Agranulocytosis: Symptoms, Diagnosis, Treatment, Causes

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Agranulocytosis

The content of the article:

1. Causes
2. Forms

3. Signs

   Features of the course of myelotoxic agranulocytosis

4. Diagnostics
5. Treatment
6. Prevention
7. Consequences and complications

Agranulocytosis is a clinical and laboratory syndrome, the main manifestation of which is a sharp decrease or complete absence of neutrophilic granulocytes in the peripheral blood, which is accompanied by an increase in the body's susceptibility to fungal and bacterial infections.

Agranulocytosis is characterized by a sharp decrease in granulocytes in the peripheral blood

Granulocytes are the most numerous group of leukocytes, in which specific granules (granularity) become visible in the cytoplasm when stained. These cells are myeloid cells and are produced in the bone marrow. Granulocytes take an active part in protecting the body from infections: when an infectious agent penetrates into tissues, they migrate from the bloodstream through the capillary walls and rush to the inflammation focus, here they absorb bacteria or fungi, and then destroy them with their enzymes. This process leads to the formation of a local inflammatory response.

With agranulocytosis, the body is not able to resist infection, which often becomes a factor in the occurrence of purulent-septic complications.

In men, agranulocytosis is diagnosed 2-3 times less often than in women; people over 40 are most susceptible to it.

Causes

In the autoimmune form of the syndrome, a certain malfunction occurs in the functioning of the immune system, as a result of which it produces antibodies (the so-called autoantibodies) that attack granulocytes, thereby causing their death. Autoimmune agranulocytosis can occur against the background of the following diseases:

• autoimmune thyroiditis;
• systemic lupus erythematosus;
• rheumatoid arthritis and other types of collagen diseases.

Systemic lupus erythematosus is one of the causes of autoimmune agranulocytosis

Agranulocytosis is also immune in nature, developing as a complication of infectious diseases, in particular:

• poliomyelitis;
• viral hepatitis;
• typhoid fever;
• yellow fever;
• malaria;
• infectious mononucleosis;
• flu.

Currently, the hapten form of agranulocytosis is often diagnosed. Haptens are chemicals whose molecular weight does not exceed 10,000 Da. These include many drugs. Haptens by themselves are not immunogenic and acquire this property only after being combined with antibodies. The resulting compounds can have a toxic effect on granulocytes, causing their death. Once developed, hapten agranulocytosis will recur every time the same hapten (drug) is taken. The most common cause of haptenic agranulocytosis is the drugs Diacarb, Amidopyrin, Antipirin, Analgin, Aspirin, Isoniazid, Meprobamat, Butadion, Phenacetin, Indomethacin, Novocainamide, Levamisole, Methicillin, Bactrim and other sulfonamides Chloroquinex, Leponex, Bartrim and other sulfonamides.

Myelotoxic agranulocytosis occurs as a result of disruption of the process of myelopoiesis in the bone marrow and associated with the suppression of the production of progenitor cells. The development of this form is associated with the effect on the body of cytostatic agents, ionizing radiation and some medications (Penicillin, Gentamicin, Streptomycin, Levomycetin, Aminazin, Colchicine). With myelotoxic agranulocytosis, the number of not only granulocytes in the blood decreases, but also platelets, reticulocytes, lymphocytes, therefore this condition is called cytotoxic disease.

Forms

Agranulocytosis is congenital and acquired. Congenital is associated with genetic factors and is extremely rare.

The acquired forms of agranulocytosis are detected with a frequency of 1 in 1300 people. It was described above that, depending on the characteristics of the pathological mechanism underlying the death of granulocytes, the following types are distinguished:

• myelotoxic (cytotoxic disease);
• autoimmune;
• haptenic (medicinal).

The genuinic (idiopathic) form is also known, in which the cause of the development of agranulocytosis cannot be established.

By the nature of the course, agranulocytosis is acute and chronic.

Signs

The first symptoms of agranulocytosis are:

• severe weakness;
• pallor of the skin;
• joint pain;
• excessive sweating;
• increased body temperature (up to 39-40 ° C).

Characterized by ulcerative-necrotic lesions of the mucous membrane of the oral cavity and pharynx, which can take the form of the following diseases:

• angina;
• pharyngitis;
• stomatitis;
• gingivitis;
• necrotization of the hard and soft palate, uvula.

Necrotic ulcerative lesions of the mucous membrane of the cavity are characteristic of agranulocytosis

These processes are accompanied by a spasm of the chewing muscles, difficulty swallowing, sore throat, increased salivation.

With agranulocytosis, the spleen and liver enlarge, regional lymphadenitis develops.

Features of the course of myelotoxic agranulocytosis

For myelotoxic agranulocytosis, in addition to the symptoms listed above, moderate hemorrhagic syndrome is characteristic:

• the formation of hematomas;
• nosebleeds;
• increased bleeding of the gums;
• hematuria;
• vomiting mixed with blood or in the form of "coffee grounds";
• visible scarlet blood in the stool or black tarry stools (melena).

Diagnostics

To confirm agranulocytosis, a complete blood count and bone marrow puncture are performed.

To diagnose agranulocytosis, a complete blood count is performed

In the general analysis of blood, severe leukopenia is determined, in which the total number of leukocytes does not exceed 1-2 x 10 ⁹ / l (the norm is 4-9 x 10 ⁹ / l). In this case, either granulocytes are not detected at all, or their number is less than 0.75 x 10 ⁹ / l (the norm is 47–75% of the total number of leukocytes).

When examining the cellular composition of the bone marrow, the following are revealed:

• decrease in the number of myelokaryocytes;
• an increase in the number of megakaryocytes and plasma cells;
• violation of maturation and a decrease in the number of cells of a neutrophilic germ.

To confirm autoimmune agranulocytosis, the presence of antineutrophil antibodies is determined.

If agranulocytosis is detected, the patient should be consulted by an otolaryngologist and dentist. In addition, he needs to undergo a three-time blood test for sterility, a biochemical blood test and an X-ray of the lungs.

Agranulocytosis requires differential diagnosis with hypoplastic anemia, acute leukemia, and HIV infection.

Treatment

Treatment of patients with confirmed agranulocytosis is carried out in the aseptic block of the hematology department, which significantly reduces the risk of infectious and inflammatory complications. First of all, it is necessary to identify and eliminate the cause of agranulocytosis, for example, to cancel the cytostatic drug.

Plasmapheresis is indicated for the treatment of agranulocytosis.

With agranulocytosis of an immune nature, high doses of glucocorticoid hormones are necessarily prescribed, plasmapheresis is performed. Shown are leukocyte transfusions, intravenous administration of antistaphylococcal plasma and immunoglobulin. To increase the production of granulocytes in the bone marrow, you need to take stimulants of leukopoiesis.

Regular mouthwash with antiseptic solutions is also prescribed.

When necrotizing enteropathy occurs, patients are transferred to parenteral nutrition (nutrients are administered by intravenous infusion of solutions containing amino acids, glucose, electrolytes, etc.).

In order to prevent secondary infection, antibiotics and antifungal drugs are used.

Prevention

Without a doctor's prescription and careful hematological control, it is unacceptable to take medications with a myelotoxic effect.

If an episode of haptenic agranulocytosis is recorded, the drug that caused its development should not be taken in the future.

Consequences and complications

The most common complications of agranulocytosis are:

• pneumonia;
• hemorrhage;
• hepatitis;
• sepsis;
• mediastinitis;
• peritonitis.

The prognosis significantly worsens with the development of severe septic conditions, as well as in the case of repeated episodes of haptenic agranulocytosis.

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Elena Minkina Doctor anesthesiologist-resuscitator About the author

Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.

Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.

The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!