The fatal disease Evans Fischer Syndrome. Fisher-Evans syndrome. Inflammation of the tubules and lacrimal sac

Fisher-Evans syndrome is a combination of autoimmune hemolytic anemia with autoimmune thrombocytopenia.

There are two forms of Fisher-Evans syndrome:

1. symptomatic;
2. idiopathic.

In diseases such as chronic hepatitis, systemic lupus erythematosus, rheumatoid arthritis, chronic lymphocytic leukemia, lymphoma, sarcoma, lymphogranulomatosis, a symptomatic form of the syndrome is observed. Sometimes this syndrome is the first signal of upcoming diseases. The idiopathic form of hemolysis (destruction of red blood cells) and thrombocytopenia is not associated in any way with other diseases. In Fischer-Evans syndrome, the destroyed number of red blood cells and platelets is taken into account. This is due directly to the fixation on their surface - autoantibodies. Autoantibodies contact in a peculiar way with the Rh antigens of blood cells, and in rare cases against antigens of other systems. Platelets and erythrocytes are subject to decay in such organs as: spleen, bone marrow, liver. Therefore, in bone marrow there is an increase in the number of erythroid cells and megakaryocytes.

If a we will talk about the clinical picture, it is first of all worth noting the appearance of anemia, high reticulocytosis and indirect hyperbilirubinemia, which are combined with thrombocytopenia. The first symptoms of the disease are pain in the joints and abdomen, fever body. Subsequently, symptoms such as weakness, shortness of breath appear, and bleeding into the skin or petechial hemorrhages on the skin. In addition, nosebleeds, bleeding in the oral cavity, and uterine bleeding. Sometimes hemorrhagic syndrome is a precursor of clinical and laboratory signs destruction of erythrocytes. Sometimes thrombocytopenia and anemia occur simultaneously. In addition, thrombocytopenia can manifest itself in a patient after several years, after surgical removal spleen. In this case, the reason for the removal of the spleen can be autoimmune hemolytic anemia.

The disease is diagnosed using a direct Coombs test, which confirms the autoimmune nature of hemolysis. Moreover, even a negative result on the Coombs test does not indicate that the patient does not experience immune hemolysis.

In order to establish the cause of hemolysis, it is necessary to investigate whether there are such deviations as: increased content in the blood of reticulocytes, shortening of the life of erythrocytes, an increase in the bone marrow of erythroid cells.

For treatment in practice, glucocorticoid hormones are prescribed, and if the result given treatment does not bring, doctors recommend removing the spleen. If the operation did not change the situation, doctors prescribe cytostatics (azathioprine, cyclophosphamide, vincristine) together with glucocorticoids. With this disease, even platelet transfusion will be ineffective.

Evans Syndrome (SE) is a very rare disease, combining the symptoms of autoimmune hemolytic anemia and thrombocytopenia. Includes symptomatic and idiopathic varieties.

The cause of the symptomatic form of SE is:

• chronic hepatitis,
• systemic diseases connective tissue,
• oncohematological diseases,
• oncological diseases lymphatic system,
• malignant tumors connective tissue,
• tuberculosis.

The causes of the idiopathic form of this syndrome are not yet known. However, it has been established that in both types of the disease, destruction of erythrocytes and platelets occurs due to the fixation of autoantibodies on their surface with subsequent utilization in the organs. hematopoietic system. SE is fraught infectious complications, hemorrhages, often fatal.

Diagnostics

In addition to a distinct clinical symptoms, the diagnosis of the disease is confirmed by laboratory data:

• anemia, thrombocytopenia, high reticulocytosis;
• indirect hyperbilirubinemia;
• direct Coombs test;
• increase in the number of erythroid cells and megakaryocytes in the myelogram.

Evans Syndrome Treatment

Includes assignment systemic GCS(prednisolone, dexamethasone) and cytostatics (aza-thioprine, cyclophosphamide, vincristine). In some cases, a splenectomy is performed. In severe anemia, red blood cell transfusions are carried out. Platelet transfusions are not effective. Drug use should be avoided acetylsalicylic acid, antithrombotic agents. Sun exposure is not recommended. Given the likelihood of complications, the prognosis of SE is not favorable.

Essential drugs

There are contraindications. Specialist consultation is required.

(J.A. Fisher; R.S. Evans, contemporary American physician)

see Evans syndrome.

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"Fischer - Evans Syndrome" in books

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