The description of a rare combination syndrome Churg-Strauss syndrome (CSS) with autoimmune thrombocytopenia. The disease is characterized by slow progression and refractory to standard types of immunosuppressive therapy. The first positive results are used prolonged courses of immunosuppressive of dipeptide thymodepressin
Introduction: Current СНОР-like therapy for angioimmunoblastic T-cell lymphoma demonstrates unsatisfactory results with respect to the achievement of complete remission. It indicates the need in the search for new approaches to therapy of angioimmunoblastic T-cell lymphoma. Objective: To define the rational approaches to the diagnosis and treatment of angioimmunoblastic T-cell lymphoma (AITL). Materials and methods: Within the period from 2002 to 2012, we followed-up 15 patients with angioimmunoblastic T-cell lymphoma, with median age of 61 years (range 29-77) and the male/female ratio of 11/4. All patients had stage IV disease; the bone marrow, lungs, spleen, and skin were involved in 14 (93 %), 9 (60 %), 12 (80 %), and 6 (40 %) patients, respectively. Results: We used prolonged chemotherapy GMALL 2002 and ALL-2009 regimens for treatment of angioimmunoblastic T-cell lymphoma (11 patients). Complete remission was achieved in 55 % of cases with median follow up of 33 mouths. Conclusion: The usage of short-term chemotherapy programs (CHOP-like) for treatment of angioimmunoblastic T-cell lymphoma doesn't seem to give good results. Administration of prolonged chemotherapy is more appropriate and allows achieving remission of the disease.
A combination of two rare diseases: T-cell lymphoblastic lymphoma (T-LBL) and chronic eosinophilic leukemia (CEL) with chromosomal anomaly is presented in this article. A patient is a man 33 years old. Areas of defeat involve all the lymphatic nodes, the liver, the spleen, the anterior mediastinum, the pleura and the lungs. Immunophenotype of atipical lymphocytes: CD10+, CD^+, CD7+, CD2+, CD43+, CD3-, CD5+, CD4+, CD8+, C38+. Ki-67 77 %. Eosinophilia reveals in the blood and the bone marrow. The diagnosis CEL is verified by means of molecular genetic research (the chimeric gene FIP1L1-PDGFRA). Complete remission is received for T-LBL by means of polychemotherapy within 2.5 years and for CEL by means of gliveс (Imatinib). Literary data on the new differentiation of long existing hypereosinophilia: the hypereosinophilic syndrom and the chronic eosinophilic leukemia (the new WHO classification).
Results of treating a group of patients with T-cell skin lymphoma are presented in article. The diagnosis mycosis fungoides (MF) established in 48 patients (24 men and 24 women). The median of age 52 years (26-77 years). The diagnosis syndrome Sezary (SS) established in 20 patients (men 12, women 8). The median of age was 61 year (23-87 years). Half of patients have arrived under supervision of our clinic with III-IV stages of disease. Cytostatic therapy does not lead to radical treatment. The positive result of treatment consists in reception of complete or partial remission or stabilization of illness within several months or years and improvement of quality of life of patients. The median follow up of patients with MF makes 85 months, at patients with SS 60 months. The median of survives of patients with MF makes 199.2 months, at patients with SS 75.3 months. The carried out researches in patients of the presented group have shown, that early application of intensive chemotherapy is irrational, as quite often leads to septic complications Alpha interferon (IFN-а) is one of the most active biological agents in therapy MF and SS, especially in the first stages of disease. IFN-а can be applied as monotherapy or in a combination to beam therapy, retinoids and cytostatics.
AIM:To study influence of thymodepressin on the course of autoimmune cytopenia.MATERIAL AND METHODS:Thymodepressin is a new synthetic hemoregulatory dipeptide (gamma-D-Glu-D-Trp). It was used for the treatment of 22 patients with autoimmune cytopenia.RESULTS:Hemoglobin levels were elevated in autoimmune hemolytic anemia and platelet levels were high in idiopathic thrombocytopenic purpura. A thymodepressin course resulted in a fall of total lymphocyte count and activated CD3+CD69+ lymphocytes.CONCLUSION:The above results, safety, absence of toxicity and allergenicity, parenteral and intranasal useability open perspectives for further studies of therapeutic action of thymodepressin as an immunodepressant in autoimmune processes.
The effect of the synthetic peptide IEW (Neogen) with immunomodulating properties on postradiation recovery of haemopoiesis was investigated. We have shown that Neogen is a potential stimulator of haemopoiesis. The administration of Neogen after irradiation shortened duration of period of the recovery of the compartment of CFU-S-8 and the amount of bone marrow cells. The comparision of the effects of Neogen and GM-CSF (Leucomax) and G-CSF (Granocyte 34) have shown that the targets for these agents are probably different: polypotent CFU-S-for Neogen, and CFU-GM-for GM-CFS. Based on the results, we suggested the mechanism of Neogen effects on heamopoiesis.
Th distribution of autoimmune thyroiditis in the patients with diseases of blood system was investigate. The attribute of autoimmune thyroiditis was revealed by the detection of antimicrosomal antibodies. It was established that the autoimmune thyroiditis are more often in patients with various hematological diseases than in control group. It is supposed that the increase in frequency of some hematological diseases in residents suffered from the Chernobyl accident can be defined not only by the influence of the radiation on blood system, but also can be connected with damage to thyroid glands.
Distribution of autoimmune thyroidites in the patients with diseases of blood system is investigated. Antimicrosomal antibodies served as an indicator of autoimmune thyroidites. Autoimmune thyroidites was found more often in patients with various hematological diseases than in the control group. A hypothesis is advanced that the increase in the frequency of some hematological diseases in the residents affected by the Chernobyl accident can be attributed not only to the influence of the radiation on blood system, but also to the damage of thyroid glands.
An important peculiarity of the Chernobyl catastrophe is the discharge into the atmosphere of tremendous amount of radioactive iodine and, as a result, selective damage of the thyroid in children from the affected areas. The most dangerous consequence is the thyroid cancer. The analysis of the situation when children's thyroids were subjected to irradiation shows that tumors can most frequently develop as late as 20-30 years after irradiation. There are reasons to believe that tumors are induced by low dose of irradiation. The most important factor in development of pathologies is for sure the age of the children of the moment of irradiation. A well-known consequence of the impact of radiation on the thyroid is the lymphocyte thyroiditis. The interest to this pathology is determined by the fact that it substantially increases the probability of development of various haematologic diseases (lympho- and myeloproliferative neoplasms).
Secretory serum immunoglobulins were studied in 25 patients with chronic lymphoid leukemia. The relationship between the lowering of the content of serum immunoglobulins and the disease stage was revealed as was the interrelationship between the rate of bacterial complications and the decrease in the immunoglobulin content. The content of serum and secretory IgA in the saliva of all the patients was found to be normal or elevated whatever the disease stage.