Vol 88, No 7 (2016)

Editorial
Hepatosplenic T-cell lymphoma: The problems of diagnosis and treatment
Chernova N.G., Julhakyan H.L., Vinogradova Y.E., Sidorova Y.V., Ryzhikova N.V., Korzhova S.M., Sinitsyna M.N., Tikhomirov D.S., Naumova E.V., Obukhova T.N., Dvirnyk V.N., Sudarikov A.B., Kovrigina A.M., Kravchenko S.K., Melikyan A.L., Kuzmina L.A., Galtseva I.V., Smirnova S.Y., Gemdzhian E.G., Zvonkov E.E., Parovichnikova E.N., Savchenko V.G.
Abstract
In the past decade, a notable advance has been made in the understanding of the pathogenesis of NK/T-cell lymphomas; however, their diagnosis remains difficult because of their rarity and clinical and morphological variabilities. The paper generalizes the ten-year experience of the Hematology Research Center, Ministry of Health of Russia, in diagnosing and treating hepatosplenic T-cell lymphoma (HSTL), considers the problems of differential diagnosis with other hematological diseases occurring with similar clinical and laboratory symptoms, and lays down current approaches to the diagnosis and treatment of this condition. A clinician’s view of the problem of diagnosis and treatment of this disease is given. HSTL is shown to be a heterogeneous group of diseases differing in a T-cell receptor chain gene rearrangement, the clinical course of the disease, and overall survival (OS). According to our data, 3-year OS was 12%; the median survival was 26 months. Two-year OS for γδ and αβ HSTL was equal to 25 and 70%, respectively. The difference in OS for the variants of HSTL failed to reach statistical significance (because the sample might be insufficient).
Terapevticheskii arkhiv. 2016;88(7):4-14
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Acute Ph-negative lymphoblastic leukemias in adults: Risk factors in the use of the ALL-2009 protocol
Parovichnikova E.N., Sokolov A.N., Troitskaya V.V., Klyasova G.A., Rusinov M.A., Akhmerzaeva Z.K., Kuzmina L.A., Bondarenko S.N., Baranova O.Y., Kaporskaya T.S., Zotina E.N., Zinina E.E., Samoilova O.S., Gavrilova L.V., Kaplanov K.D., Konstantinova T.S., Lapin V.A., Kravchenko S.K., Gribanova E.O., Zvonkov E.E., Gavrilina O.A., Baskhaeva G.A., Galstyan G.M., Obukhova T.N., Galtseva I.V., Kulikov S.M., Savchenko V.G.
Abstract
Aim. To analyze well-known risk factors (RFs), such as age, immunophenotype, baseline leukocytosis, enhanced lactate dehydrogenase (LDH) activity, time to achieve complete remission, a risk group, and cytogenetic abnormalities) in patients with acute lymphoblastic leukemia (ALL) in the use of the ALL-2009 protocol. Subjects and methods. The protocol covered 298 patients (137 women (including 13 pregnant women) and 161 men) aged 15 to 55 years (median age 28 years) with Ph-negative ALL. The phenotype was unknown in 6 patients. Three (1%) were ascertained to have a biphenotypic variant. 182 (62.4%) patients were found to have B-cell ALL (early pre-B ALL (n=51); common ALL (n=92), and pre-B ALL (n=39); 107 (36.6%) patients had T-cell ALL (early T-ALL (n=56); thymic T-ALL (n=41), and mature T-ALL (n=10). According to the baseline clinical and laboratory parameters (leukocytosis of 30·109/l and more for B-ALL; and that of 100·109/l and more for T-ALL; phenotype В-I for B-ALL, phenotype Т-I-II-IV for T-ALL; LDH activity was more than twice the normal values; the presence of translocation t(4;11)), the high-risk group included most patients with B-ALL (n=110 (72.8%)) and T-ALL (n=76 (76%)). Thirty-five patients with T-ALL underwent autologous bone marrow transplantation (BMT). Allogeneic BMT was performed in 18 (7%) of the 258 patients who had undergone an induction phase. Results. Five-year overall survival for all the patients included in the investigation was 59%; relapse-free survival was 65%, which was significantly different in the patients with B-ALL and in those with T-ALL: the overall survival rates were 53.3 and 67.5% (p=0.1); the relapse-free survival was 56 and 79% (p=0.005), respectively. Multivariate analysis including the well-known RFs demonstrated that the latter for T-ALL were of no independent prognostic value and only the patient’s age was identified for B-ALL (p=0.013). Conclusion. A lower chemotherapeutic load and a small number of allogeneic BMTs did not affect total positive treatment results in adult patients with ALL, by complying with the principle achieving the continuity of cytostatic effects and by preserving the total cytostatic loading dose. The results of the Russian investigation casts some doubt on the necessity of using very intensive consolidation cycles and performing a large number of allogeneic BMTs in adult patients with ALL.
Terapevticheskii arkhiv. 2016;88(7):15-24
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Diagnosis of latent polycythemia vera: A clinician’s opinion
Melikian A.L., Subortseva I.N., Kovrigina A.M., Kolosheynova T.I., Abdullaev A.O., Kuznetsova P.I., Sudarikov A.B., Kulikov S.M.
Abstract
Aim. To identify the clinical features of latent polycythemia vera (PV) as an independent nosological entity. Subjects and methods. The investigation enrolled 81 patients (50 with extensive (manifest) PV and 31 with latent PV) who had visited the Outpatient Department, Hematology Research Center, Ministry of Health of Russia, in 2014 to October 2015. Results. The gender distribution of the patients was statistically comparable in the analyzed groups. The patients with manifest PV were slightly older than those with latent PV: the median age in the compared groups was 56 and 44 years, respectively. Red blood cell counts, hemoglobin concentrations, and packed cell volume were higher in the patients with manifest PV. Blood platelet counts were higher in the latent PV group. There were no differences in the number of white blood cells in the compared groups. All the patients were JAK2 V617F mutation carriers. The JAK2 allele load was significantly higher in the manifest PV group than in the latent PV group. The compared patient groups differed in the rate of thromboses in the history or at diagnosis. In the patients with latent PV, thromboses were detected in 38% of cases versus 16% in those with manifest PV. In latent PV, there were mainly venous thromboses; abdominal vascular thromboses were diagnosed with a high frequency. Arterial thromboses were revealed in only 2 cases. Conclusion. Chronic myeloproliferative disease that is characterized by the JAK2 V617F mutation, borderline hemoglobin counts, and morphological features of a bone marrow trephine biopsy specimen, which are specific for PV, is an independent PV variant, namely: latent PV.
Terapevticheskii arkhiv. 2016;88(7):25-30
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Aberrant methylation of the promoter regions of the SOX7 and p15INK4b genes and Wnt signaling pathway antagonists in patients with acute myeloid leukemias
Kostroma I.I., Gritsaev S.V., Sidorova Z.Y., Tiranova S.A., Svitina S.P., Drizhun Y.S., Chubukina Z.V., Martynkevich I.S., Kapustin S.I., Bessmeltsev S.S.
Abstract
Aim. To investigate the methylation status of the SOX7 and p15NK4b genes and Wnt signaling pathway antagonists in patients with acute myeloid leukemia (AML) in order to assess the association of the rate of aberrant methylation (AM) with the morphological variant and pattern of chromosomal aberrations, as well as the impact of the methylation status on survival. Subjects and methods. The data of 57 AML patients aged 20 to 79 years were analyzed. The methylation status of the genes was studied by methylation-specific polymerase chain reaction. Results. The signs of the AM of ≥1 gene were detected in 52 (91.2%) of the 57 patients. The most common finding was AM of simultaneously 2 or 3 genes: in 29.8 and 21.1% of the patients, respectively. Concurrent methylation of 3-5 genes proved to be a more frequent finding in AML patients with myelodysplasia: in 7 (70%) of 10 patients. The proportion of patients with methylation of 5 genes was considerably higher in a group of patients with a complex karyotype: 50% versus 8.3% among other patients (odds ratio: 11.0; 95% confidence interval 2.0 to 61.6; p=0.01). There were no differences in the median overall and relapse-free survival rates in patients with a normal karyotype and without FLT3 and NPM mutations, who received induction therapy, in relation to the number of genes with AM. Conclusion. AM of the p15NK4b and SOX7 genes and Wnt signaling pathway antagonists is detected in the majority of patients with AML, which allows hypomethylating agents to be recommended for the treatment of patients who cannot use intensive cytostatic therapy for different reasons. The detection of a large number of genes with the aberrant methylation status in most AML patients with myelodysplasia or a complex karyotype serves as the basis for initiating trials to evaluate the efficiency of a combination of 5-azacytidine and cytostatics.
Terapevticheskii arkhiv. 2016;88(7):31-36
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Therapy for primary mediastinal large B-cell lymphoma in accordance with the R-DA-EPOCH-21 program: The first results
Mangasarova Y.K., Magomedova A.U., Nesterova E.S., Volodicheva E.M., Vorobyev V.I., Kravchenko S.K.
Abstract
Aim. To evaluate the efficiency of the R-DA-EPOCH-21 + R-DHAP protocol and autologous hematopoietic stem cell transplantation (a BEAM conditioning mode) in first-line therapy for primary mediastinal large B-cell lymphoma (PMBCL). Subjects and methods. In 2013 to 2016, the investigation enrolled 57 patients with newly diagnosed PMBCL (according to the 2008 WHO criteria). The results were analyzed in 40 patients who had completed their treatment. Results. All the 40 patients (14 men and 26 women) (median age, 27 years (19 to 67 years)) received 6 cycles of polychemotherapy (PCT) in accordance with the R-DA-EPOCH-21 regimen. After induction PCT cycles, 32/40 (80%) patients achieved complete remission. Partial remission was stated in 8/40 (20%) patients who had further 2 cycles of chemotherapy using the R-DHAP program and autologous hematopoietic stem cell transplantation (a BEAM conditioning mode). Two-year overall and relapse-free survival rates were 100% and 96%, respectively; the median follow-up was 17 months. Conclusion. The R-DA-EPOCH regimen allows complete remission in 80% of the cases and two-year survival in 100%. If there are unfavorable factors at onset and in partial remission, it is appropriate to intensify treatment at early stages, by using high-dose chemotherapy and autologous hematopoietic stem transplantation.
Terapevticheskii arkhiv. 2016;88(7):37-42
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Late myelotoxicity of high-dose chemotherapy according to the modified NHL-BFM-90 program in adult patients with diffuse large B-cell lymphoma
Dorokhina E.I., Magomedova A.U., Dvirnyk V.N., Galtseva I.V., Glinkina S.A., Kulikov S.M., Obukhova T.N., Kravchenko S.K.
Abstract
Aim. To evaluate late myelotoxicity (MT) relate to high-dose chemotherapy (CT) according to the modified NHL-BFM-90 (mNHL-BFM-90) program in adult patients with diffuse large B-cell lymphoma (DLBCL). Subjects and methods. The results of a complex clinical, laboratory, and instrumental examination, including cytologic, histologic, and routine cytogenetic studies of the bone marrow (BM), were analyzed in 40 DLBCL patients treated according to the mNHL-BFM-90 program in the National Research Center for Hematology (NRCH), Ministry of Health of the Russian Federation (MHRF), in 2002 to 2009; among them, there were 20 men and 20 women (median age, 57 years). A comparison group consisted of 19 patients who had received high-dose СНОР/R-СНОР-21 CT in HRC, MHRF, in the same period of time; out of them, there were 8 men and 11 women (median age, 70 years). The median posttherapy follow-up period was 6 years. The results of BM studies were analyzed before and 5-10 years after treatment in complete remission. The cytological and histological studies of BM determined its cellularity, the sizes of erythroid, granulocytic, and megakaryocytic lineages, their ratios, the signs of dysplasia, and stromal dysplastic changes. Routine BM cytogenetic study was conducted to identify karyological problems. Only myelopoietic changes that had been revealed for the first time 5-10 years after completion of CT were kept in mind as late MT. Cases of baseline and post-CT changes and those of baseline and no post-CT changes were not taken into account. Results. Cytopenic syndromes (having no signs of myelopoietic lineage dysplasia or needing no blood component replacement transfusions) were revealed in 52% of the patients in the high-dose CT; thrombocytopenia amounted to 46%. In the late follow-up period, the patient group after high-dose mNHL-BFM-90 CT were found to have BM hypocellularity in 15 (38%) cases, a narrowing of erythroid and megakaryocytic lineages in 13 (33%) and 19 (48%) cases, respectively, and obvious secondary stromal changes in 17 (43%). The first 6 patients underwent routine BM cytogenetic study; all the patients were ascertained to have a normal karyotype; in this connection further BM study was stopped. Conclusion. The late MT of high-dose mNHL-BFM 90 CT is statistically significantly higher than that of the standard CHOP/R-CHOP-21 therapy. However, signs of myelodysplastic syndromes and those of cytopenia requiring blood component transfusions were observed in none patient.
Terapevticheskii arkhiv. 2016;88(7):43-48
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Subcutaneous panniculitis-like T-cell lymphoma: The authors’ experience of diagnosis and treatment
Gorodetskiy V.R., Probatova N.A., Vasilyev V.I., Mukhortova O.V., Aslanidi I.P., Sidorova Y.V., Ryzhikova N.V., Radenska-lopovok S.G., Egorova O.N.
Abstract
The paper gives the data of clinical, histological, immunohistochemical, and molecular studies and the results of positron emission tomography in 3 cases of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). It shows the high efficiency of a GEM-P regimen in the treatment of patients with SPTCL.
Terapevticheskii arkhiv. 2016;88(7):49-55
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Diffuse large B-cell lymphoma with monoclonal immunoglobulin secretion
Gavrilina O.A., Parovichnikova E.N., Zvonkov E.E., Troitskaya V.V., Kravchenko S.K., Savchenko V.G.
Abstract
Aim. To provide the clinical characteristics of patients with diffuse large B-cell lymphoma (DLBCL) with monoclonal immunoglobulin secretion and to evaluate the efficiency of intensified mNHL-BFM-90 or R-DA-EPOCH/R-HMA therapy programs in patients with Ig-secreting DLBCL. Subjects and methods. A clinical trial was conducted in 93 patients with newly diagnosed DLBCL, among whom 21 (22.6%) were found to have monoclonal immunoglobulin secretion. Results. Ig-secreting DLBCL is shown to be characterized by bone marrow involvement (p<0.001), as well as generalized injury (Ann Arbor Stage 4) and a high risk in accordance with the international prognostic index (p=0.001 and p=0.026, respectively). Analysis of overall and event-free survival rates has indicated that the patients have a poor prognosis versus those with non-Ig-secreting DLBCL and poor prognostic factors even when implementing intensified therapy programs, such as mNHL-BFM-90 or R-DA-EPOCH/R-HMA ones. Conclusion. The investigation has demonstrated that there is a high association of the secretion of monoclonal paraproteins with bone marrow involvement in DLBCL (p<0.001). The intensified therapy using the mNHL-BFM-90 and R-DA-EPOCH/R-HMA programs involving autologous hematopoietic stem cell transplantation also permits the patients with Ig-secreting DLBCL to achieve long-term sustained remissions in not all cases.
Terapevticheskii arkhiv. 2016;88(7):56-61
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Follicular lymphoma. High-dose immunochemotherapy with autologous blood stem cell transplantation: Results of the first prospective study in Russia
Nesterova E.S., Kravchenko S.K., Mangasarova Y.K., Baryakh E.A., Misyurina A.E., Vorobyev V.I., Plastinina L.V., Chernova N.G., Kovrigina A.M., Obukhova T.N., Klyasova G.A., Shevelev A.A., Kostina I.E., Gemdzhian E.G., Gaponova T.V., Vorobyev A.I.
Abstract
Aim: To evaluate the efficiency of high-dose chemotherapy (HDCT) with further autologous blood stem cell transplantation (auto-BSCT) in the first-line therapy of patients with follicular lymphoma (FL) and poor prognostic factors. Subjects and methods. In 2000 to 2015, the National Research Center for Hematology, Ministry of Health of the Russian Federation, performed therapy in 39 patients with FL and poor prognostic factors (a total of 215 patients with FL). The R-CHOP treatment was done as induction therapy. Sequential HCT and further auto-BSCT were performed in 29 (74%) of the 39 patients, who had shown a partial tumor response to the induction therapy or achieved partial remission after 4-6 cycles of CT, but had poor prognostic factors. 22 of the 29 patients underwent auto-BSCT in first-line therapy after induction R-CHOP regimens. Among them, there were 17 men with a median age of 46 years (31-68 years). 21 of the 22 patients were recorded to have Stage IV by the Ann Arbor staging classification. Bulky peritoneal and retroperitoneal tumors larger than 7 cm were detectable at disease onset in 14 of the 22 cases. Two patients were noted to have phenomena of leukemization. 16 patients had bone marrow (BM) involvement. According to the Follicular Lymphoma International Prognostic Index-1 (FLIPI-1), the patients were divided into 3 groups: 1) a low risk (n=5); 2) an intermediate risk (n=3); a high risk (n=14). B-symptoms were observed in 16 cases. 16 patients were diagnosed with cytological grade I-II FL and 6 had grade IIIA. According to the tumor proliferative pattern, the distribution turned out to be as follows: nodular (n=6), nodular-diffuse (n=13), and diffuse (n=3). The proliferative activity index averaged 30% (8-90%). Serum and urine proteins were immmunochemically assayed in 18 cases, out of them 8 patients were diagnosed as having serum β2-microglobulin concentrations above normal as a poor prognostic factor. In 14 of the 22 patients, the activity of lactate dehydrogenase was greater than normal (266-7806 U/l). Results. Out of the 22 patients, 20 who have undergone auto-BSCT in first-line therapy are survivors and have remission of the underlying disease: 18 and 2 patients achieved complete and partial remission, respectively. The follow-up period was 7 to 178 months (median, 32 months). After auto-BSCT in the first remission, 2 patients developed disease recurrences: an early recurrence after 9 months in one case and a late recurrence 6 years after completion of therapy in the other. Conclusion. The first prospective study of intensive therapy for FL in Russia has demonstrated that HDCT with further auto-BSCT in first-line therapy allows complete remission in patients with poor prognostic factors and higher overall and progression-free survival rates.
Terapevticheskii arkhiv. 2016;88(7):62-71
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Perianal infection in patients with hemoblastosis: Risk factors and possibilities of prevention
Shtyrkova S.V., Klyasova G.A., Ntanishyan K.I., Gemdzhian E.G., Troitskaya V.V., Karagyulyan S.R.
Abstract
Aim. To identify poor prognostic factors for perianal infection (PI) in patients with hemoblastosis and to define an effective tactic for preventive and therapeutic measures. Subjects and methods. The prospective study enrolled 72 patients (37 men and 35 women; mean age, 47 years) with hemoblastosis that was complicated by the development of one of the following forms of PI: abscess, infiltrate, multiple ulcers. Different clinical and laboratory characteristics of the patients were examined to identify risk factors for PI. The species-specific concordance of microorganisms isolated from the anus and blood in the development of PI was assessed to record the latter as a source of sepsis. Treatment policy was defined according to the clinical form of PI. Results. Acute myeloid leukemias and lymphomas were the most common background diseases in 30 (41.7%) and 22 (30.6%) patients, respectively. During induction chemotherapy cycles, perianal tissue infection occurred twice more frequently (66%) than totally at the onset of hemoblastosis (13%) and after achievement of remission (during consolidation and maintenance therapy) (21%; Fisher’s exact test; p=0.01). PI in agranulocytosis was more than twice as common as in its absence: 69.4% vs 30.6% (p=0.01) and was responsible for sepsis in 9 (18%) of 50 patients. The main source of perianal tissue infection in patients with granulocytopenia was anal fissures and fistulas and ulcers of the anal canal: 44 (88%) cases of the 50 cases. In PI as an abscess, the average white blood cell count was 5 times higher (p=0.01) than that in PI as an infiltrate (or multiple ulcers): 6.6·109/l and 1.2·109 g/l. Abscess formation was observed in 16 (22.2%) patients and an indication for surgical drain. The inflammatory infiltrate was found to develop in 48 (66.7%) patients; multiple ulcers were seen in 8 (11.1%); in this group, parenteral antimicrobial therapy proved to be effective in 36 (78%) patients. 29 patients were operated on for anal fissures and fistulas at intercycle intervals. After continuing CT, PI recurrences were observed in 4 (9.1%) patients. In the operated versus medically treated patients, the risk of complications associated with abnormalities in the perianal area during continued CT was 5 times statistically significantly lower (odds ratio=0.2; 95% confidence interval 0.1 to 0.5; p=0.04; Cochran-Mantel test). Conclusion. Induction CT cycles, the status of granulocytopenia, and the presence of infection sources in the anal canal as an anal fissure, skin ulcerations, or a fistula should be considered as independent statistically significant prognostic risk factors for PI. The number of granulocytes determines the form of inflammation, the course of infection, and the chance of developing sepsis. The effective prevention encompassing surgical treatment for anal canal diseases reduces the risk of septic complications and the number of paraproctitis recurrences, contributing to the implementation of a planned CT program in patients with hemoblastosis.
Terapevticheskii arkhiv. 2016;88(7):72-77
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Molecular serological characteristics of weak D antigen types of the Rhesus system
Golovkina L.L., Stremoukhova A.G., Pushkina T.D., Kalandarov R.S., Atroshchenko G.V., Vasilyeva M.N., Surin V.L., Salomashkina V.V., Pshenichnikova O.S., Miterev G.Y., Parovichnikova E.N., Savchenko V.G.
Abstract
Aim. To estimate the spread of weak D antigen types of the Rhesus system in the citizens of the Russian Federation and a possibility of serologically identifying these types. Subjects and methods. The red blood cells and DNA of people with weakened expression of D antigen were investigated using erythrocyte agglutination reaction in salt medium (2 methods); agglutination reaction in the gel columns containing IgM + IgG anti-D antibodies, indirect antiglobulin test with IgG anti-D antibodies (2 methods); polymerase chain reaction to establish the type of weak D. Results. A rhesus phenotype was determined in 5100 people in 2014-2015. The weakened agglutinable properties of red blood cells were detected in 102 (2%) examinees. 63 examinees underwent genotyping to identify the variants of the weak D antigen, which identified 6 weak D types. There were the most common weak D types 3 (n=31 (49.2%)) and weak D type 1 (n=18 (28.6%)), including weak D type 1.1 in one (1.6%) case. The other 4 weak D antigen types were as follows: weak D type 2 (14.3% (n=9)), weak D type 15 (4.8% (n=3)), weak D type 4.2 (DAR) (1.6% (n=1)) and weak D type 6 (1.6% (n=1)). The antiglobulin test in the gel column containing antiglobulin serum was the most sensitive serological assay to identify the weak D antigen. Only a molecular test could establish weak D type 15 in 2 samples of red blood cells with Ccdee and ccdEe phenotypes. Conclusion. The weak D antigen could be serologically identified in 96.8% of cases. When testing for weak D, particular attention should be given to people with the D-negative phenotype who had the C or E antigens. Our investigations conducted for the first time in Russia will be able to improve the immunological safety of red blood cell-containing medium transfusions for patients.
Terapevticheskii arkhiv. 2016;88(7):78-83
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The specific features of pain syndrome in patients with hemophilia
Levchenko O.K., Shulutko E.M., Zorenko V.Y., Galstyan G.M.
Abstract
Aim. To study the nature of pain in hemophilic arthropathy and its prevention ways used by patients with hemophilia. Subjects and methods. A prospective, multicenter, cross-sectional study was conducted to interview 136 patients with hemophilia A or B. The survey included 32 points, including questions about drug and nondrug pain treatment, as well as questionnaires to assess the severity of pain and its impact on daily life (Brief Pain Inventory) and those to identify a neuropathic pain component (PainDetect questionnaire). Results. 83 (75%) patients with hemophilia experienced acute pain associated with bleeding into the large joints; 44 (39%) patients had chronic pain that had lasted longer than six months; 33% assessed the moderate pain experienced in the past 24 hours as severe (more than 5-8 of the 10 scores). In addition to the above, only 32% of the respondents indicated that they had painless intervals within the last 24 hours. 75% of the hemophilia patients mentioned to have limited daily activities. 74% reported their partial or complete disability when pain occurred. 77% of the respondents pointed out that when having pain, they had experienced great difficulty walking. 55% of the hemophilia patients had sleep disorders because of pain. When acute pain occurred, only 91 (81%) respondents injected a factor VIII or IX preparation, 37% of the respondents used narcotic analgesics; 51% received different nonsteroidal anti-inflammatory drugs; 13% took paracetamol. Some patients indicated that they used alcohol and illegal narcotic drugs to relieve pain. Attention is drawn to the fact that the hemophilia patients very frequently took painkillers: 60% of the respondents used analgesics every month; of them 49% had them every week, 11% every day. Most patients (n=83 (74%)) stated that they treated pain (prescribed drugs) themselves; 49 (44%) patients held they were dissatisfied with their pain treatment. When the question as to how the analgesic you used could “remove’ pain was asked, only 12 (10%) patients answered that this could fully relieve the pain; 31 (26%) patients told that the pain was not reduced even by half. The study has shown that a neuropathic pain component is very common in hemophilia patients (31%). Conclusion. Pain in patients with hemophilia is a serious problem that negatively affects their quality of life, including their working capacity. The prevention and treatment of pain in hemophilia patients should involve the following: organization of educational activities and pain services; adequate treatment of acute pain; detection and prevention of a neuropathic pain component.
Terapevticheskii arkhiv. 2016;88(7):84-88
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The new endothelin receptor antagonist macitentan: Prospects for therapy of pulmonary arterial hypertension
Avdeev S.N.
Abstract
Pulmonary arterial hypertension (PAH) is a clinical group of severe and rare diseases with similar morphological, hemodynamic, and therapeutic characteristics. One of the novel drugs to treat PAH is macitentan, a new double endothelin ETA and ETB receptor antagonist that is characterized by special physicochemical properties, ensuring the penetration of the drug into tissues and its improved receptor-binding properties. The SERAPHIN trial has demonstrated that therapy with macitentan 10 mg versus placebo statistically significantly reduces the risk of poor outcomes and death by 45%. The treatment with macitentan 10 is observed to be highly effective regardless of the presence/absence of basic PAH-specific therapy. The drug considerably improves clinically important outcomes, including 6-minute walk distance and WHO functional class. Macitentan exerts a steady-state therapeutic effect, by improving pulmonary hemodynamics. Macitentan 10 mg statistically significantly reduces the risk of PAH, frequency of its related hospitalizations, and the number of days spent in hospital. The drug has a favorable safety profile; its most common side effects are headache, nasopharyngitis, and anemia. Macitentan is an effective first-line drug to improve long-term outcomes in patients with newly and previously diagnosed PAH.
Terapevticheskii arkhiv. 2016;88(7):89-97
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A rare case of myeloproliferative disease with t(8;13)(p11;q12) associated with eosinophilia and lymphadenopathy
Tsyba N.N., Turkina A.G., Chelysheva E.Y., Nemchenko I.S., Kovrigina A.M., Obukhova T.N., Urnova E.S., Kuzmina L.A., Savchenko V.G.
Abstract
Myeloproliferative disease associated with FGFR1 rearrangement (8p11), which is included in the 2008 WHO Classification of Myeloid Neoplasms, is a rare and extremely aggressive abnormality. The paper describes a clinical case of a 39-year-old female patient who was detected to have leukocytosis (as high as 47.2·109/l), absolute eosinophilia (as high as 3.1·109/l), and enlarged peripheral lymph nodes during her visit to a doctor. The bone marrow (BM) showed the changes typically encountered in myeloproliferative disease with eosinophilia. The patient was found to have t(8;13)(p11;q12) translocation associated with the rearrangement of the FGFR1 gene located at the 8p11 locus. Molecular and cytogenetic examinations failed to reveal BCR-ABL chimeric transcript, Jak2 V617F mutation, and deletions and translocations involving PDGFRA (4q12) and PDGFRB (5q32-33). The similar changes in the karyotype were also found in the lymph node cells. The undertaken treatment with hydroxyurea and the tyrosine kinase inhibitor dasatinib turned out to be ineffective. The patient underwent allogeneic BM transplantation from a HLA-identical sibling. Graft rejection occurred 6 months later. Allogeneic BM transplantation from the same donor (100% donor chimerism; FGFR1/8р11 translocation was not detected), which was complicated by the development of chronic graft-versus-host reaction, was performed again in March 2015. The patient is being followed up and continues to receive immunosuppressive therapy.
Terapevticheskii arkhiv. 2016;88(7):98-103
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Transformation of secondary myelodysplastic syndrome to atypical chronic myeloid leukemia in a female patient with acute myeloid leukemia
Gritsaev S.V., Kostroma I.I., Zapreev I.M., Shmidt A.V., Tiranova S.A., Balashova V.A., Martynkevich I.S., Chubukina Z.V., Semenova N.Y., Chechetkin A.V.
Abstract
Secondary myeloid neoplasia may be a complication of intensive cytostatic therapy. The most common types of secondary neoplasias are acute myeloid leukemia and myelodysplastic syndrome. The development of secondary atypical chronic myeloid leukemia (aCML) is an extremely rare phenomenon. The paper describes transformation of secondary myelodysplastic syndrome to aCML 6 months after its diagnosis. The development of aCML was accompanied by additional chromosomal aberration as monosomy of chromosome 17. No mutations in the JAK2, MPL, and CalR genes were detected. It is concluded that the clinical course of secondary myeloid neoplasias is variable.
Terapevticheskii arkhiv. 2016;88(7):104-108
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Laparoscopic splenectomy in immune thrombocytopenic purpura in pregnant women
Ntanishyan K.I., Soboleva O.A., Galstyan G.M., Zvereva A.V., Sorkina O.M.
Abstract
The paper describes 4 cases of laparoscopic splenectomy in pregnant women with immune thrombocytopenic purpura. No complications of surgery were noted in all the patients. The postoperative period was marked by sustained clinical and hematological remission that made it possible to discontinue prednisolone therapy and to ensure an uncomplicated course of pregnancy and labor.
Terapevticheskii arkhiv. 2016;88(7):109-113
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Pregravid preparation of diabetic women
Grigoryan O.R., Volevodz N.N., Andreeva E.N.
Abstract
Pregnancy in women with type 1 or 2 diabetes mellitus (DM) is associated with an increased risk for complications in both the mother and her fetus. The impact of these complications on modifiable risk factors may substantially improve pregnancy outcomes and reduce malformation rates in children. This is a goal of pregravid preparation (PGP) in this category of patients. The review gives the main points of PGP in patients with types 1 and DM and shows the results of main studies providing evidence for PGP in DM. In particular, by the moment of conception, DM patients should achieve a glycosylated hemoglobin (HbA1c) goal of <6% no later than 4 weeks before conception and during the first trimester of pregnancy, take folic acid in a high dose (at least 4000 µg, or 4 mg, daily), quit tobacco smoking and alcohol use, receive potentially teratogenic drugs, and, if need be, lose weight (the target body mass index of <27 kg/m2).
Terapevticheskii arkhiv. 2016;88(7):114-119
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