Agranulocytosis - Causes, Symptoms, Treatment And Diagnosis

2024 Author: Josephine Shorter | [email protected]. Last modified: 2024-01-07 17:49

Agranulocytosis: symptoms and treatment

Agranulocytosis is a condition that characterizes a violation of the qualitative composition of the blood. At the same time, the level of granulocytes, which are a special type of leukocytes, decreases in the peripheral blood. Granulocytes include neutrophils, eosinophils, and basophils. Agranulocytosis is characteristic of many diseases. In women, it is detected more often than in men, especially for people who have crossed the age limit of 40 years.

These blood elements received the name "granulocytes" for the reason that after staining them with a number of special dyes (during the study), they acquire granularity. Since granulocytes are a type of leukocytes, agranulocytosis is always accompanied by leukopenia.

The basic constituent of granulocytes are neutrophils (90%). They are designed to protect the body from various harmful factors, including their action aimed at destroying cancer cells. Neutrophils absorb microbes, as well as infected cells, foreign components and tissue debris. Neutrophils produce lysozyme and interferon. These substances are the body's natural defenses that enable it to fight viruses.

So, the most important effects of neutrophils include:

• Maintaining the normal functions of the immune system.
• Activation of the blood coagulation system.
• Maintaining the purity of the blood.

All granulocytes are born in the bone marrow. When an infection enters the body, this process proceeds at an accelerated pace. Granulocytes are sent to the site of infection, where they die during the fight against pathogens. By the way, there is always a large number of dead neutrophils in the purulent masses.

Agranulocytosis in the modern world is diagnosed quite often, since people are forced to take cytostatics and undergo radiation therapy to get rid of many diseases. Agranulocytosis, if left untreated, can cause serious health complications. Many of them are even life threatening. These include sepsis, peritonitis, mediastinitis. The acute form of agranulocytosis in 80% of cases leads to the death of patients.

Content:

• The reasons for the development of agranulocytosis
• Agranulocytosis: forms of the disease
• Agranulocytosis symptoms
• How to detect agranulocytosis?
• How is agranulocytosis treated?

The reasons for the development of agranulocytosis

Agranulocytosis cannot develop by itself; there is always some reason for its occurrence.

Internal factors that can provoke agranulocytosis:

• Predisposition to agranulocytosis at the genetic level.
• Various diseases that affect the immune system, for example, systemic lupus erythematosus, thyroiditis, ankylosing spondylitis, glomerulonephritis, etc.
• Leukemia and aplastic anemia.
• Metastases that have entered the bone marrow.
• Extreme exhaustion.

External reasons that can lead to the development of agranulocytosis include:

• Viral infections: tuberculosis, hepatitis, cytomegalovirus, Epstein-Barr virus.
• Bacterial diseases that have a generalized forum.
• Sepsis.
• Undergoing therapy with a number of drugs: Aminazine, cytostatic drugs, antibiotics from the beta-lactam group.
• Receiving radiation therapy.
• Receiving radiation therapy.
• Poisoning with chemicals, including those that are part of household chemicals.
• Drinking low-quality alcoholic beverages.

Agranulocytosis: forms of the disease

Agranulocytosis can develop throughout life, or it can be a hereditary disorder. However, the genetically transmitted form of the disease is extremely rare.

The disease can be chronic and acute.

Depending on the reason that provoked the development of agranulocytosis, the following forms are distinguished:

• Cytostatic disease (myelotoxic agranulocytosis).
• Immune agranulocytosis and haptenic agranulocytosis.
• Idiopathic agranulocytosis (genuinic), while the cause of the development of the disorder is not established.

Immune form

The disorder manifests itself against the background of the death of mature granulocytes, which are pathogenically affected by the body's own antibodies. Diagnosis can also be made by a blood test that detects neutrophil precursor cells. Since there is a massive death of granulocytes in the body, this leads to its poisoning. Therefore, the symptoms of such agranulocytosis will be acutely expressed.

Autoimmune agranulocytosis is characterized by signs of diseases such as collagenosis, vasculitis, and scleroderma. Antibodies circulate in the blood, which are aimed at fighting their own cells and tissues. It is believed that even serious psychological trauma or a viral infection can provoke the disease. Do not exclude a person's congenital predisposition to autoimmune diseases. The prognosis of autoimmune agranulocytosis depends on the severity of the underlying disease.

Haptenic agranulocytosis always has a severe course. A disorder develops after the introduction of drugs into the body that can act as haptens. Once in the body, haptens can interact with granulocyte proteins and become dangerous. They begin to attract antibodies to themselves, which destroy them along with granulocytes adhered to them.

The role of haptens can be performed by drugs such as: Acetylsalicylic acid, Diacarb, Amidopyrine, Analgin, Indomethacin, Trimethoprim, Isoniazid, Pipolfen, Norsulfazol, PASK, Erythromycin, Butadion, Ftivazid. If you take these drugs in courses, then the risk of developing agranulocytosis increases.

Myelotoxic agranulocytosis

This form of disorder develops against the background of treatment with cytostatics or during the passage of radiation therapy. As a result of this effect on the body, the synthesis of granulocyte progenitor cells, which are born in the bone marrow, is inhibited.

The higher the dose of the drug or radiation, as well as the stronger its toxicity, the harder the agranulocytosis will be.

In addition to cytostatics (Methotrexate, Cyclophosphamide), drugs from the penicillin group, as well as aminoglycosides and macrolides, are capable of provoking the development of agranulocytosis.

• Endogenous myelotoxic agranulocytosis is accompanied by suppression of blood cells that are formed in the bone marrow. They are destroyed by tumor toxins. Later, healthy cells themselves are transformed into cancerous ones.
• Exogenous myelotoxic agranulocytosis develops against the background of severe damage to the bone marrow, which occurs as a result of external factors. In this case, the cells of the red bone marrow begin to multiply actively, but their sensitivity increases. They react to every negative impact coming from the external environment.
• Drug agranulocytosis is caused by the use of cytostatics, which are prescribed for the treatment of cancer and autoimmune processes. They suppress immunity and negatively affect the process of granulocyte formation.

Agranulocytosis symptoms

The myelotoxic form of agranulocytosis can have a latent course, or it can make itself felt with symptoms such as:

• Bleeds from the nose and uterine bleeding that recur regularly.
• The appearance of bruises on the skin and the appearance of a hemorrhagic rash on it.
• The appearance of blood impurities in the urine.
• Pain in the abdomen, like contractions.
• Vomiting and diarrhea.
• Increased bloating, intestinal overflow.
• Blood in the stool.

The walls of the intestine against the background of agranulocytosis are quickly covered with ulcers, areas of necrosis appear on them. With a severe course of the disease, internal bleeding may open, and the person will begin to suffer from the clinic of an acute abdomen.

Areas of necrosis can also occur on the inner surface of the organs of the genitourinary system, lungs, and liver. If the lung tissue is affected, then the patient develops shortness of breath, a cough and pain in the chest. In this case, an abscess is formed in the lungs themselves, followed by gangrene of the organ.

The following signs may indicate immune agranulocytosis:

• The disease always manifests itself acutely, accompanied by a high body temperature.
• The skin acquires an unnatural pallor, sweating of the palms increases.
• Joint pains appear, pharyngitis, tonsillitis, stomatitis and gingivitis develop.
• Salivation increases.
• The lymph nodes increase in size.
• Symptoms of esophageal dysphagia are observed.
• The liver becomes larger in volume, which can be detected by palpation.

When the mucous membranes of the oral cavity are affected, not only the gums become inflamed, but also the tongue, tonsils, and pharynx. Films appear on them, under which the bacterial flora multiplies. Their waste products are absorbed into the systemic circulation, causing severe poisoning of the body. A person develops severe headaches, nausea, vomiting and other symptoms of intoxication. Serological blood tests can detect anti-leukocyte antibodies in it.

In childhood, Kostman agranulocytosis is most often diagnosed. The disease is transmitted with genes, and both the father and the mother can be the carrier of the gene. Children lag behind their peers in mental and physical development, their blood composition changes. Why genes mutate has not yet been established.

If a child is born with Kostman's agranulocytosis, then his skin will be covered with purulent rashes, ulcers and subcutaneous hemorrhages will be present in the mouth. Older children often develop otitis media, rhinitis, pneumonia. The disease is always accompanied by high body temperature, enlarged lymph nodes and liver.

The course of the disease is chronic, in its acute phase ulcers appear on the mucous membranes. If the number of granulocytes in the blood increases, then the disease fades away. The older the child becomes, the less intensity the periods of exacerbation acquire.

However, the severe course of agranulocytosis is associated with a number of complications, including:

• Pneumonia.
• Lung abscess.
• Intestinal perforation.
• Peritonitis.
• Sepsis.
• Endotoxic shock.
• Inflammation of the kidneys and other organs of the urinary and reproductive system.

How to detect agranulocytosis?

To confirm the diagnosis, you must go to the hospital.

The doctor will assess the patient's condition and, based on the existing symptoms, prescribe the following studies:

• Blood donation for general analysis.
• Delivery of urine for general analysis.
• Immunogram, myelogram and sternal puncture.
• Blood test for sterility.
• X-ray examination of the lungs.

Perhaps the patient will be referred for a consultation with an otolaryngologist and a dentist.

How is agranulocytosis treated?

To cure a person from agranulocytosis, the following therapeutic scheme will need to be implemented:

• The patient is admitted to the hospital in the hematology department of the hospital.
• The patient should be in a box, where the air is regularly treated to achieve a sterile environment.
• Treatment should be based on the cause that triggered the development of agranulocytosis. Sometimes it is enough to get rid of the infection to bring the granulocyte count back to normal.
• If the patient is seriously affected by the intestine, then he is transferred to parenteral nutrition.
• The mouth must be rinsed with antiseptic solutions.
• If agranulocytosis is triggered by radiation therapy or medication, treatment should be discontinued.
• Purulent processes require the appointment of antibiotics from two different groups. These can be drugs such as: Neomycin, Polymyxin, Oletetrin. It is imperative to prescribe antimycotic drugs to the patient: Fluconazole, Nystatin, Ketoconazole. Antibiotics are indicated for patients whose leukocyte count falls below 1.5 * 10 ⁹ / l. In a complex treatment regimen, immunoglobulins can be prescribed at a dosage of 400 mg / kg once.
• In order to increase the production of leukocytes, the patient is prescribed drugs Pentoxil, Leukomax, Leucogen. Course treatment, it should last for 2-4 weeks.
• Perhaps the appointment of high doses of hormonal drugs: Prednisolone, Dexamethasone, Diprospan.
• If the situation so requires, then the patient can be transplanted bone marrow, or transfused a concentrate of leukocytes. When a leukocyte mass is transfused, it should be carefully checked for compatibility with the patient's blood using the HLA antigen system.
• To eliminate the symptoms of anemia, iron supplementation is required, for example, Sorbifer Durules.
• To remove intoxication from the body, the patient is prescribed the introduction of Gemodez, Ringer's solution and isotonic sodium chloride solution.
• The oral cavity is treated with Levorin, to accelerate the healing of ulcers, they are lubricated with sea buckthorn oil.
• The platelet mass is transfused to patients with hemorrhagic syndrome. To eliminate it, the drug Vikasol or Dicinon can be prescribed.

Prevention of agranulocytosis comes down to regular monitoring of the blood picture during therapy with myelotoxic drugs, during radiation and chemotherapy.

Patient nutrition should be aimed at restoring bone marrow performance. The menu should be enriched with fatty fish, chicken eggs, walnuts, chicken fillets, beets, salad and carrots. It is useful to drink freshly pressed juices, eat seaweed. It is imperative to take vitamins, especially during periods of their deficiency.

As for the prognosis for recovery, it directly depends on what exactly caused agranulocytosis. If a patient develops sepsis, then the risk of death increases significantly. With a severe course of agranulocytosis, a person can become disabled and even die.

Video: about agranulocytosis during chemotherapy:

The author of the article: Shutov Maxim Evgenievich | Hematologist

Education: In 2013 he graduated from the Kursk State Medical University and received a diploma "General Medicine". After 2 years, completed residency in the specialty "Oncology". In 2016 completed postgraduate studies at the National Medical and Surgical Center named after N. I. Pirogov.