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To ascertain efficacy of modern treatment methods in idiopathic thrombocytopenic purpura (ITP), to characterize basic indications for administration of these methods; perspective trends in ITP therapy.The article presents 20-year experience in observation and treatment of 1000 ITP patients gained from 1988 by the department of standardization of blood disease treatment methods on the basis of a consultative and outpatient department of Hematological Research Center. The results were assessed by conventional ITP treatment lines.First-line therapy--glucocorticoids--provided remission in 70% patients, long-term (60 months and longer) in 14%, prednisolone resistance was in 19% patients.Intravenous immunoglobulin provided a rapid hemostatic effect (in 1-2 days) in all the patients. A positive response to treatment was seen in 86% patients but it was short-term (for a year and longer in 27%, resistance to the drug--14%). Second line treatment--splenectomy--is the most effective treatment: 80% remission, 32% patients had remission for longer than 60 months. Resistance occurred in 6% patients. Ineffectiveness of treatment in 20% patients stimulated the search for new pathogenetic treatment among synthetic analogues of thrombopoietin. Clinical trials proved high efficacy of two of them--eltrombopag and romiplostim (86-87% response), possible maintenance of remission in long-term interrupted administration of low doses.Modern ITP treatment allows a complete management of hemorrhagic syndrome and deep thrombocytopeny in 80% patients with provision of good quality of life and ability to work. Introduction into clinical practice of thrombopoietin analogues improves treatment results including in 20% patients resistant to all treatments in the absence of marked side effects even in long-term 3-year administration of such medication.

To evaluate efficacy and tolerance of glivek in chronic myeloid leukemia (CML) in patients who failed interferon-alpha (If-a) preparations.79 patients in a chronic phase of Ph + CML with hematological and cytogenetic resistance or intolerance of If-a. The response to glivec was assessed by achievement of a complete hematological remission and the cytogenetic effect (the degree of reduction of cell clone Ph+ in bone marrow). Tolerance and safety of the drug was studied by monthly standard clinicohematological tests.Not only a hematological remission (92.4%), but also partial (46.8%) or complete (27.8%) elimination of BCR-ABL +/- cells were achieved after 12 months of the treatment. Glivec was well tolerated. Hematological toxicity primarily as neutropenia and thrombocytopenia were observed in 54.4 and 42% patients, respectively. Neutropenia of the third degree which made impossible to continue the treatment was observed in 29.1% patients; throbocytopenia of the third degree was registered in 16.5% patients. Among most frequent non-hematological side effects there were moderate edema, nausea, leg muscle convulsions, weight gain, arthralgias, skin eruption. All the complications were transient, were managed in all cases with only a short-time discontinuation of glivec therapy.High activity of glivec at early stages of CML allows using this drug as a first-line therapy in patients with CML.

According to the classification of chronic lymphocytic leukemia (CLL) proposed by A. I. Vorob'ev and M. D. Brilliant in 1983, benign CLL is a distinct form of CLL which is characterized by low level of absolute lymphocytosis, absent or mild peripheral lymphadenopathy, slow progression. No specific therapy is needed. The paper presents clinical, morphological and immunological analysis of 34 cases of benign CLL (17 males and 17 females, mean age 58 years). Patients were included in the study if they had lymphocyte count less than 30,000 and no significant growth of lymphoid tissue for at least 3 years. They were followed up from 3 to 24 years (11 years, on the average). The main features of benign CLL are the following: no "B" symptoms, no essential enlargement of the lymphoid organs, a stable low level of absolute lymphocytosis, low prolymphocyte count in the blood smear (0.95% +/- 0.2), nodular or nodular-interstitial proliferation in the bone marrow. We failed to find any cases with paraprotein secretion. There was immunophenotype typical for CLL in 91% of cases (CD19+, CD20+, CD23+, CD5+, EM+, CR1-/CR2+, sIg+(-)). None was positive for CD38 activation marker. One trisomy 12 cases was detected by FISH method. 8 patients died so far, but not because of the tumor progression or transformation, median survival was 22 years.

The paper presents new findings in favor of recognition of splenic lymphocytoma (SLC). This disease was characterized by A. I. Vorob'ev and M. D. Brilliant in 1982 in terms of detailed clinicomorphological features, prognosis and optimal treatment policy. The study included 52 patients (mean age 53 years) of which 36 were females and 16 males. They were followed up for 5.7 years, on the average. SLC manifested clinically by splenomegaly with minimally enlarged lymph nodes, morphologically by nodular lymphocytic proliferates in the spleen, bone marrow and liver, diffuse or diffuse-nodular proliferation in the lymph node. Peripheral blood contained middle-size lymphoid cells with round nuclei. SLC immunophenotype exhibits moderate or marked expression of CD22 and membrane immunoglobulins, the absence of CD5, CD23 and EM receptor, combination of CR1-/ CR2+. Paraprotein secretion was recorded in 49% of cases. There were frequent autoimmune reactions, especially against erythroid cells and platelets (42%). Optimal therapeutic policy is expectation and eventual splenectomy producing a persistent clinical effect in 94% of patients. In progressive disease long-term therapy with cyclophosphamide is recommended. Thus, SLC is a mature-cell lymphatic tumor growing as a rule in the spleen. Its prognosis in valid therapy is favourable.

Patients with idiopathic thrombocytopenic purpura (ITP) tolerated well plasmapheresis. In no cases hemorrhage intensification was observed. An immediate clinical effect was recorded in all patients that correlated with the immunological parameters improvement in most of them. The platelet count significantly rose only in one patient immediately after the plasmapheresis course. The follow-up of the patients during 11-20 months has enabled revealing not only immediate clinical but also late hematological effect of plasmapheresis, that was manifest in a delayed rise of the platelet levels in most patients, and abolished resistance to glucocorticoids in one of them. It has been concluded that plasmapheresis produces immunocorrecting effect and could be used for the treatment of ITP patients.