Agranulotsitarny quinsy – the atypical form of tonsillitis caused by violation of a granulotsitopoez. The main clinical manifestations are presented by piretichesky fever, pain when swallowing, ulcer and necrotic changes of a mucous membrane of a mouth, the increased salivation, a regional limfadenopatiya. Diagnostics includes collecting anamnestichesky data, visual survey, laboratory blood tests and a puncture of marrow. Specific treatment demands transfusion of leykotsitarny weight, performing system antibacterial therapy, washing of a mouth solutions of anti-septic tanks.
The epidemiological characteristic of agranulotsitarny quinsy is closely connected with the main reason for its development – agranulotsitozy. The defeat of palatal almonds provoked by congenital deficiency of granulocytes meets seldom that is caused by low prevalence of this form of violation of a granulotsitopoez – 1:200000. Only 10% of all cases of agranulotsitarny quinsy are observed at children and young people. Quinsy against the background of the acquired agranulotsitoz arises at persons more often 40 years are more senior. Women are ill 1,5-2 times more often than men. The most part of cases is connected with misuse of antibacterial and cytostatic medicines.
Reasons of agranulotsitarny quinsy
This option of defeat of palatal almonds is not an independent disease. It represents one of manifestations of the kliniko-hematologic syndrome caused by oppression of the haematogenic function of marrow. The acquired deficiency of granular leukocytes is caused by two main groups of pathogenic factors:
- Miyelotoksicheskiye. Can make direct toxic impact on miyelotsita (a cage predecessors of granulocytes) of marrow ionizing radiation, including at radiation therapy, certain pharmacological means (cytostatics, β-laktamy antibiotics) and chemicals (benzene, arsenic, mercury, insecticides).
- Autoimmune. Here diseases and medicines which work as gapten belong infectious (malaria, infectious , a typhoid, viral hepatitis, the TsMV-infection), autoimmune (autoimmune tireoidit, system red a wolf cub, rheumatoid arthritis) (antimicrobic medicines from group of sulfanylamides, NPVS of group of a pirazolon). In such situations oppression of a granulotsitopoez is caused by development autoantitet.
Granulocytes are responsible for immune reaction in response to penetration of infectious agents into a human body. Agranulotsitoz is shown by decrease in level of granular leukocytes in peripheral blood below 0,5×109/. Against the background of an immunodeficiency pathogenic and opportunistic microflora, getting into a parenchyma of palatal almonds, has an opportunity of uncontrolled reproduction and distribution to surrounding fabrics. Pathoanatomical agranulotsitarny quinsy is shown by eritematozno-erosive changes of a mouth which quickly gain character of ulcer and necrotic. At the same time there are no signs of leykotsitarny infiltration and suppuration.
Symptoms of agranulotsitarny quinsy
The Prodromalny period makes 1-2 days. Quinsy debuts sharp deterioration in the general state, a fever, temperature increase of a body up to 40 °C and above. Afterwards there is a sore throat amplifying when swallowing. Palatal almonds become sharply hyperemic and increase in sizes. On a mucous membrane of an oral cavity multiple ulcers and sites of a necrosis are formed. Their greatest number is localized in the field of palatal almonds, it is slightly less – on gums and a back wall of a throat. Nekrotizirovanny fabrics are quickly torn away, leaving deep defects. Also salivation increases and the putrefactive smell from a mouth appears.
With further development of agranulotsitarny quinsy the condition of the patient remains steadily heavy or worsens. Mandibular, podborodochny, perednesheyny and zadnesheyny groups of lymph nodes increase. There is a moderate gepatosplenomegaliya. Body temperature can exceed 41 °C. Pain in joints of various localization joins. Also there is a yellowness a skler and gums which quickly extends to integuments. High temperature and the expressed intoxication cause confusion of consciousness or nonsense in some patients. In the absence of timely treatment complications which lead to a lethal outcome quickly develop.
Development of complications at agranulotsitarny quinsy is caused by the general immunodeficiency and generalization of an infection. Distribution of necrotic processes deep into of soft fabrics, on the top and lower jaw, perforation of the sky is most often observed. The probability of sepsis with the subsequent infectious and toxic shock, peritonitis, mediastinity and destructive changes in intestines, a bladder, a liver, lungs is high. At defeat of all three sprouts of marrow (leykotsitarny, eritrotsitarny and trombotsitarny) violation of immunity is followed anemichesky and hemorrhagic by syndromes.
Time for diagnostics at agranulotsitarny quinsy is limited. It is connected with the progressing deterioration in a condition of the patient and bystry development of dangerous complications. Have the greatest diagnostic value for the hematologist and the ENT specialist at this disease:
- Anamnesis. Correctly collected anamnestichesky data allow to establish a probable etiology of quinsy (reception of medicines, the accompanying infectious diseases, passing of radiation therapy etc.), to make the preliminary diagnosis.
- Faringoskopiya. Direct survey of a throat and pharynx at initial stages gives the chance to find the expressed hyperaemia of mucous membranes and increase in palatal almonds. After a while at repeated survey a large number of ulcers and sites of a necrosis with distribution on a back wall of a throat, a throat and gums comes to light.
- General blood test. At agranulotsitarny quinsy in blood characteristic pathological changes are defined: the total quantity of leukocytes are less 1,0×109/, the neytropeniye is lower 0,5×109/, relative and . At various types of damage of marrow concentration of erythrocytes and platelets can vary.
- Research of a punktat of marrow. At an agranulotsitoza oppression of a leykotsitarny sprout of marrow is observed that is shown by sharp decrease or total absence of miyelotsit. At autoimmune option of an agranulotsitoz antineytrofilny antibodies are defined. Activity of trombotsitarny and eritrotsitarny sprouts depend on etiologichesky factors.
Differential diagnosis of agranulotsitarny quinsy is carried out with quinsy of Simanovskogo-Plauta-Vensana, an aleykemichesky form of a sharp leukosis, diphtheria. At ulcer and film quinsy the general condition of the patient remains satisfactory, temperature reaction often is absent, and ulcer and necrotic changes arise only on one of palatal almonds. The expressed neck hypostasis, lack of a limfadenopatiya, existence of a gray or pale white film on almonds which is densely soldered to the subject fabrics is characteristic of diphtheria. At a microbiological research of dab Leffer's stick comes to light. In the general blood test at two above-mentioned pathologies is defined . Distinctive feature of an aleykemichesky form of a sharp leukosis is existence big quantity of blastny cages in a marrow punktata.
Treatment of agranulotsitarny quinsy
Detection of pathology demands intensive therapeutic measures for the purpose of normalization of a leykopoez, elimination of a secondary infection and influence of etiologichesky factors. Therapy is performed by experts in the sphere of hematology and otolaryngology. Local treatment consists in regular rinsing of a mouth antiseptic solutions and surgical removal of nekrotizirovanny sites of a mucous membrane under local anesthesia. For system influence resort to intravenous infusion of the following groups of pharmacological means:
- Stimulators of a leykopoez. Osnovny medicine which is used in such cases – nukleinovokisly sodium. It stimulates proliferation of miyelotsit, increasing the level of neytrofilny leukocytes in blood. At inefficiency of similar therapy transplantation of marrow is shown.
- Blood medicines. They allow to fill shortage of uniform elements of blood. Infusion of leykotsitarny weight is in most cases carried out. In addition use the eritrotsitarny and trombotsitarny weight or freshly frozen plasma.
- Antibiotics and antifungal medicines. Are used for fight against a bacterial and fungal infection, prevention of development of septic complications. Preference is given to the antibiotics of a broad spectrum of activity which are not influencing on .
- Donor immunoglobulins and anti-staphylococcal plasma. These medicines provide the necessary immune answer at penetration of infectious activators. Are applied at spread of an infection and development of sepsis.
- Glucocorticosteroids. Are used at an autoimmune origin of an agranulotsitoz as are capable to oppress development autoantitet. At identification of a large number of the circulating immune complexes the plasma exchange is shown.
Forecast and prevention
The forecast at agranulotsitarny quinsy heavy and directly depends on the present and full value of therapeutic actions. The mortality indicator depending on a form of an agranulotsitoz makes from 5 to 25%. The most part of lethal outcomes is connected with generalization of an infection and development of heavy septic complications. Specific preventive measures do not exist. Nonspecific prevention consists in prevention of development of an agranulotsitoz. Here control of doses and frequency rate of reception of the appointed medicines, restriction of contact with toxic substances, timely treatment of infectious diseases belong. At already developed agranulotsitoz for the purpose of prevention of quinsy rinsing of a mouth is shown by antiseptics regular.