without papillae (glositis)
Changes in character and personality
The functioning of the thyroid gland is impaired
Diarrhea
Paresthesia: tingling in arms/legs
The most difficult thing: neurological disorders: problems with balance, gait, muscle weakness. In an elderly person with signs of dementia, a deficiency of vitamin B12 may occur and memory problems, hallucinations, and irritability may occur.

Diagnosis:

Laboratory tests: complete blood count, peripheral blood count, levels of vitamin B12, folic acid, methylmalonic acid and homocysteine ​​in the blood
testing the patient's ability to absorb vitamin B12.

The presence of antibodies and internal factors in the blood.

THERAPY:
As you probably assumed, therapy consists of vitamin B12 replacement. Vitamin B12 does not exist in nature, but is synthesized and administered to patients in tablet form, transdermal, nasal, or by injection (intramuscular, subcutaneous).
Today there are tablets that contain high doses of vitamin B12: 500-1000 mcg, so that there is a sufficient amount of vitamin for the cells of the body.

Is there any doubt, in any case, to provide replacement therapy in the form of injections!
Famous people with pernicious anemia
Alexander Graham Bell
Annie Oakley: 1925. died of pernicious anemia at 65 years old

Description of pernicious anemia

Pernicious anemia is an endogenous B12 vitamin deficiency caused by atrophy of the glands of the fundus of the stomach that produce gastromucoprotein. This leads to impaired absorption of vitamin B12, necessary for normal hematopoiesis, and the development of pathological megaloblastic hematopoiesis, resulting in “pernicious” type anemia. People over the age of 50 get sick.

Characterized by disorders of the cardiovascular, nervous, digestive and hematopoietic systems. The complaints of patients are varied: general weakness, shortness of breath, palpitations, pain in the heart, swelling of the legs, a crawling sensation in the hands and feet, gait disorder, burning pain in the tongue, periodic diarrhea. The patient's appearance is characterized by pale skin with a lemon-yellow tint. The sclera is subicteric. The patients are not exhausted. When examining the cardiovascular system, anemic murmurs are typical, associated with a decrease in blood viscosity and acceleration of blood flow.

On the part of the digestive organs, the so-called Gunter's glossitis (the tongue is bright red, the papillae are smoothed), histamine-resistant achilia (lack of free hydrochloric acid and pepsin in the gastric contents) are detected. The liver and spleen are enlarged. With a significant decrease in the number of red blood cells (below 2 million), a fever of the wrong type is observed. Changes in the nervous system are associated with degeneration and sclerosis of the posterior and lateral columns of the spinal cord (funicular myelosis). Blood picture: hyperchromic type anemia, macrocytes, megalocytes, red blood cells with Jolly bodies, Cabot rings, leukopenia, thrombocytopenia (during exacerbation).

Treatment is carried out with vitamin B12-100-200 mcg intramuscularly daily or every other day until remission occurs. If an anemic coma occurs, urgent hospitalization, blood transfusion, preferably red blood cells (150-200 ml). Maintenance therapy with vitamin B12 is necessary to prevent relapses. Systematic monitoring of blood composition in people with persistent achylia, as well as those who have undergone gastrectomy, is indicated. Patients suffering from pernicious anemia should be under medical supervision (stomach cancer may occur).