Agranulocytic angina is an atypical form of tonsillitis caused by a violation of granulocytopoiesis. The main clinical manifestations are represented by pyretic fever, pain when swallowing, ulcerative-necrotic changes in the oral mucosa, increased salivation, regional lymphadenopathy. Diagnosis includes the collection of anamnestic data, visual examination, laboratory blood tests and bone marrow puncture. Specific treatment requires transfusion of leukocyte mass, systemic antibacterial therapy, washing of the oral cavity with antiseptic solutions.
ICD 10
J03.9 Acute tonsillitis, unspecified
General information
The epidemiological characteristics of agranulocytic angina are closely related to the main cause of its development – agranulocytosis. The defeat of the palatine tonsils, provoked by congenital granulocyte deficiency, is rare, due to the low prevalence of this form of granulocytopoiesis disorder – 1:200,000.
Only 10% of all cases of agranulocytic angina are observed in children and young people. Angina against the background of acquired agranulocytosis occurs more often in people over 40 years of age. Women get sick 1.5-2 times more often than men. Most cases of the disease are associated with the improper use of antibacterial and cytostatic drugs.
Causes
This variant of the lesion of the palatine tonsils is not an independent disease. It is one of the manifestations of the clinical and hematological syndrome caused by the suppression of the hematopoietic function of the bone marrow. Acquired deficiency of granular leukocytes is caused by two main groups of pathogenic factors:
Myelotoxic. Ionizing radiation, including radiation therapy, certain pharmacological agents (cytostatics, beta-lactam antibiotics) and chemicals (benzene, arsenic, mercury, insecticides) can have a direct toxic effect on myelocytes (granulocyte progenitor cells) of the bone marrow.
Autoimmune. This includes infectious (malaria, infectious mononucleosis, typhoid fever, viral hepatitis, CMV infection), autoimmune (autoimmune thyroiditis, systemic lupus erythematosus, rheumatoid arthritis) diseases and medications that act as haptens (antimicrobials from the sulfonamide group, NSAIDs from the pyrazolone group). In such situations, the suppression of granulocytopoiesis is due to the production of autoantibodies.
Pathogenesis
Granulocytes are responsible for the immune response in response to the penetration of infectious agents into the human body. Agranulocytosis is manifested by a decrease in the level of granular leukocytes in peripheral blood below 0.5×109/l. Against the background of immunodeficiency, pathogenic and conditionally pathogenic microflora, penetrating into the parenchyma of the palatine tonsils, gets the opportunity for uncontrolled reproduction and spread into the surrounding tissues. Pathoanatomically, agranulocytic angina is manifested by erythematous erosive changes in the oral cavity, which quickly acquire the character of ulcerative necrotic. At the same time, there are no signs of leukocyte infiltration and suppuration.
Symptoms of agranulocytic angina
The prodromal period is 1-2 days. Angina debuts with a sharp deterioration of the general condition, chills, an increase in body temperature to 40 ° C and above. This is followed by a sore throat, which increases when swallowing. Palatine tonsils become sharply hyperemic and increase in size. Multiple ulcers and necrosis sites form on the mucous membrane of the oral cavity. Their greatest number is localized in the area of the palatine tonsils, somewhat less – on the gums and the back wall of the pharynx.
Necrotic tissues are quickly rejected, leaving deep defects. Salivation also increases and a putrid smell from the mouth appears. With the further development of agranulocytic angina, the patient’s condition remains stably severe or worsens. The mandibular, chin, anterior and posterior groups of lymph nodes increase. Moderate hepatosplenomegaly occurs. Body temperature can exceed 41 ° C.
Pain in the joints of various localization joins. There is also jaundice of the sclera and gums, which quickly spreads to the skin. In some patients, high fever and severe intoxication cause confusion or delirium. In the absence of timely treatment, complications develop rapidly, which lead to a fatal outcome.
Complications
The development of complications in agranulocytic angina is due to general immunodeficiency and generalization of infection. The most common is the spread of necrotic processes deep into the soft tissues, to the upper and lower jaw, perforation of the palate. There is a high probability of sepsis with subsequent infectious and toxic shock, peritonitis, mediastinitis and destructive changes in the intestine, bladder, liver, lungs. With the defeat of all three bone marrow sprouts (leukocyte, erythrocyte and platelet), the violation of immunity is accompanied by anemic and hemorrhagic syndromes.
Diagnostics of agranulocytic angina
The time for diagnosis with agranulocytic angina is limited. This is due to the progressive deterioration of the patient’s condition and the rapid development of dangerous complications. The greatest diagnostic value for a hematologist and ENT doctor with this disease are:
- Anamnesis. Correctly collected anamnestic data allow us to establish the probable etiology of angina (taking medications, concomitant infectious diseases, undergoing radiation therapy, etc.), to make a preliminary diagnosis.
- Pharyngoscopy. A direct examination of the pharynx and pharynx in the initial stages makes it possible to detect pronounced hyperemia of the mucous membranes and an increase in the palatine tonsils. After some time, a second examination reveals a large number of ulcers and necrosis sites with spread to the back wall of the pharynx, larynx and gums.
- Blood test. With agranulocytic angina, characteristic pathological changes are determined in the blood: the total number of leukocytes is less than 1.0 × 109 / l, neutropenia is lower than 0.5 ×109 / l, relative lymphocytosis and monocytosis. With different types of bone marrow lesions, the concentration of erythrocytes and platelets may vary.
- Examination of bone marrow punctate. With agranulocytosis, suppression of the leukocyte growth of the bone marrow is observed, which is manifested by a sharp decrease or complete absence of myelocytes. In the autoimmune variant of agranulocytosis, antineutrophil antibodies are determined. The activity of platelet and erythrocyte sprouts depends on etiological factors.
Differential diagnosis of agranulocytic angina is carried out with Simanovsky-Plaut-Vincent’s angina, aleikemic form of acute leukemia, diphtheria. With ulcerative-film angina, the general condition of the patient remains satisfactory, the temperature reaction is often absent, and ulcerative-necrotic changes occur only on one of the palatine tonsils.
Diphtheria is characterized by pronounced swelling of the neck, the absence of lymphadenopathy, the presence of a gray or pale white film on the tonsils, which is tightly soldered to the underlying tissues. Microbiological examination of the smear reveals Leffer’s wand. In the general blood test, leukocytosis is determined in the two above-mentioned pathologies. A distinctive feature of the aleikemic form of acute leukemia is the presence of a large number of blast cells in the bone marrow punctate.
Treatment of agranulocytic angina
The detection of pathology requires intensive therapeutic measures to normalize leukopoiesis, eliminate secondary infection and the influence of etiological factors. Therapy is carried out by specialists in the field of hematology and otolaryngology. Local treatment consists in regular rinsing of the oral cavity with antiseptic solutions and surgical removal of necrotic areas of the mucous membrane under local anesthesia. For systemic effects , intravenous infusion of the following groups of pharmacological agents is resorted to:
- Leukopoiesis stimulants. The main drug that is used in such cases is sodium nucleic acid. It stimulates the proliferation of myelocytes, increasing the level of neutrophilic leukocytes in the blood. If such therapy is ineffective, bone marrow transplantation is indicated.
- Blood preparations. They make it possible to make up for the shortage of shaped blood elements. In most cases, an infusion of leukocyte mass is performed. Additionally, erythrocyte and platelet mass or freshly frozen plasma are used.
- Antibiotics and antifungal drugs. They are used to fight bacterial and fungal infections, prevent the development of septic complications. Preference is given to broad-spectrum antibiotics that do not affect granulocytopoiesis.
- Donor immunoglobulins and antistaphylococcal plasma. These drugs provide the necessary immune response when infectious agents enter. They are used for the spread of infection and the development of sepsis.
- Glucocorticosteroids. They are used in the autoimmune origin of agranulocytosis, as they are able to inhibit the production of autoantibodies. Plasmapheresis is indicated when a large number of circulating immune complexes are detected.
Prognosis and prevention
The prognosis for agranulocytic angina is severe and directly depends on the modernity and usefulness of therapeutic measures. The mortality rate, depending on the form of agranulocytosis, ranges from 5 to 25%. Most of the deaths are associated with the generalization of infection and the development of severe septic complications.
There are no specific preventive measures. Nonspecific prevention consists in preventing the development of agranulocytosis. This includes the control of doses and frequency of administration of prescribed medications, restriction of contact with toxic substances, timely treatment of infectious diseases. With already developed agranulocytosis, regular rinsing of the oral cavity with antiseptics is indicated in order to prevent angina.
