Asadov Ch.,Institute of Hematology and Transfusiology |
Alimirzoyeva Z.,Institute of Hematology and Transfusiology |
Hasanova M.,Institute of Hematology and Transfusiology |
Mammadova T.,Institute of Hematology and Transfusiology |
Shirinova A.,Institute of Hematology and Transfusiology
Georgian medical news | Year: 2016
Research objective is to study the efficacy of recombinant erythropoietin (epoetin alfa) as alternative method of treatment beta-thalassemia intermedia. Study involved 58 patients with beta-thalassemia intermedia (23 women and 35 men). In all observed patients was defined levels of hemoglobin (Hb), red blood cells (RBC), erythrocyte indexes (MCV, MCH, MCHC), hemoglobin fractions (HbA, HbA2, HbF), serum ferritin, serum erythropoietin before and after administrated rEPO. All patients received rEPO during 6 month at the dose - 10000 IU subcutaneously. The majority of patients - 39 (67%) had a good response to rEPO (increase in hemoglobin level more than 20 g/l); 16 patients (28%) had a mean response (increase in Hb 10 - 20 g/l); in 3 (5%) patients occurred poor response to rEPO therapy (increase in Hb <10 g/l). After rEPO treatment of beta-thalassemia intermedia patients there was a statistically significant change in the number of RBC, levels of HbF and sEPO. The evaluation of interdependence between the indices of the baseline sEPO and increased Hb values in patients after rEPO treatment revealed the presence of the reverse direct relationship (r=-0.67). Based on the results, it can be concluded that the use of rEPO in complex therapy of beta-thalassemia intermedia leads to increased levels of Hb and consequently reducing the need for blood transfusions, and accordingly expected to prevent severe complications of blood transfusion (alloimmunization, hypersplenism, iron overload, contamination transmissible infections) facilitating normal growth and development, and a better quality of life.
Zakrzewski D.,Institute of Cardiology |
Seferynska I.,Institute of Hematology and Transfusiology |
Warzocha K.,Institute of Hematology and Transfusiology |
Hryniewiecki T.,Institute of Cardiology
International Journal of Hematology | Year: 2012
We present the case of a 72-year-old male with chronic phase myeloid leukemia. Elevation of the pulmonary artery pressure due to nilotinib therapy was noted. This effect on pulmonary artery pressure was nilotinib dose dependent. © The Japanese Society of Hematology 2012.
PubMed | Institute of Hematology and Transfusiology, Medical University of Warsaw, Medical University of Lódz, Holycross Cancer Center and 3 more.
Type: Journal Article | Journal: Advances in clinical and experimental medicine : official organ Wroclaw Medical University | Year: 2016
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders characterized by ineffective hematopoiesis, cytopenias and a risk of progression to acute myeloid leukemia (AML). Anemia is the most frequent cytopenia diagnosed in patients with MDS. Regular RBC transfusions are the only treatment option for about 40% of patients. Transfusion-dependent patients develop secondary iron overload. The influence of serum ferritin (SF) concentration on survival and acute myeloid leukemia transformation in MDS patients remains controversial. The data for the Central European population is scarce and so far there is no description for Poland.The aim of this study was to perform a retrospective analysis of the relationship of SF concentration with red blood cell transfusion dependency, survival and transformation to acute myeloid leukemia.We retrospectively evaluated the data of the 819 MDS patients (58% male; median age 70 years) included in the MDS Registry of the MDS Section of the Polish Adult Leukemia Group (PALG).Analyses were performed on 190 patients diagnosed with MDS, maximal 6 months before inclusion to the registry in order to avoid selection bias (a shorter survival of higher risk MDS patients). Patients with hyperferritinemia higher than 1000 ng/L vs. patients with SF concentration lower than 1000 ng/L had a median survival of 320 days vs. 568 days, respectively (p log-rank = 0.014). The following factors were found to significantly worsen survival: RBC-transfusion dependence (p = 0.0033; HR 2.67L), platelet transfusion dependence (p = 0.0071; HR 3.321), hemoglobin concentration lower than 10 g/dL (p = 0.0036; HR 2.97), SF concentration higher than 1000 ng/L (p = 0.0023; HR = 2.94), platelet count lower than 10 G/L (p = 0.0081 HR = 5.04), acute leukemia transformation (p = 0.0081; HR 1.968).Taking into account the relatively low number of patients in previous studies exploring hyperferritinemia in MDS, the results of the first Polish MDS Registry provide important insights. Hyperferritinemia higher than 1000 ng/L can be an important indicator of poor prognosis in MDS.
Malinowska I.,Medical University of Warsaw |
Machaczka M.,Karolinska Institutet |
Machaczka M.,University of Rzeszow |
Popko K.,Medical University of Warsaw |
And 3 more authors.
Archivum Immunologiae et Therapiae Experimentalis | Year: 2014
Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a heterogenic syndrome, which leads to an acute, life-threatening inflammatory reaction. HLH occurs both in children and adults, and can be triggered by various inherited as well as acquired factors. Depending on the etiology, HLH can be divided into genetic (i.e., primary) and acquired (i.e., secondary) forms. Among genetic HLH forms, one can distinguish between familial HLH and other genetically conditioned forms of HLH. Acquired HLH can be typically triggered by infections, autoimmune diseases, and malignancies. The most common symptoms of HLH are unremitting fever, splenomegaly, and peripheral blood cytopenia. Some severely ill patients present with central nervous system involvement. Laboratory tests reveal hyperferritinemia (often >10,000 μg/L), increased serum concentration of soluble receptor α for interleukin-2 (>2,400 U/L), hypertriglyceridemia, hypofibrinogenemia, coagulopathy, hyponatremia, hypoproteinemia, and elevated liver transaminases and bilirubin. Prognosis in HLH is very serious. Genetic HLH is always lethal if adequate therapy is not administered. Similarly, severe acquired cases often lead to death without appropriate treatment. Since HLH can be encountered by various specialists in the medical field, basic knowledge of this entity such as diagnostic criteria and treatment should be familiar to all physicians. © 2014, The Author(s).
Kisiel E.,Institute of Hematology and Transfusiology |
Cortez K.C.,Medical University of Warsaw |
Pawelczyk A.,Medical University of Warsaw |
Osko I.B.,Medical University of Warsaw |
And 3 more authors.
Infection, Genetics and Evolution | Year: 2013
Background: HGV/GBV-C is highly prevalent in the general population but its significance remains unclear. It is known that HGV/GBV-C is not primary hepatotropic and its replication was reported in PBMC, bone marrow and other tissues. To investigate a possible role of HGV/GBV-C 115 consecutive patients with hematological malignancies were analyzed for virus RNA presence and quasispecies composition in three compartments: serum, PBMC and bone marrow. Methods: RT-PCR was used to amplify 5'UTR HGV/GBV-C in serum, PBMC and bone marrow. Viral sequences obtained from three compartments were subjected for comparative molecular analysis performed by single strand conformational polymorphism (SSCP) and pyrosequencing. Results: HGV/GBV-C RNA was detected in 23 out of 115 (20.0%) patients, most often in bone marrow (18 patients), followed by PBMC (11 patients) and serum (10 patients). Differences in SSCP bands distribution corresponding to different viral variants and confirmed by direct sequencing were observed in three patients. Conclusion: HGV/GBV-C infection is frequent in patients with hematological malignancies. Common detection of HGV/GBV-C in bone marrow supports the hypothesis that it is a major replication site of this virus. © 2013 Elsevier B.V.
Kuchar E.,Wroclaw Medical University |
Nitsch-Osuch A.,Medical University of Warsaw |
Stolarczyk C.,Institute of Hematology and Transfusiology |
Kurpas D.,Medical University of Warsaw |
And 3 more authors.
Advances in Experimental Medicine and Biology | Year: 2013
Splenectomy significantly increases the risk of severe invasive infections caused by capsular bacteria, such as sepsis and meningitis. Immunizations before and after splenectomy reduce the risk and are routinely recommended. Little is known about compliance with actual immunization guidelines in Poland. The aim of this study was to analyze the vaccination rate and the knowledge of splenectomized patients concerning immunizations in Poland. We applied a questionnaire to survey 85 adult patients (F/M 49/36) splenectomized in 2009-2010 and analyzed the patients' medical files and immunization certificates. Patients were also questioned over the phone. We found that the patients were most commonly immunized against Streptococcus pneumoniae (17/85, 20 %), less often against Haemophilus influenzae b (8/85, 9.4 %), and rarely against Niesseria meningitidis C (3/85, 3.5 %). In contrast, hepatitis B immunization coverage rate was as high as 67 % (57/85). The majority of respondents (59/85, 69.4 %) regarded information about the recommended immunizations as insufficient and rated their doctor's reasoning as inconsistent, a smaller number (20/85, 23.5 %) confirmed they received sound information before splenectomy. Both surgeons and primary care physicians did not offer immunizations to the majority of patients (59/85, 69.4 %); as a result, only 30.6 % of patients (26/85) were immunized against any capsular bacteria before splenectomy. In conclusion, the majority of splenectomized patients are not immunized despite current guidelines and do show an inadequate level of knowledge concerning the consequences of splenectomy. It is important that both surgeons and primary care doctors give patients clear instructions about immunizations and antibiotics recommended before and after their splenectomy. © 2013 Springer Science+Business Media Dordrecht.
PubMed | Institute of Hematology and Transfusiology, StanisAaw Staszic Specialist Hospital, University of Chicago, Hospital in Gorzow Wlkp and 5 more.
Type: Journal Article | Journal: Oncotarget | Year: 2016
Identifying biomarkers of the resistance in multiple myeloma (MM) is a key research challenge. We aimed to identify proteins that differentiate plasma cells in patients with refractory/relapsed MM (RRMM) who achieved at least very good partial response (VGPR) and in those with reduced response to PAD chemotherapy (bortezomib, doxorubicin and dexamethasone). Comparative proteomic analysis was conducted on pretreatment plasma cells from 77 proteasome inhibitor nave patients treated subsequently with PAD due to RRMM. To increase data confidence we used two independent proteomic platforms: isobaric Tags for Relative and Absolute Quantitation (iTRAQ) and label free (LF). Proteins were considered as differentially expressed when their accumulation between groups differed by at least 50% in iTRAQ and LF. The proteomic signature revealed 118 proteins (35 up-regulated and 83 down-regulated in VGPR group). Proteins were classified into four classes: (1) involved in proteasome function; (2) involved in the response to oxidative stress; (3) related to defense response; and (4) regulating the apoptotic process. We confirmed the differential expression of proteasome activator complex subunit 1 (PSME1) by enzyme-linked immunosorbent assay. Increased expression of proteasomes and proteins involved in protection from oxidative stress (eg., TXN, TXNDC5) plays a major role in bortezomib resistance.
PubMed | State Optical Institute, Influenza Research Institute and Institute of Hematology and Transfusiology
Type: Journal Article | Journal: Photodiagnosis and photodynamic therapy | Year: 2014
The problem of transfusion-transmitted infections still remains serious and actual for health care despite the detailed testing of donors. Human immunodeficiency virus, hepatitis B and C viruses and human cytomegalovirus are among the most dangerous pathogens that can be transmitted with blood. Previously, a composition consisting of fullerene layer applied on silica gel particles was shown to inactivate influenza virus up to complete loss of infectivity.In the present study the unit has been developed with source of irradiation whose spectrum is appropriate for solid-phase fullerene. The ability of the unit to inactivate the enveloped influenza virus in protein fraction of donor blood has been studied.It was shown that at optimized conditions complete inactivation of enveloped virus of extremely high initial titer (7.0-9.5 log 10 EID 50/0.2 mL) in the solution of albumin was achieved after as short time as 30 min of irradiation. This process did not affect the oxidative metabolism of neutrophils and membranes of erythrocytes evaluated by NBT reduction test and morphological analysis of erythrocytes, respectively.The data obtained suggests that the method described can be recommended for further development and optimization of the procedure of inactivation of viruses in the preparations of the plasma of donor blood.