Symptoms of agranulocytosis

In agranulocytosis, the characteristic symptom is the formation of ulcers, and at a high speed. Tissue necrosis spreads not only to the affected areas, but also to adjacent surfaces. In this case, this process can acquire manifestations of noma, that is, water cancer, in which this pathological process passes to the mucous membrane of the cheeks, which causes gangrenous complications in the tissues of the face.

The same inflammations affect the gastrointestinal tract, where manifestations of ulcerative-necrotic enteropathy occur. In this process, necrosis of the mucous membrane of organs such as the esophagus, stomach and intestines is formed. Symptoms of this disease are expressed in the appearance of diarrhea, in some cases, with blood and vomiting. At a severe stage of necrotic lesions of the epithelium of the gastrointestinal tract, severe bleeding occurs, which poses a threat to human life.

Some patients may develop jaundice, as the liver also experiences tissue damage.

Sometimes, but quite rarely, ulcers may perforate, causing all the clinical symptoms of an “acute abdomen.”

It is also possible that pneumonia may develop, which proceeds quite atypically – with the appearance of abscesses, the development of gangrene and the penetration of the pathological process into the tissues of adjacent organs. In this case, the patient begins to be bothered by symptoms such as coughing, shortness of breath and chest pain (if the pleura has been affected). Severe cases of the disease are accompanied by such a phenomenon as acute respiratory failure.

Sometimes, agranulocytosis causes infectious diseases of the genitourinary system that affect the urethra and bladder, and in women, the uterus and vagina. In this case, a burning sensation and painful sensations during urination, as well as pain in the lower abdomen, are characteristic. Women may also complain of itching and pathological discharge from the vagina.

Intoxication processes in agranulocytosis begin to trigger arterial and venous hypotension. If auscultation of the heart is performed, then functional noises can be detected.

The processes in the kidneys are characterized by the symptom of albuminuria, that is, the excretion of an increased amount of protein with urine. Such changes are caused by the body's reaction to the processes of infection spread. If the kidneys are affected by infection through the penetration of pathological microorganisms from the urinary tract, then the appearance of blood, as well as protein and cells that line the epithelium of the renal tubules, can be observed in the urine.

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Leukopenia and agranulocytosis

Granulocytes are a significant part of leukocytes, therefore agranulocytosis and leukopenia are two parallel processes that occur when the number of granulocytes in the blood decreases.

Leukopenia is a critical decrease in the number of leukocytes in a conventional unit of human blood. To diagnose leukopenia, the number of leukocytes must decrease to 4x109 per µl of blood. Usually, this condition is temporary, and also characterizes many types of diseases as one of the symptoms.

There are several types of leukopenia:

• caused by errors in the production of leukocytes,
• associated with problems in the movement of neutrophils and their migration from the bone marrow,
• caused by the destruction of leukocytes, which occurs in the blood vessels and their leaching from the body,
• caused by redistributive neutropenia.

With leukopenia, various infections rapidly develop in the body. The disease is characterized by the following symptoms:

• presence of chills,
• elevated body temperature,
• increased heart rate,
• the appearance of a headache,
• the emergence of an anxious state,
• high degree of human exhaustion.

In addition, inflammations of various localizations begin to occur in the body: in the mouth, in the intestines (in the form of ulcers), blood infections, pneumonia. The lymph nodes also swell, the tonsils and spleen enlarge.

Neutropenia and agranulocytosis

Neutropenia is a decrease in the number of neutrophils in the blood, which becomes critical. Neutrophils are one of the types of granulocytes, so neutropenia and agranulocytosis occur simultaneously. Sometimes agranulocytosis is called critical neutropenia, since in both diseases, one of the important symptoms is a sharply reduced level of neutrophils.

Neutropenia occurs when the number of neutrophils falls below one and a half thousand in one µl of blood. This leads to a decrease in human immunity, as well as to an increase in the body's sensitivity to the activity of bacteria and fungi that cause various infectious diseases.

Neutropenia comes in several degrees:

• Light - from a thousand units in one µl of blood.
• Moderate – from five hundred to one thousand units in one microliter of blood.
• Severe – less than five hundred units in one microliter of blood.

The disease can be acute (occurs in a matter of days) or chronic (develops over several months or years).

A severe form of neutropenia in acute form, which occurs due to a disruption in the production of neutrophils, poses a threat to human life.

Agranulocytosis and granulocytopenia

Agranulocytosis and granulocytopenia are different stages of the same phenomenon – a decrease in the number of granulocytes in the peripheral blood.

Granulocytopenia is characterized by a decrease in the level of granulocytes, which is not critical for human health. This disease may not manifest itself at all in the patient, proceed in a latent state and without any significant symptoms.

Agranulocytosis is a critical pathological condition that poses a danger to human health, and in some cases, to life. The symptoms of the disease are quite vivid, which is accompanied by severe changes in the patient's body. With agranulocytosis, a complete disappearance of granulocytes from the blood plasma can be observed.

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Acute agranulocytosis

Agranulocytosis is divided into two forms - acute and chronic. This division is due to the cause of the disease.

Acute agranulocytosis manifests itself in a very strong and violent form. It is a consequence of acute radiation sickness and hapten agranulocytosis. The chronic form of the disease manifests itself gradually and is caused by chronic poisoning with benzene or mercury, lupus erythematosus, as well as agranulocytosis in metastatic lesions of the bone marrow and acute leukemia.

The acute condition is characterized by a sharp decrease in the number of granulocytes in the blood serum, as well as a strong deterioration in the patient's condition against this background.

The possibility of recovery from acute manifestations of the disease depends on the level of granulocyte reduction. An important factor is also the patient's health condition together with his immune system before the deterioration of health. The timeliness and correctness of the therapy provided are of great importance.

In chronic forms of the disease, treatment methods and chances of normalizing the condition depend on the course of the underlying disease that caused agranulocytosis.

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Symptoms of myelotoxic agranulocytosis

Myelotoxic agranulocytosis can be of two types:

• of exogenous origin,
• endogenous nature.

Let's take a closer look at each of these types of disease:

• The exogenous form of the disease is provoked by some external factors that have an adverse effect on the human body. Red bone marrow is very sensitive to many environmental factors, so the following factors can affect its functioning:
  • radioactive radiation affecting humans,
  • poisonous toxins that suppress the functions of hematopoiesis, such effects are known from the use of benzene, toluene, arsenic, mercury, etc.,
  • taking certain types of medications.

Based on the information received, it can be concluded that this form of agranulocytosis develops as a consequence of severe damage to the body. And against the background of the latter, the patient shows signs of radiation sickness, benzene poisoning, cytostatic disease, and so on.

The drug-induced form of agranulocytosis is curable. The effect is achieved by discontinuing the drug that provokes the pathological condition. In addition, it is important to know that after recovery, the body becomes more resistant to the drug, meaning that now problems with hematopoiesis are caused by a larger amount of the drug.

This type of agranulocytosis is characterized by a pause between the received external influence and a strong decrease in the number of granulocytes in the blood.

• The endogenous form of the disease is caused by internal factors of the body, namely some diseases. These include manifestations of:
  • acute leukemia,
  • chronic myelogenous leukemia, which has progressed to the terminal stage,
  • metastasis of oncological processes that have grown into the bone marrow.

In this case, normal hematopoiesis is suppressed by the secreted tumor toxins. In addition, the cellular elements of the red bone marrow begin to be replaced by cancer cells.

Immune agranulocytosis

Immune agranulocytosis is characterized by the following clinical picture. As this disease develops, the level of granulocytes decreases due to the high rate of their elimination with the help of antigranulocyte antibodies. These antibodies are capable of destroying granulocytes not only in the blood, but also in some organs that are essential for the blood composition. This applies to the spleen, lungs, and red bone marrow. In some cases, the destruction mechanism also affects the cells that precede the formation of granulocytes, which is very similar to the myelotoxic form of the disease.

One of the symptoms of the disease is severe intoxication of the body, in which there is widespread poisoning of organs and tissues with the help of decay products of destroyed cells. But in some cases, signs of intoxication are mixed with the clinical picture of an infectious complication or with symptoms of the underlying disease.

Laboratory blood tests can show a complete absence of granulocytes and monocytes with a virtually intact number of lymphocytes. The level of leukocytes is much lower than 1.5x109 cells per µl of blood.

This type of agranulocytosis contributes to the development of concomitant diseases, namely thrombocytopenia and anemia. This is due to the appearance of antibodies that destroy not only leukocytes, but also other blood particles. It is also stated that immune damage can also be experienced by a multipotent cell originating from the bone marrow and being the precursor of granulocytes, erythrocytes and thrombocytes.

Drug-induced agranulocytosis

Drug-induced agranulocytosis is a disease characterized by a decrease in the level of white blood cells due to the use of certain medications.

This type of disease is divided into subtypes:

• myelotoxic - caused by cytostatics, chloramphenicol and other drugs,
• haptenic – caused by taking sulfonamides, butadione, etc.

It happens that the same medication can cause different types of agranulocytosis in different people. Such medications include phenositic drugs – aminazine and others. For example, aminazine causes myelotoxic agranulocytosis in a number of people who have an idiosyncrasy to the medication. But the same medication can also provoke an immune type of the disease in other people.

Often, under the influence of medical drugs, a person develops certain antibodies that destroy granulocytes and allow them to be washed out of the body. This mechanism characterizes the hapten type of disease, which will be discussed in more detail below. In this case, the course of the disease is rapid and stormy. And when the drug is discontinued, the pathological symptoms cease, and the body is restored.

Other patients may complain of developing autoimmune reactions, during which antibodies are formed that target protein particles in the structure of leukocytes. For example, systemic lupus erythematosus is characterized by such a clinical picture. In this case, agranulocytosis develops slowly and takes chronic forms.

Therefore, in drug-induced agranulocytosis, it is important to consider not only the characteristics of the drug, but also the individual factors of the patient. A person's reactions may depend on their gender, age, level of immunity, degree of diseases that may accompany the pathology, and so on.

Symptoms of hapten agranulocytosis

Haptenic agranulocytosis occurs when the process of settling of haptens, called incomplete antigens, on granulocyte membranes occurs in the human body. When haptens combine with antibodies, which are located on the surface of leukocytes, an agglutination reaction occurs - that is, a kind of gluing of granulocytes. This process leads to their death and a decrease in their number in the blood. Haptens can be considered different drugs, which is why this type of agranulocytosis has a different name, which is also called medicinal or medicinal.

The onset of haptenic agranulocytosis can be very acute, often bright symptoms of the disease appear immediately after the start of using the drug. When the drug is discontinued, the patient's body also quickly restores blood counts, which indicates an acute form of the disease.

An important characteristic of haptenic agranulocytosis is that after recovery, the human body will react pathologically even to the smallest doses of the drug that caused the disease.

This disease develops at any age, but it is quite rare among children. Women suffer from the above pathology more often than men, but with age this difference becomes unnoticeable.

In elderly people, such dysfunction of the body occurs more often and significantly. The explanation for this fact is in the greater number of drugs taken due to the deterioration of health in elderly people. Also, the state of the immune system deteriorates with age - it loses flexibility, therefore, a decrease in immunity and the occurrence of immune reactions of a pathological nature are observed.

Experts have data that the disease can be provoked by almost any medication. However, there are a number of drugs that are most often the culprits of hapten agranulocytosis. Pathological changes in the blood can be caused by:

The group of sulfonamides, which includes drugs used for type 2 diabetes,

• analgin,
• amidopyrine,
• group of barbiturates,
• butadion,
• a number of anti-tuberculosis drugs, such as PAS, ftivazid, tubazid,
• novocainamide,
• methyluracil,
• antibacterial drugs related to macrolides - erythromycin and so on,
• drugs with antithyroid effect, which are used for hypertrophied thyroid function.

Agranulocytosis from Tyrosol

Thyrozol is a drug used to treat diffuse toxic goiter. Diffuse toxic goiter is an autoimmune disease characterized by thyrotoxicosis, as well as the presence of goiter, ophthalmopathy and, in some cases, dermopathy.

When using Tyrozol, some patients may develop agranulocytosis, i.e. a decrease in the number of granulocytes in the blood below five hundred units per µl of blood. This condition of the patient poses a threat to his health and life. In elderly people, this complication occurs more often than in young patients. In some cases, the side effect of Tyrozol occurs suddenly, within a few days. But most often, this complication develops slowly, within three to four months from the start of therapy.

Agranulocytosis from Tyrozol requires a number of measures:

• drug withdrawal,
• prescribing antibacterial drugs,
• the use of medications with a growth factor effect, which promote rapid restoration of bone marrow.

If appropriate measures are taken, the patient's recovery can be expected within two to three weeks from the start of therapy. Although there have been cases of death with a similar problem with the hematopoietic system.

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