HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CARE
Standard therapy of high risk patients with Langerhans cell histiocytosis (LCH) is associated with essential risk of therapy failure. In the present study results of 2-clorodeoxyadenosine usage as alternative therapy in this group of patients are analyzed. In this study 31 patients with multisystem form LCH with involving of organs at risk were included. Nineteen patients have received Prednisone and Vinblastine as front line therapy (standard group). Nine patients have received therapy with 2-CdA and cytosine-arabinoside (group 2-CdA). The patients in standard group statistically significant more often (55.5 % vs. 0 %, р = 0.0095) required second line therapy. Permanent disease conse-quences was more often developed in standard group (63.6 % vs. 0 %, р = 0.0147). The overall survival was 65.7 ± 12,8 % among standard group patients and 88.8% ±10% in 2-CdA group; p = 0.27. The event-free survival was in standard group 33.9 ± 12 %, in 2-CdA group – 88.8 ± 10 %, p = 0.0207. Severe hematological toxicity was observed at 2-CdA therapy. 2-CdA therapy is highly effective in treatment of high risk patients with LCH. The drug should be used only in a specialized hospital.
In given article non-Hodgkin lymphomas epidemiology, main attempts of gastrointestinal tract (GIT) lymphomas sistematization, basic methods of stomach NHL diagnostics, such as radiologic and endoscopic are described. Also importance of modern endoscopic diagnostics methods is discussed: chromogastroscopy, magnifying and narrow spectral endoscopy. Morphological confirmation problems of neoplasm histologic structure using forceps biopsies during endoscopic examination are noted; possibilities of expanded procedures taking a sample, such as endoscopic resection mucous and submucosal layers are described. Possibilities and importance of endosonography both in diagnostics and in differentiation of NHL and a various malignant and non-malignant stomach pathology are in detail described.
In this article mechanisms of anaemia development in patients with lymphatic tissue malignant diseases are presented. Therapy efficacy of erythropoiesis-stimulating agents (EPO) in patients with lymphoproliferative disorders and anaemia (n = 21) is studied. Study group included patients with a chronic lymphoid leukemia (n = 5), indolent lymphomas (n = 7) and multiple myeloma (n = 9). Patients were 49–80 years of age (63.6 ± 8.2 years). The positive response to EPO therapy was considered if hemoglobin level increased on 20 g/l or its rising to 120 g/l. With given therapy hemoglobin level was increased from 86.8 ± 18.5 g/l to 111.4 ± 26.5 g/l (p < 0.001). EPO therapy efficacy was 61.9 % for total group of patients. Importance of TNF-alpha detection as the prognosis factor of EPO therapy efficacy was studied. Patients with low level of TNF-alpha (less than 15 pg/ml) achieved the positive response in 92.9 %, with a high level of TNF-alpha (more than 15 pg/ml) – the positive response was not observed. Inversely proportional correlation between initial TNF-alpha level and therapy response has been established (r = -0.487; p < 0.03; n = 21). Thus, detection of TNF-alpha level in patients with lymphoproliferative disorders before EPO therapy allows to predict therapy response with high degree of significance.
The aim of the study was to assess the prevalence of cancer predisposition syndromes among children with cancer in Moscow Region (MR).
The data on patients were retrieved from the database of Childhood Population-based Cancer Registry of MR. 35 (3.0 %) children with cancer predisposition syndromes were revealed among 1173 registered from 2000 till February 2010. The most prevalent syndromes were hereditary retinoblastoma (RB) – 9 (25.7 %), Down syndrome – 8 (22.8 %) and neurofibromatosis type 1–8 (22.8 %). Genetic syndromes were observed in patients with retinoblastoma – 31 %, germ-cell tumors – 6.8 % and soft tissue sarcomas (STS) – 4.5 %. The increased risk of development of leukemia in patients with Down syndrome was observed. The relative risk (RR) of 15.0 (95 % CI 1.9–333.1) for leukemia and 57.1 (95 % CI 9.3–1398.0) for AML was observed. The association of neurofibromatosis type 1 with the development of tumors of central nervous system and soft tissue sarcomas was proved. The RR was 62.5 (95 % CI 8.1–1388.6) for CNS tumors and 150,0 (95 % CI 26.9–3480.9) for STS.
New guidelines on diagnosis and treatment of a primary immune thrombocytopenia (ITP) in children and adults, on the basis of modern pathogenesis conceptions and considering possibilities of hematological practice in Russia are presented. Guidelines are based on a consensus of international and Russian experts on ITP. Diagnostic features of ITP are noted and various I, II and III lines treatment, including new effective medical products which have recently appeared in Russia, in particular, trombopoietin receptor agonists (romiplostim, eltrombopag), considerably expanded conservative treatment possibilities are offered. The presented guidelines can help hematologists to develop individual schedule for each given patients with ITP and to improve patients quality of life.
PHARMACOTHERAPY
Chronic lymphocytic leukemia (CLL) is the most frequent leukemia type in adults. Despite significant achievements in therapy of this disease in young adults, treatment of elderly patients represents the problem, first of all, because of the unsatisfactory acceptability of the regimens containing purine analogues. The new drug – ribomustin – combining properties of alkylating agents and purine analogues is registered in 2010 in Russia. The major advantage of ribomustin is its low toxicity. In several researches it is shown, that ribomustin is effective both in relapse and as front line therapy in CLL patients. Results of clinical studies of ribomustin in CLL patients are discussed in the given review.
The aim of the given study is investigation of influence onosteodestruction markers and quality of life parameters of therapy with generic preparation of pamidronic acid (pomegara) in patients with multiple myeloma (MM) and lytic bone lesions.
For this purpose prior to the beginning of the study and every 4 week after pomegara injection throughout 16 weeks blood СТХ (terminal crosslinking telopeptide of type I collagene) concentration and urine DPD (deoxypyridinoline) were detected. In addition monitoring of tumor symptoms intensity using MDASI (M.D. Anderson Symptom Inventory) and FACT (Functional Assessment of Cancer Therapy) questionnaires was conducted and pain intensity was also investigated using analogue scale. Study is finished in eighteen patients. Bone resorbtion markers (serum СТХ and urine DPD) have essential decreased after fourth pomegara injection to 33 % of median initial value for CTX (р < 0.05) and to 73 % – for DPD (р=ns). Statistically significant increasing of integrated quality of life parameter in comparison with initial value is registered by 12th week of treatment (р < 0.05). The majority of pomegara side effects were mild and moderate severity and preparation cancelling has not necessary. The frequency and spectrum of complications (fever, skeletal and muscular pain, etc.) corresponded to the similar parameters revealed in large controlled studies of bisphosphonate treatment in MM. Careful monitoring of renal function during pomegara treatment has not shown significant negative effect, including patients with initial renal involvement. According to data received in this restricted volume study we can conclude that pomegara treatment in patients with MM and lytic bone lesions result in decrease of bone destruction, quality of life improvement and decrease severity of pain. Drug acceptability did not principally differ from original pamidronate shown in controlled studies.
HEMATOPOIETIC STEM CELL TRANSPLANTATION
Epstein–Barr virus (EBV) is a member of herpes-viruses family. Now it is well-known, that it can be a cause of wide spectrum lymphoproliferative disoders. The given problem is especially serious after hematopoietic stem cells transplantation (HSCT); frequency of death linked to this complication can achieve 50–90 %. Risk factors for development of posttransplant lymphoproliferative syndromes (PTLS) are: using partially compatible graft, T-cell depletion, presence of severe acute graft-versus-host reaction (GVHD), using of antitymocytic globulin, etc. Severity of associated with EBV posttransplant complications and problems relate to absence of clear diagnostic algorithm, PTLS prophylactic and therapy schedule are argument for necessity of the further studies.
BASIC RESEARCH
Mesenchymal stem cells (MSC) have been widely used in different areas of medicine because of their immunosupressive properties and influence on regeneration of the damaged tissues. The improvement of MSC expansion standard protocols allowing receiving necessary MSC amount with the ability of self-renewal and multilineage differentiation potential for clinical application is required. The present article summarizes the current experience concerning uses of various culture mediums and supplements for the ex vivo MSC expansion. The author's experience of ex vivo MSC expansion using both standard medium DMEM with the supplementation of fetal bovine serum (FBS) and serum-free medium MesenCult MSC Basal Medium (Human) (StemCell technologies Inc.) with addition Mesenchymal Stem Cell Stimulatory Supplements (Human) is presented. The influence of medium type on MSC growth and immunophenotype is analyzed.
ISSN 2413-4023 (Online)