Agranulocytosis

Immune agranulocytosis

The disease is characterized by a sharp decrease or absence in peripheral blood leukocytes grained.

Currently, agranulocytosis conventionally assumed reduction granulocyte up 0,75 T in 1 L or leukocytes to 1 T in 1 l.

Women suffer most agranulocytosis, than men, Children less, than adults.

According to the mechanism of release two forms of immune agranulocytosis:

• Hapten agranulocytosis;
• Autoimmune agranulocytosis.

Hapten agranulocytosis

The disease is caused by the appearance of antibodies against granulocytes. Antigen-antibody reaction occurs in the presence of the hapten, whose role is often performed medicines.

Antigranulotsitarnye antibodies exert their effect in the peripheral blood at the beginning, resulting in the death of mature neutrophils, and then a young granulocyte, until granulocyte progenitor cells in the bone marrow of a number.

At the heart of the development of this form of agranulocytosis is hyperergic reaction of the organism in response to the receipt of a drug.

Razvitiyu gaptenovogo agranulocytosis may contribute to various drugs: aminazin (chlorpromazine), amidopirin, antidiabetic sulfa drugs, antipirin, acetylsalicylic acid, barbiturates, ʙutadion, Indomethacin, Isoniazid, insekticidы, klozapyn, levazol, levomicetin, Biseptol (trimetaprin), phenacetin, pipolfen, Diacarbum etc..

The development of clinical drug agranulocytosis may vary depending on the calling medicament. Medicinal agranulocytosis different acute onset with fever up to 38-39 ° C, the appearance of angina, stomatitis, in some cases the development of candidiasis of the oral mucosa, throat, esophagus. In severe cases,, sometimes during relapse, agranulocytosis oslozhnyaetsya nekroticheskoy эnteropatiey.

Changes in peripheral blood and the speed of their development are directly dependent on the severity of the disease. Typical quickly narastayuschaya leukopenia (1-2 F in 1 l) by reducing the number of neutrophils in the peripheral blood, until their complete disappearance. There may be monotsitopeniya. The number of eosinophilic granulocytes can be normal. In the cytoplasm of the remaining single neutrophilic granulocytes is found toksogennaya grain. Phagocytic activity of neutrophils is reduced, kinetics gee impaired.

Cytochemical studies can detect a decrease in glycogen content in neutrophil granulocytes. The smear a very small number of cells, they are represented mainly by lymphocytes (relative lymphocytosis). Sometimes detected in peripheral blood cells and single reticular plasmocytes. Anemia and thrombocytopenia are observed. In some cases, it may be a slight reduction in the number of platelets; hemorrhagic syndrome in hapten agranulocytosis does not happen. In severe disease can develop anemia.

On exit from a granulocytosis often arises leukemoid reaction with the number of leukocytes in peripheral blood before 30 T in 1 l and more, the appearance of blast cells, promyelocytes etc.. This pattern is short-term, it can be observed within a few hours. Then, as the recovery of the patient, normalized blood counts.

Changes in the bone marrow are to depletion of its cellular composition due to a sharp decrease in the number of granulocytes. First fade stab and segmented form, and metamyelocytes and myelocytes Reserved, and the number of recent increases markedly, appearing cells in mitosis. Such myelocytic marrow soon replaced promyelocytic; granulocytic cell number in these cases are presented primarily promyelocytes. In the same period in the peripheral blood revealed pronounced leukopenia.

With increasing severity of the disease the bone marrow is depleted not only granulocytes (disappear and promyelocytes), but erythrokaryocytes and germ cells of megakaryocytic. The sternum punctate found in a small number of plasma cells and reticular, macrophages, lipofagi, isolated granulocytes, erythro- kariotsity and megakaryocytes. This bone marrow is able even to full recovery, but further progression of the disease may develop panmieloftiz.

For Pathomorphology agranulocytosis is characterized by the lack of demarcation of the shaft on the periphery of necrotizing areas. There are no signs of inflammation, despite the presence of abundant bacterial flora, No leukocyte reaction and festering. On the border with necrotic areas of a small number of monocytes, lymphocytes and plasma cells.

The duration of drug immune agranulocytosis 1.5-2 weeks, sometimes more.

Autoimmune agranulocytosis

Autoimmune agranulocytosis in systemic connective tissue diseases, leukemia and other diseases develops slowly and is characterized by a prolonged course.

Citostaticheskaя boleznь

The disease was first described by A. AND. Vorobiev in 1965 g. It arises as a result of effects on the body of powerful cytotoxic drug or radiation factors of nature, causing the death of dividing cells of the bone marrow, epithelium of the alimentary canal, skin. It can affect the liver.

Established, that damage to normal bone marrow cells require large doses of cytotoxic drugs, Damage than leukemic cells, especially in the terminal period. Cytotoxic drugs (merkaptopurin, cyclophosphamide, tioTЭF, vynkrystyn, rubomycin et al.), as well as the exposure of patients, especially total, They are often the cause of the disease with cytotoxic agranulocytosis.

Even before the onset of clinical signs of disease in patients with reduced white blood cell count, Platelet and reticulocyte. Leukopenia may develop severe - up to 0.5-1.0 G 1 l. The number of neutrophils decreases sharply, until their complete disappearance. There may be, and anemia. Although thrombocytopenia, bleeding is rare.

In the bone marrow punctate sharply reduced the number of not only the granulocytes, and megakaryocytic cells and germs eritrokariotsitarnogo. Reserved lymphoid, retikulyarnыe and plazmaticheskie cells.

Against the background of pronounced changes in peripheral blood and bone marrow appear fever, sore throat, stomatitis, sometimes bleeding gums, nosebleeds, bleeding into the skin. In other cases, it affected only the mucosa oral cavity and pharynx.

The reason for the defeat of the mucous membranes in myelotoxic agranulocytosis is weakening the body's defenses, in connection with which it is possible to effect pathogens. Besides, cytotoxic drugs affect all proliferating cells, including the mucosa of the alimentary canal. These changes mucosa can last from one to two weeks to a month or more. Under the influence of cytostatics, and especially after radiation therapy, may develop necrotic enteropathy, that, how and agranulocytosis, complicates the course of leukemia and its treatment.

Improve the patient's condition is preceded by the appearance of a large number of bone marrow blast cells and promyelocytes. In peripheral blood found metamyelocytes, myelocytes, monotsitы and plazmaticheskie cells.