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Oncohematology

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No 1 (2009)
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https://doi.org/10.17650/1818-8346-2009-0-1

HEMATOLOGIC MALIGNANCIES: TREATMENT, SUPPORTIVE CARE

5-13 206
Abstract

Protocol AML-MM-2000 treatment results of unfavorable prognostic group in children with primary acute myeloid leukemia (AML) in Russia and Belarus are presented. 105 children at the age from 2 weeks till 18 years (a median age — 10.8 years) are included in the study. In 91 patients (86.7%) hematological remission have reached, from them 34 patients (37.4%) are in the first continuous complete remission (CCR). 6-years overall survival (OS), event-free survival (EFS) and relapse-free survival (RFS) rate for all group of patients was 0.35 ±} 0.05; 0.32 ±} 0.04 and 0.43 ±} 0.05, respectively. Survival analysis of children with different cytogenetic anomalies has allowed to define two prognostic groups of patients: the intermediate prognosis, including patients with normal kariotype and t(9;11) which EFS and RFS rate was 40—50%, and unfavorable prognosis with EFS and RFS rate less than 40%. The efficiency analysis of three postremission therapy type (allogeneic and autological hematopoietic stem cells transplantation (HSCT) and chemotherapy (HT)) in intermediate and unfavorable prognostis groups was carried out. High efficiency of related HLA-identical HSCT in patients both intermediate and unfavorable prognostis groups have been shown. Autological HSCT is possible in intermediate prognosis patients with absence of HLA-identical siblings and in unfavorable prognosis patients with absence of HLA-identical unrelated donor.

14-20 669
Abstract

The purpose of this study was evalueted of a long-term treatment outcome in chronic myeloid leukemia (CML) patients in accelerated phase treated with GlivecR and determination of an optimum therapy schedule. 105 patients (men — 46, women — 59) at the age from 15 till 74 years (a median age — 40 years) enrolled between 02.2001 and 02.2007 were studied. Treatment started a dose of 600 mg/day. At the insufficient primary therapy response or loss complete hematological and/or complete cytogenetic remission in the course of treatment, dose have been increased to 800 mg/day. In 82 (78.1%) patients complete hematological remissions have been reached. In 44 (41.9%) patients complete cytogenetic response (CR) is received, and in 27 (61.4 %) of them with molecular response: complete — 17 (63%) and major — 10 (27%). 6-year overall survival rate (OS) was 61.9%, 6-year event-free survival rate (EFS) — 30.5%. Achievement of complete CR was a predictor of long longterm survival rate: OS — 95.5% versus 37.7 % (р <0.001). In case of absence CR (n=16) imatinib dose escalation allowed to receive complete CR in 5 (31.25%) and minor — in 4 (25.0%) patients. Loss or absence of complete CR on imatinib therapy not always leads to CML progression: in 18 (17.1 %) patients without complete CR hematological parameters remain normal and there are no signs of disease progression.

21-28 263
Abstract

Peripheral neuropathy is one of the most common complications of Bortezomib treatment. In this article you can find a brief literature review and single center experience: we estimated the prevalence of neuropathy development and it’s characteristics in a group of 33 patients. Peripheral neuropathy was found in 16 (48.5%) patients. In this group initial manifestations of neurotoxicity (grade I and II) were found in 8 (50%) patients. Advanced neuropathy (grade III and IV) was found in 6 (37.5%) and 2 (12.5%) patients respectively. In the majority of patients neuropathy was reversible if Bortezomibe dose was modified and symptomatic treatment was administered at the proper time.

HEMATOPOIETIC STEM CELL TRANSPLANTATION

29-38 189
Abstract

Early complications of hematopoietic stem cell transplantation (HSCT) of vascular origin within the first 30-60 days after HSCT, which are resulting from endothelial injury, are an important cause of transplant-associated morbidity and mortality. Authors describe a case report serving as an example of several endothelial syndromes development with overlapping clinical features. Risk factors and therapy approaches of these complications are discussed.

39-43 147
Abstract

Purpose: to study influence of platelet antigens system (HPA — Human Platelet Antigens) differences between donor and recipient on neutropenia and trombocytopenia duration after hematopoietic stem cells transplantation (HSCT).

Materials and methods: 40 patients at the age from 19 till 55 years (a median age ~ 28.5) after allogeneic HSCT from HLA-identical sibling are included in the study. For the majority of patients (94%) a source of haematopoetic stem cells (HSC) were a bone marrow. 28 patients received myeloablative conditioning regimens and 12 patients — a lowered intensity conditioning. Achievement of 0.5×109/l or more considered as beginning of neutrophil count recovery. As the beginning of platelet count recovery considered achievement value of 50×109/l after platelet transfusions stopping. Identification of eight allele ≪a≫ and ≪b≫ genes HPA-1,-2,-3 and-5 locuses performed by polymerase chain reaction (PCR) with use allele-specific primers. Serological HlAtyping performed by microlymphocytotoxic test with use of specific serum panels.

Results: the basic criterion of patient groups distribution (HPA-identical — 1st group; HPA-compatible — 2nd group; HPA-incompatible — 3rd group) was identity or qualitative characteristic of their differences with donor for HPA-genes. Earlier neutrophils and platelet counts recovery in patients transplanted from HPA-identical/compatible donor in comparison with patients with HPA-incompatible transplant, it is shown. In first two groups neutropenia duration was 13.45 and 14.3 days, respectively; in 3 groups — 19.0 days. This pattern of recovery was shown irrespective of conditioning regimens and leukemia type. Platelet recovery occurred earlier in patients of 1st (16.22 days) and 2nd groups (18.2 days) in comparison with patients of 3rd groups (24.2 days).

Conclusion: HPA-incompatibility can influence neutrophils and platelet recovery duration after allogeneic HSCT.

ФУНДАМЕНТАЛЬНЫЕ ИССЛЕДОВАНИЯ

44-50 247
Abstract

Human mesenchimal stem cells (MSC) growth dynamics, immunophenotype change, genetic stability on early (3—4) and late (10—12) passages at in vitro culturing was studied. Results of bone marrow stromal fibroblasts culturing (n=25) from allogeneic transplant recipients with hematological disorders are presented. It has been shown, that in MSC population cultivated in vitro linear homogeneity of cells is observed on 3—4 passage, and remains on 10—12 passage. Clonal heterogeneities of MSC population and selective advantage of certain clones during culturing was revealed as aneuploidy analysis results of cultivated cells. The MSC culturing protocol used in our work allows receiving well characterized MSC number sufficient for clinical application as early as 3—4 passage. While using in the therapeutic purposes MSC later terms of expansion it is necessary to have close control of cells immunophenotype and genetic stability, which will allow avoiding in the future the undesirable long-term consequences of MSC cultures clinical use. The obtained data are discussed and compared with literature data.

PHARMACOTHERAPY

51-56 446
Abstract

Chronic immune thrombocytopenic purpura (ITP) is one of the most frequent immune hematological disorders in which development the leading part is played by the antibodies directed against a narrow spectrum of platelet antigens. Though death rate at chronic ITP is insignificant, no more than 1%; disease strongly reduces patients quality of a life, and frequently requires difficult and expensive treatment. Antiplatelets antibodies mediating ITP, are directed against glycoprotein GP IIb/IIIa and, less often, GP Ib/IX. At ITP can be registered both raised bone marrow platelet production and accelerated peripheral blood platelet clearance, and strongly decreased production and normal platelet life-span. Antibodies to platelet membrane glycoproteins directly disturb platelet production in bone marrow. Standard drug interventions at chronic ITP - glucocorticoids in various doses, high doses intravenous immunoglobulin, anti-D immunoglobulin, rituximab - rare lead to stable increase platelet number while in 2/3 patients splenectomy is effective. The new therapy approach in chronic ITP is stimulation of platelet production in bone marrow. Recombinant fusion protein Romiplostim stimulates trombopoietin receptor and it is effective at long-term therapy in 87% patients with chronic ITP irrespective of previous splenectomy. TPO antibodies development with romiplostim therapy was not revealed.

QUALITY OF LIFE AND FOLLOW-UP

57-64 209
Abstract

In the article the international experience of vaccination in children with cancer receiving standard-dose cancer therapy is presented. Vaccination of children during and after completion of anticancer therapy is discussed. Reduction in immunity to vaccine-preventable diseases after completion of standard-dose chemotherapy that may demand reimmunization is demonstrated.

RARE DISEASES: DIFFERENTIAL DIAGNOSIS AND THERAPY APPROACHES

CORRESPONDENCE



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ISSN 1818-8346 (Print)
ISSN 2413-4023 (Online)