ISRN Hematology The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Preventative Effects of Caffeic Acid Phenyl Ester on Cadmium Intoxication Induced Hematological and Blood Coagulation Disturbances and Hepatorenal Damage in Rats Sun, 30 Mar 2014 13:56:52 +0000 The preventative effect of caffeic acid phenyl ester (CAPE) against hematological, blood coagulation, and hepatorenal disturbances in cadmium (Cd) intoxication was investigated in rats. Male Wistar rats were randomly assigned into control group, Cd-group, and Cd + CAPE group. Cd intoxication was induced by intraperitoneal injection (i.p.) of CdCl2 (1 mg/kg/day) for 21 days, and CAPE was daily given (10 micromol/kg; i.p.) for also 21 days. The results showed that Cd intoxication impaired hepatorenal function and significantly prolonged prothrombin time and activated partial thromboplastin time and decreased fibrinogen level, red blood cells and platelets counts, hemoglobin concentration, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration. Interestingly, all these hematological, blood coagulation, and hepatorenal deteriorations of Cd toxicity were significantly prevented by CAPE. Additionally, CAPE significantly reversed the significant decreases in levels of total reduced glutathione and superoxide dismutase and increases in levels of thiobarbituric acid reactive substances that were observed in the sera and liver and kidney homogenates of Cd group. It is concluded that CAPE is a promising compound that can counteract the hematological and blood coagulation disturbances, oxidative stress, and hepatorenal damages in Cd intoxication. However, further studies are crucially needed to improve this treatment in patients. Tariq Helal Ashour Copyright © 2014 Tariq Helal Ashour. All rights reserved. Relevant Aspects of Centrifugation Step in the Preparation of Platelet-Rich Plasma Tue, 25 Mar 2014 08:38:36 +0000 Introduction. Platelet-Rich Plasma (PRP) is rich in growth factors, playing important role in tissue healing. The wide variation of reported protocols for preparation of PRP leads to variable compositions, which induce different biological responses and prevent results comparison. This study aims to highlight relevant aspects of the centrifugation step to obtain reproducible results and overall quality. Material and Methods. Samples of blood were collected from 20 healthy donors that have signed free informed consent. Two centrifugation steps (spins) were analyzed for the influence of centrifugal acceleration, time, processed volume, and platelet gradient. The Pure Platelet-Rich Plasma (P-PRP) was characterized as platelet concentration, integrity, and viability (sP-selectin measurement). Results. Lower centrifugal accelerations favour platelet separation. The processing of 3.5 mL of blood at 100 ×g for 10 min (1st spin), 400 ×g for 10 min (2nd spin), withdrawing 2/3 of remnant plasma, promoted high platelet recovery (70–80%) and concentration (5x) maintaining platelet integrity and viability. The recovery of platelets was reduced for a larger WB volume (8.5 mL) processed. Conclusion. Centrifugal acceleration, time, WB processed volume, and minimization of the platelet gradient before sampling are relevant aspects to ensure reproducible compositions within the autologous nature of PRP. Amanda G. M. Perez, José Fábio S. D. Lana, Ana Amélia Rodrigues, Angela Cristina M. Luzo, William D. Belangero, and Maria Helena A. Santana Copyright © 2014 Amanda G. M. Perez et al. All rights reserved. Phagocytized Neutrophil Fragments in the Bone Marrow: A Phenomenon Most Commonly Associated with Hodgkin Lymphoma Tue, 18 Mar 2014 07:20:22 +0000 Bone marrow macrophages containing other cells, or large pieces of other cells, represent a distinctive feature of diseases such as Hemophagocytic Lymphohistiocytosis (HLH) and Rosai-Dorfman disease. We describe a distinct variation of phagocytic histiocyte morphology, featuring histiocytes containing predominantly fragments of neutrophil nuclei. We retrospectively reviewed initial bone marrow samples for Hodgkin lymphoma, Burkitt lymphoma, Ewing sarcoma, or evaluation for nonneoplastic conditions, scoring the presence or absence of the above-described histiocytes. We find that these histiocytes, which we term “fragmentophages,” are associated with staging marrow sampling for malignancy, especially Hodgkin lymphoma (Hodgkin lymphoma: 28/34 or 82.4%, Ewing sarcoma: 11/26 or 42.3%, Burkitt lymphoma: 4/13 or 30.8%). These cells are significantly less common in marrow samples for nonneoplastic conditions (4/21 or 19.0%). Fragmentophages are significantly associated with malignancy, especially Hodgkin lymphoma, and their recognition has the potential to provide a clue to an underlying malignancy. Michael A. Arnold and Samir B. Kahwash Copyright © 2014 Michael A. Arnold and Samir B. Kahwash. All rights reserved. Coexisting Iron Deficiency Anemia and Beta Thalassemia Trait: Effect of Iron Therapy on Red Cell Parameters and Hemoglobin Subtypes Wed, 12 Mar 2014 09:46:54 +0000 Background. Coexistence of iron deficiency anemia (IDA) and beta thalassemia trait (BTT) has been the topic of few studies. However, no study from our country was found evaluating the effect of iron therapy in patients with concomitant IDA and BTT. Methods. Over a period of two years, 30 patients with concomitant IDA and BTT were included. All the patients had a complete blood count, serum iron studies, and thalassemia screening using BIORADTM hemoglobin testing system. The patients received oral iron therapy in appropriate dosages for a period of twenty weeks, after which all the investigations were repeated. Appropriate statistical methods were applied for comparison of pre- and posttherapy data. Results. All except two patients were adults with a marked female preponderance. Oral iron therapy led to statistically significant improvement in hemoglobin, red cell indices (), and marked change in serum iron, ferritin, and HbA2 levels (). There was a significant reduction in the total iron binding capacity levels. Conclusion. The present study shows the frequent occurrence of iron deficiency anemia in patients with beta thalassemia trait, which can potentially confound the diagnosis of the latter. Hence, iron deficiency should be identified and rectified in patients with suspicion of beta thalassemia trait. Sarika Verma, Ruchika Gupta, Madhur Kudesia, Alka Mathur, Gopal Krishan, and Sompal Singh Copyright © 2014 Sarika Verma et al. All rights reserved. Prevalence of Deletional Alpha Thalassemia and Sickle Gene in a Tribal Dominated Malaria Endemic Area of Eastern India Tue, 11 Mar 2014 08:46:26 +0000 Inherited hemoglobin disorders like alpha thalassemia and sickle gene are common in the Indian subcontinent. These disorders in the heterozygous state act as malaria resistance genes and influence the susceptibility to Plasmodium falciparum malaria. There is inadequate knowledge about the epidemiology of these malaria resistance genes in the tribal dominated malaria endemic region of the state of Odisha in eastern India. A cross sectional prevalence study was undertaken in 594 subjects in five tribal populations in this region, namely, Sahara (42.4%), Kutia Kandha (30.0%), Kuda (15.8%), Gond (9.8%), and Oraon (2.0%). Sickling test, Hb electrophoresis, HPLC, and molecular studies were undertaken to diagnose the prevalence of sickle allele, β-thalassemia allele, and deletional alpha thalassemia. Sickle and β thalassemia alleles were found in 13.1% and 3.4% of subjects, respectively. Sickle allele was found both in heterozygous (10.1%) and homozygous state (3.03%). The prevalence of alpha thalassemia was 50.84% with an allelic frequency of 0.37. Both α−3.7 and α−4.2 alpha thalassemia were detected with an allele frequency of 0.33 and 0.04, respectively. The high prevalence of alpha thalassemia and sickle gene in this population is probably due to selection pressure of endemic malaria in this part of India. Prasanta Purohit, Snehadhini Dehury, Siris Patel, and Dilip Kumar Patel Copyright © 2014 Prasanta Purohit et al. All rights reserved. Pretransplant Comorbidities Maintain Their Impact on Allogeneic Stem Cell Transplantation Outcome 5 Years Posttransplant: A Retrospective Study in a Single German Institution Wed, 05 Mar 2014 17:26:11 +0000 The introduction of reduced-intensity conditioning regimens has allowed elderly patients with preexisting comorbidities access to the potentially curative allogeneic stem cell transplantation. Patient’s comorbidities at the time of treatment consideration play a significant role in transplant outcome in terms of both overall survival (OS) and nonrelapse mortality (NRM). The hematopoietic stem cell transplantation comorbidity index (HCT-CI) quantifies these patient specific risks and has established itself as a major tool in the pretransplant assessment of patients. Many single center and multicenter studies have assessed the HCT-CI score and reported conflicting outcomes. The present study aimed to evaluate the HCT-CI in a single large European transplant centre. 245 patients were retrospectively analyzed and the predictive value of the score was assessed with respect to OS and NRM. We confirm that the HCT-CI predicts outcome for both OS and NRM. Moreover, we identified age of the patient as an independent prognostic parameter for OS. Incorporation of age in the HCT-CI would improve its ability to prognosticate and allow the transplant physician to assess the patient specific risks appropriately at the time of counseling for transplant. Jens M. Chemnitz, Geothy Chakupurakal, Maya Bäßler, Udo Holtick, Sebastian Theurich, Alexander Shimabukuro-Vornhagen, Silke Leitzke, Michael S. Von Bergwelt-Baildon, and Christof Scheid Copyright © 2014 Jens M. Chemnitz et al. All rights reserved. Oral and Dental Considerations in Pediatric Leukemic Patient Tue, 04 Mar 2014 07:29:53 +0000 Throughout the world, there have been drastic decline in mortality rate in pediatric leukemic population due to early diagnosis and improvements in oncology treatment. The pediatric dentist plays an important role in the prevention, stabilization, and treatment of oral and dental problems that can compromise the child’s health and quality of life during, and follow up of the cancer treatment. This manuscript discusses recommendations and promotes dental care of the pediatric leukemic patients. Chiyadu Padmini and K. Yellamma Bai Copyright © 2014 Chiyadu Padmini and K. Yellamma Bai. All rights reserved. The Problem of Anaemia in Patients with Colorectal Cancer Wed, 12 Feb 2014 11:47:07 +0000 Background. Surgical patients are often anaemic preoperatively subsequently requiring blood transfusion. The aim of this study was to assess the problem of anaemia and transfusion rates in patients undergoing surgery for colorectal cancer. Methods. Haemoglobin levels and transfusion requirements were assessed retrospectively in 199 sequential patients operated on for colorectal cancer. This was followed by prospective analysis of 147 patients to correlate preoperative anaemia, stage of bowel cancer, and operation performed with rates of blood transfusion and length of hospital stay. Results. Preoperatively 44% patients were anaemic retrospectively and 60% prospectively. Anaemia increased the risk of transfusion in both studies (69% anaemic versus 31% nonanaemic, in retrospective series, and 83.7% versus 16.3%, in prospective series). Anaemia was proportionally higher in patients with Dukes B (65.2%) and Dukes C (66.6%) than in patients with Dukes A (28.5%). Length of stay was prolonged in transfused patients excluding those requiring major blood transfusion (median 13 versus 7 days, ). Transfusion was also associated with higher mortality (). Conclusion. Anaemia is common in patients with colorectal cancer. Anaemic patients were at high risk of receiving blood transfusion, which in turn increased length of stay and mortality. M. Khanbhai, M. Shah, G. Cantanhede, S. Ilyas, and T. Richards Copyright © 2014 M. Khanbhai et al. All rights reserved. Evaluation of the Utility of Thromboelastography in a Tertiary Trauma Care Centre Wed, 12 Feb 2014 08:05:17 +0000 Background. Thromboelastography (TEG) unlike conventional coagulation assays evaluates the dynamic interaction of clotting factors and platelets indicating an overall clot quality. Literature assessing the efficacy of TEG in identifying trauma associated bleeding is lacking. Aim. To compare TEG with conventional plasma based coagulation tests and assess whether TEG can serve as a screening test or replace the conventional routine test. Materials. Retrospective data was collected for 150 severe trauma patients. Patients with known evidence of severe comorbidities, which may influence the outcome, were excluded. Detailed evaluation of the patient’s clinical and laboratory records was conducted. Diagnostic characteristics such as sensitivity, specificity, and accuracy were calculated. Results. Fifty-one patients were defined as coagulopathic by the conventional coagulation test, 30 by the laboratory established range for TEG indices and 105 by manufactures range. Specificity and sensitivity for the laboratory established range for TEG were 29.4% and 84.8%; for manufactures range sensitivity was 74.5%, specificity was 32.3%. Conclusion. We observed that conventional coagulation assays are the most sensitive tests for diagnosis of coagulopathy due to trauma. However in emergency trauma situations, where immediate corrective measures need to be taken, coagulation parameters and conventional coagulation tests may cause delay. TEG can give highly specific results depicting the underlying coagulopathy. Arulselvi Subramanian, Venencia Albert, Deepak Agrawal, Renu Saxena, and Ravindra Mohan Pandey Copyright © 2014 Arulselvi Subramanian et al. All rights reserved. Association of Body Mass Index with Hemoglobin Concentration and Iron Parameters in Iranian Population Mon, 10 Feb 2014 00:00:00 +0000 Background. Studies have reported that obesity has an adverse effect on iron metabolism. Obesity is characterized by chronic, low-grade, systemic inflammation and anemia of chronic disease with elevated serum ferritin and decreased level of serum iron, transferrin saturation, and hemoglobin. Therefore, we examined the association of body mass index with hemoglobin concentration and iron parameters in this study. Methods. This cross-sectional study was conducted in Yazd to assess the relation of body mass index with hemoglobin and iron parameters among 406 adult patients 18–65 years old. Diabetes and conditions that could influence body iron stores were excluded. Results. There is no difference in hemoglobin concentrations, MCV, serum iron, TIBC, transferrin saturation index, and ferritin between normal weight, overweight, and obese persons. Conclusion. Nutritional status of persons and intake of high iron foods by obese persons should be considered. Also, other inflammatory markers should be evaluated in the future studies. Akram Ghadiri-Anari, Narjes Nazemian, and Hassan-Ali Vahedian-Ardakani Copyright © 2014 Akram Ghadiri-Anari et al. All rights reserved. Clinicohematological Study of Thrombocytosis in Children Wed, 29 Jan 2014 10:02:42 +0000 Introduction. Primary thrombocytosis is very rare in children; reactive thrombocytosis is frequently observed in children with infections, anemia, and many other causes. Aims and Objectives. To identify the etiology of thrombocytosis in children and to analyze platelet indices (MPV, PDW, and PCT) in children with thrombocytosis. Study Design. A prospective observational study. Material and Methods. A total of 1000 patients with thrombocytosis (platelet > ) were studied over a period of 2 years. Platelet distribution width (PDW), mean platelet volume (MPV), and plateletcrit (PCT) were noted. Results. Of 1000 patients, 99.8% had secondary thrombocytosis and only two children had primary thrombocytosis (chronic myeloid leukemia and acute myelogenous leukemia, M7). The majority of the children belonged to the age group of 1month to 2 years (39.7%) and male to female ratio was 1.6 : 1. Infection with anemia (48.3%) was the most common cause of secondary thrombocytosis followed by iron deficiency alone (17.2%) and infection alone (16.2%). Respiratory infection (28.3%) was the predominant infectious cause observed. Thrombocytosis was commonly associated with IDA among all causes of anemia and severity of thrombocytosis increased with severity of anemia (). With increasing platelet count, there was a decrease in MPV (<0.001). Platelet count and mean PDW among children with infection and anemia were significantly higher than those among children with infection alone and anemia alone. None were observed to have thromboembolic manifestations. Conclusions. Primary thrombocytosis is extremely rare in children than secondary thrombocytosis. Infections in association with anemia are most commonly associated with reactive thrombocytosis and severity of thrombocytosis increases with severity of anemia. Nathiya Subramaniam, Suneel Mundkur, Pushpa Kini, Nalini Bhaskaranand, and Shrikiran Aroor Copyright © 2014 Nathiya Subramaniam et al. All rights reserved. Two Cases and Review of the Literature: Primary Percutaneous Angiography and Antiplatelet Management in Patients with Immune Thrombocytopenic Purpura Thu, 26 Dec 2013 11:49:25 +0000 We report two cases of immune thrombocytopenic purpura (ITP) associated with acute coronary artery syndrome highlighting the interventions done in every case along with the medications used during intervention and as outpatient. The first case is that of a woman with ITP exacerbation while on dual antiplatelet therapy and the second case is that of a male presenting with non-ST elevation myocardial infarction (NSTEMI) while in a thrombocytopenic crisis. In both cases antiplatelet therapy was held and thrombopoietic therapy was initiated before resuming full anticoagulation and coronary intervention. Given the paucity of data on ITP and antiplatelets treatment in the setting of acute coronary syndrome, no strict recommendations can be proposed, but antiplatelets appear to be safe acutely and in the long term in this category of patients as long as few measures are undertaken to minimize the risks of bleeding and thrombosis. Estelle Torbey, Harout Yacoub, Donald McCord, and James Lafferty Copyright © 2013 Estelle Torbey et al. All rights reserved. Addition of Multimodal Therapy to Standard Management of Steady State Sickle Cell Disease Mon, 09 Dec 2013 10:03:51 +0000 Most people on folic acid to boost erythropoiesis and prophylactic antimicrobials, the standard management of steady state sickle cell disease (SCD), have unacceptable numbers of crises. The objective of this study was to evaluate the effects of adding multimodal therapy with potassium thiocyanate and omega-3 fatty acids to the standard management of steady state SCD. Pre- and post-treatment numbers of crises and other disease indices were compared in 16 HbSS individuals on folic acid and paludrine after 12 months of adding eicosapentaenoic acid 15 mg/kg/day, docosahexaenoic acid 10 mg/kg/day, and potassium thiocyanate 1-2 mL/day, each milliliter of which contained 250 mg of thiocyanate and 100 micrograms of iodine to prevent hypothyroidism: a possible side-effect due to competitive inhibition of the transport of iodide into the thyroid gland by thiocyanate. Median number of crises reduced from 3/yr to 1/yr (). There was no evidence of impaired thyroid function. Plasma level of tri-iodothyronine improved (). Steady state full blood count and bilirubin level did not change significantly. The findings suggest that addition of potassium thiocyanate and eicosapentaenoic and docosahexaenoic acids to standard management of steady state SCD reduces the number of crises. This observation needs to be evaluated in larger studies. Iheanyi Okpala, Osita Ezenwosu, Anthony Ikefuna, Augustine Duru, Barth Chukwu, Anazoeze Madu, Theresa Nwagha, Sunday Ocheni, Obike Ibegbulam, Ifeoma Emodi, Uche Anike, Charles Nonyelu, Chukwudi Anigbo, Kingsley Agu, Ifeoma Ajuba, Awele Chukwura, Ogechukwu Ugwu, and Uche Ololo Copyright © 2013 Iheanyi Okpala et al. All rights reserved. Assessment of Regulatory T Cells in Childhood Immune Thrombocytopenic Purpura Tue, 05 Nov 2013 09:05:13 +0000 This study had the objective to assess the frequency of Tregs in children newly diagnosed with ITP and ascertain whether an association exists between Tregs and platelet counts, by means of a comparison with healthy controls. This case-control study included 19 patients newly diagnosed with ITP—whose blood samples were collected at four points in time: before any therapy and 1, 3, and 6 months after diagnosis—and 19 healthy controls. Tregs (CD4+ CD25+Foxp3 T cells) were evaluated by flow cytometry. There was a statistically significant difference in platelet count between the case and control groups. There were no significant differences in Treg counts between cases and controls at any point during the course of the study and no difference in Treg counts between the chronic and nonchronic groups and no significant correlation between Tregs and platelet counts in the case and control groups. The findings of this study did not show any statistically significant correlation between Tregs and number of platelets in the case and control groups. Treg cells did not play a role in the regulation of autoimmunity in children with ITP. Karina L. M. Mazzucco, Lauro M. Junior, Natália E. Lemos, Andréa Wieck, Annelise Pezzi, Alvaro M. Laureano, Bruna Amorin, Vanessa Valim, Lucia Silla, Liane E. Daudt, and Paulo J. C. Marostica Copyright © 2013 Karina L. M. Mazzucco et al. All rights reserved. Survival and Prognostic Factors for AIDS and Non-AIDS Patients with Non-Hodgkin’s Lymphoma in Bahia, Brazil: A Retrospective Cohort Study Tue, 29 Oct 2013 11:29:55 +0000 Despite the benefits of HAART, HIV-infected patients are increasingly affected by different malignancies. We compared a 5-year-period survival time and prognostic factors for HIV-1-infected individuals diagnosed with non-Hodgkin lymphomas (NHL) in a nested case-control study, with non-HIV-infected individuals in Salvador, Brazil. Survival time and prognostic factors were compared to HIV-negative patients. 31 cases (versus 63 controls) had a significantly more advanced NHL at diagnosis and lower mean CD4 count (26 cells/mm3) than controls. Mean overall survival (OS) was 35.8 versus 75.4 months, for cases and controls, respectively (), while mean event-free survival time (EFS) was 34.5 months for cases, versus 68.8 for controls (). Higher IPI, increased LDH levels, bone marrow infiltration, lower absolute lymphocyte counts (<1,000 cells/mm3), and type B symptoms were associated with a shorter survival time for cases. Although patients without poorer prognostic factors at baseline had an OS comparable to controls, the mean CD4 cell count for cases was similar for patients with favorable and nonfavorable response to therapy. Our findings suggest that HIV-1 infection is significantly associated with a shorter survival time for patients with NHL, independently of other predictive factors and of disease stage. Estela Luz, Marinho Marques, Ivana Luz, Cristiani Stelitano, Eduardo Netto, Iguaracyra Araújo, and Carlos Brites Copyright © 2013 Estela Luz et al. All rights reserved. Interaction of Normal and Sickle Hemoglobins for Sodium Dodecylsulphate and Hydrogen Peroxide at pH 5.0 and 7.2 Thu, 10 Oct 2013 09:03:36 +0000 Clinical manifestations of malaria primarily result from proliferation of the parasite within the hosts’ erythrocytes. The malaria parasite digests hemoglobin within its digestive vacuole through a sequential metabolic process involving multiple proteases. The activities of these proteases could lead to the production of ROS which could lead to the death of the parasites due to the destruction of their membrane. The action of SDS on hemoglobins can be likened to the way malarial proteases destabilizes host hemoglobin. Hence, the study was designed to determine the binding parameters of SDS and H2O2 for normal, sickle trait carrier and sickle hemoglobins at pH 5.0 and 7.2 using UV-VIS Titration Spectrophotometry. Hb-SDS interactions were significantly different at pH 5.0 but were not at pH 7.2. Also, Hb-H2O2 interactions were statistically different at pH 5.0 and 7.2. The interactions suggest that HbA and HbS are easily destabilized than HbAS and that HbAS has more affinity for H2O2. These suggest a production of more ferryl intermediates or hydroxyl radicals. All these interactions may hinder the development of the malaria parasite at the intraerythrocytic stage and could likely account for a significant proportion of the mechanism that favours the resistance to malaria by individuals with HbAS. Fortunatus C. Ezebuo, Sabinus Oscar O. Eze, Colin B. Lukong, and Ferdinand C. Chilaka Copyright © 2013 Fortunatus C. Ezebuo et al. All rights reserved. The Modern Primitives: Applying New Technological Approaches to Explore the Biology of the Earliest Red Blood Cells Thu, 03 Oct 2013 15:09:11 +0000 One of the most critical stages in mammalian embryogenesis is the independent production of the embryo's own circulating, functional red blood cells. Correspondingly, erythrocytes are the first cell type to become functionally mature during embryogenesis. Failure to achieve this invariably leads to in utero lethality. The recent application of technologies such as transcriptome analysis, flow cytometry, mutant embryo analysis, and transgenic fluorescent gene expression reporter systems has shed new light on the distinct erythroid lineages that arise early in development. Here, I will describe the similarities and differences between the distinct erythroid populations that must form for the embryo to survive. While much of the focus of this review will be the poorly understood primitive erythroid lineage, a discussion of other erythroid and hematopoietic lineages, as well as the cell types making up the different niches that give rise to these lineages, is essential for presenting an appropriate developmental context of these cells. Stuart T. Fraser Copyright © 2013 Stuart T. Fraser. All rights reserved. Portal Hypertension and Myeloproliferative Neoplasms: A Relationship Revealed Mon, 16 Sep 2013 15:11:01 +0000 Background/Objectives. Patients with myeloproliferative neoplasms have a well-established increased risk of thrombosis. Many trials report identification of an underlying myeloproliferative neoplasm by investigation of the patients developing portal hypertensive esophagus and/or fundus variceal hemorrhage in the absence of any known etiology. This trial was designed to investigate the association between myeloproliferative neoplasms and portal hypertension and to detect the frequency of portal hypertension development in this subset of patients. Methodology. Twenty-nine patients previously diagnosed with polycythemia vera, essential thrombocytopenia, and primary myelofibrosis, who were under followup at the hematology outpatient clinic of our hospital, were included in the trial. Results. In our trial, we detected portal hypertension in 13.8% of the patients (), as a finding that was similar to those obtained in other studies performed to date. Conclusions. Considering the fact that diagnosis of myeloproliferative neoplasms usually takes a long time, treatment should be started (while, on the other hand, assessing the investigational and therapeutical choices for the complications) right after the bone marrow biopsy or cytogenetic studies required for establishing the final diagnosis have been performed. Ahmet Burak Toros, Serkan Gokcay, Guven Cetin, Muhlis Cem Ar, Yesim Karagoz, and Besir Kesici Copyright © 2013 Ahmet Burak Toros et al. All rights reserved. Waldenström Macroglobulinemia: Clinical and Immunological Aspects, Natural History, Cell of Origin, and Emerging Mouse Models Mon, 09 Sep 2013 12:02:30 +0000 Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function mutation in the MYD88 adapter protein. This not only provides an intriguing link to new findings that natural effector IgM+IgD+ memory B-cells are dependent on MYD88 signaling, but also supports the hypothesis that WM derives from primitive, innate-like B-cells, such as marginal zone and B1 B-cells. Following a brief review of the clinical aspects and natural history of WM, this review discusses the thorny issue of WM’s cell of origin in greater depth. Also included are emerging, genetically engineered mouse models of human WM that may enhance our understanding of the biologic and genetic underpinnings of the disease and facilitate the design and testing of new approaches to treat and prevent WM more effectively. Siegfried Janz Copyright © 2013 Siegfried Janz. All rights reserved. HBsAg Positive Patient Characteristics in Hospital and Blood Donation Camps Wed, 04 Sep 2013 15:36:36 +0000 Background. Prevention of the residual risk of transfusion transmitted hepatitis B virus infection (HBV) is mostly dependant on serological screening of blood donors for HBsAg and antibody to hepatitis B core antigen (anti-HBc Ab). This study aimed to study the prevalence of HBsAg and anti-HBc Ab and to compare the profile of blood donors attending a blood donation camp and people attending a hospital based camp. Methods. In the blood donor camp, all the blood units were screened for HBV, (HBsAg and anti-HBc), and in the hospital based camp, screening was done for HBsAg alone. Baseline demographic characteristics were noted. Results. The number of blood bank donors was 363 (47.5%) and hospital camp attendees was 402 (52.5%). Prevalence of HBsAg positivity was similar in both the groups at 1.7% and 1.9%, respectively. Anti-HBc Ab positivity (Total) was 6% among the blood donors; Overall prevalence of HBV infection in this group was 3.2%. Conclusion. Policy for checking the collected blood unit by 3 tests for anti-HBc, anti-HBsAb, and HBsAg should be reconsidered to possibly achieve the zero risk goal of transfusion transmitted HBV infection. Blood obtained from a vaccinated donor may give an added protection to the recipient. Deepti Sachan, Joy Varghese, Jensingh Joseph, Vijaya Srinivasan, Venkataraman Jayanthi, and Mohamed Rela Copyright © 2013 Deepti Sachan et al. All rights reserved. Detailed Analysis of Diffuse Large B Cell Lymphoma Patients: A Single-Center, Retrospective Study Tue, 30 Jul 2013 12:14:27 +0000 The aim of this single-center, retrospective study was to investigate the impact of rituximab, reconsider the validity of International Prognostic Index (IPI), and evaluate the prognostic role of the cell of origin (CoO) in a relatively young cohort. Three hundred twelve diffuse large B cell lymphoma patients (median age: 52) were included. Rituximab significantly improved the 3- and 5-year progression free survival (PFS) (70% versus 65% and 41% versus 36%, resp.; ) but led only to a slight, insignificant increase in 3- and 5-year overall survival (OS) (71% versus 77.3% and %67 versus 74.5%, resp.; ). In the young, low risk patient subgroup (aaIPI = 0&1; ), rituximab improved 3- and 5-year PFS and OS rates ( and , resp.). The efficacy of rituximab in young high risk patients was comparable to the literature. CoO data were available in 190 patients. The OS at 3 years was 79% for GC and 64% for non-GC subgroups (). To the best of our knowledge, this is the first study which investigated the impact of R-CHOP in the context of CoO and IPI in a relatively young cohort. CoO was not an independent risk factor for prognosis in the multivariate analysis although patients with GC showed a significant survival advantage in the univariate analysis. CoO was also found to be a significant determinant of response in refractory/relapsed patients. Our results confirm the efficacy of rituximab in low and high risk, young patients outside of a randomized clinical trial setting. Murat Ozbalak, M. Cem Ar, Nukhet Tuzuner, Ayse Salihoglu, A. Emre Eskazan, Seniz Ongoren Aydin, Zafer Baslar, Teoman Soysal, Yildiz Aydin, Anil Barak Dolgun, Onder Ergonul, and Burhan Ferhanoglu Copyright © 2013 Murat Ozbalak et al. All rights reserved. Frequency and Outcome of Graft versus Host Disease after Stem Cell Transplantation: A Six-Year Experience from a Tertiary Care Center in Pakistan Thu, 27 Jun 2013 08:47:31 +0000 Objective. The objective of this study was to evaluate the frequency and outcome of graft versus host disease after stem cell transplantation for various haematological disorders in Pakistan. Materials and Methods. Pretransplant workup of the patient and donor was performed. Mobilization was done with G-CSF 300 μg twice daily for five day. Standard GvHD prophylaxis was done with methotrexate 15 mg/m2 on day +1 followed by 10 mg/m2 on days +3 and +6 and cyclosporine. Grading was done according to the Glucksberg classification. Results. A total of 153 transplants were done from April 2004 to December 2011. Out of these were allogeneic transplants. There were females and males. The overall frequency of any degree of graft versus host disease was 34%. Acute GvHD was present in patients while had chronic GvHD. Grade II GvHD was present in patients while grade III and IV GvHD was seen in patients each. Acute myeloid leukemia and chronic myeloid leukemia were most commonly associated with GvHD. The mortality in acute and chronic GvHD was 8.8% and 12% respectively. Conclusion. The frequency of graft versus host disease in this study was 34% which is lower compared to international literature. The decreased incidence can be attributed to reduced diversity of histocompatibility antigens in our population. Natasha Ali, Salman Naseem Adil, Mohammad Usman Shaikh, and Nehal Masood Copyright © 2013 Natasha Ali et al. All rights reserved. Detection of Minimal Residual Disease by Flow Cytometry for Patients with Multiple Myeloma Submitted to Autologous Hematopoietic Stem Cell Transplantation Thu, 20 Jun 2013 13:28:57 +0000 The treatment strategy in multiple myeloma (MM) is to get complete remission followed by high-dose chemotherapy and autologous Hematopoietic Stem Cell Transplantation (HSCT). Neoplastic Plasma Cells (NPCs) are , , CD138+, CD19−, and   in most cases. The description of this immunophenotype is of major importance as it leads to the correct identification of minimal residual disease (MRD). Samples from 44 Patients were analyzed prospectively in this study. We analyzed if the presence of MRD at three months after HSCT was predictive of relapse or death. There were 40 evaluable patients of whom 16/40 patients had MRD at three moths after HSCT and there were none in cytological relapse. The mean overall survival (OS) was 34 months and disease-free survival (RFS) was 28 months after HSCT. There was no significant difference in the log rank analysis comparing OS and the presence of MRD () and RFS (). Here, we demonstrate that three color flow cytometry (FCM) is more sensitive for MDR evaluation than cytological analyzes. However, based in our data we can not affirm that MRD is a good predictor of MM relapse or death. In conclusion, our results could be attributed to a short followup, small sample size, and over most to the inability of a three-color FCM to detect the NPC population. Suzane Dal Bó, Annelise Pezzi, Bruna Amorin, Vanessa Valim, Rosane Isabel Bittencourt, and Lucia Silla Copyright © 2013 Suzane Dal Bó et al. All rights reserved. Bacteriological Safety of Blood Collected for Transfusion at University of Gondar Hospital Blood Bank, Northwest Ethiopia Thu, 20 Jun 2013 10:48:06 +0000 Background. Transfusion associated bacterial infection has remained more frequent with a sever risk of morbidity and mortality. This study assessed the bacteriological safety of blood collected for transfusion. Method. A cross-sectional study was conducted at University of Gondar hospital blood bank from December 2011 to June 2012. Bacterial isolation, identification, and antimicrobial susceptibility tests were done as per the standard procedure. Chi-square test and value were used to assess associations between risk factors and the bacterial isolation rate. Results. Twenty-one (15.33%) blood units were found contaminated with bacteria, and 95.24% contamination was due to external sources. The commonly isolated bacteria were Staphylococcus aureus, Coagulase negative Staphylococci, Escherichia coli, Klebsiella species, Streptococci species, Enterobacter species, and Citrobacter species. All of the bacteria isolated were 100% sensitive to Gentamicin, Chloramphenicol, Amoxicillin, and Doxycycline. Multiple antimicrobial resistances were observed in 66.7% of the isolates. Not using glove by phlebotomist, touching disinfected phlebotomy site and double puncture at the same hand or both hands of a donor were found to be risk factors for bacterial contamination. Conclusion. Bacterial contamination of blood to be transfused is a common problem in the hospital. So attention should be given to activities performed at the blood bank for safe transfusion practices. Hailegebriel Wondimu, Zelalem Addis, Feleke Moges, and Yitayal Shiferaw Copyright © 2013 Hailegebriel Wondimu et al. All rights reserved. Expression Level of IL-6 Secreted by Bone Marrow Stromal Cells in Mice with Aplastic Anemia Tue, 18 Jun 2013 12:55:18 +0000 Parasecretion of the hematopoietic cytokines is considered as one of the mechanisms account for bone marrow hematopoiesis disorder. In this study, the level of IL-6 secreted by bone marrow stromal cells from a mouse model of aplastic anemia was analyzed. The aplastic anemia mouse model was established with cyclophosphamide in combination with chloramphenicol and 60Coγ radiation. The impairment of bone marrow hematopoiesis induced by irradiation and chemotherapeutic drugs was subsequently characterized by peripheral blood cell count, pathomorphological changes, and apoptosis rate. Furthermore, the in vitro proliferation of bone marrow stromal cells (BMSC) and the IL-6 secretion levels of BMSC were analyzed. In our model of aplastic anemia, the number of peripheral blood cells and bone marrow cells (BMC) were notably decreased, and the apoptosis rate of BMC increased. Furthermore, the proliferation of BMSC was obviously impeded while the IL-6 secretion levels of BMSC significantly increased. The findings of our study suggested that the IL-6 secretion level may be enhanced to some extent by the induction of aplastic anemia caused by irradiation and chemotherapeutic drugs and that the abnormal level of IL-6 might probably interfere with the stability of the bone marrow hematopoietic microenvironment. Yong Feng Chen, Zhong Min Wu, Cong Xie, Shi Bai, and Li Dong Zhao Copyright © 2013 Yong Feng Chen et al. All rights reserved. Hematological and Genetic Predictors of Daytime Hemoglobin Saturation in Tanzanian Children with and without Sickle Cell Anemia Wed, 03 Apr 2013 08:59:10 +0000 Low hemoglobin oxygen saturation (SpO2) is common in Sickle Cell Anemia (SCA) and associated with complications including stroke, although determinants remain unknown. We investigated potential hematological, genetic, and nutritional predictors of daytime SpO2 in Tanzanian children with SCA and compared them with non-SCA controls. Steady-state resting pulse oximetry, full blood count, transferrin saturation, and clinical chemistry were measured. Median daytime SpO2 was 97% (IQ range 94–99%) in SCA (N = 458), lower () than non-SCA (median 99%, IQ range 98–100%; N = 394). Within SCA, associations with SpO2 were observed for hematological variables, transferrin saturation, body-mass-index z-score, hemoglobin F (HbF%), genotypes, and hemolytic markers; mean cell hemoglobin (MCH) explained most variability (, Adj ). In non-SCA only age correlated with SpO2. -thalassemia 3.7 deletion highly correlated with decreased MCH (Pearson correlation coefficient 0.60, ). In multivariable models, lower SpO2 correlated with higher MCH (-coefficient 0.32, ) or with decreased copies of -thalassemia 3.7 deletion (-coefficient 1.1, ), and independently in both models with lower HbF% (-coefficient 0.15, ) and Glucose-6-Phosphate Dehydrogenase genotype (-coefficient 1.12, ). This study provides evidence to support the hypothesis that effects on red cell rheology are important in determining SpO2 in children with SCA. Potential mechanisms and implications are discussed. Sharon E. Cox, Julie Makani, Charles R. Newton, Andrew M. Prentice, and Fenella J. Kirkham Copyright © 2013 Sharon E. Cox et al. All rights reserved. Assessment of Hepatic and Pancreatic Iron Overload in Pediatric Beta-Thalassemic Major Patients by Weighted Gradient Echo Magnetic Resonance Imaging Thu, 28 Mar 2013 08:05:57 +0000 Background. MRI has emerged for the noninvasive assessment of iron overload in various tissues. The aim of this paper is to evaluate hepatic and pancreatic iron overload by weighted gradient echo MRI in young beta-thalassemia major patients and to correlate it with glucose disturbance and postsplenectomy status. Subjects and Methods. 50 thalassemic patients, in addition to 15 healthy controls. All patients underwent clinical assessment and laboratory investigations. Out of 50 thalassemic patients, 37 patients were splenectomized. MRI was performed for all subjects. Results. All patients showed significant reduction in the signal intensity of the liver and the pancreas on GRD compared to controls, thalassemic patients who had abnormal glucose tolerance; diabetic and impaired glucose tolerance patients displayed a higher degree of pancreatic and hepatic siderosis and more drop in their signal intensity than those with normal blood sugar level. Splenectomized thalassemic patients had significantly lower signal intensity of the liver and pancreas compared to nonsplenectomized patients. Conclusion. gradient echo MRI is noninvasive highly sensitive method in assessing hepatic and pancreatic iron overload in thalassemic patients, more evident in patients with abnormal glucose tolerance, and is accelerated in thalassemic splenectomized patients. Doaa Mohammed Youssef, Faten Fawzy Mohammad, Ayman Ahmed Fathy, and Maha Aly Abdelbasset Copyright © 2013 Doaa Mohammed Youssef et al. All rights reserved. Hemoglobin A2 Lowered by Iron Deficiency and α-Thalassemia: Should Screening Recommendation for β-Thalassemia Change? Tue, 12 Mar 2013 14:23:03 +0000 Screening for β-thalassemia trait (BTT) relies on measuring hemoglobin (Hb) A2. Since multiple factors can affect HbA2 levels, the screening can become unreliable. In 1356 healthy Arabs enrolled into a federally funded premarital BTT screening program, the effects of iron deficiency (ID), -thalassemia trait, gender, smoking, and tribalism on HbA2 were studied. The complete blood count and hemoglobin fractions were determined on the entire cohort; serum ferritin (<15 μg/L) in 391 subjects was used to determine ID. BTT was present in 29 (2.1%) subjects (HbA2 > 3.5%). Among 77(20.3%) subjects with ID, the mean HbA2 (%) was 0.2% lower than in subjects without iron deficiency (%, ). In 65 (38%)/172 subjects with phenotypic -thalassemia trait, the mean HbA2 (%) was 0.13% lower than in subjects without -thalassemia trait, . The mean HbA2 did not differ between males and females, smokers and nonsmokers, and between the tribes. Thus, 35 (2.6%) subjects with HbA2 between 3.2 and 3.5% were at a risk of false negative diagnosis of BTT. Since iron deficiency and -thalassemia are both common and both lower HbA2, modifications in screening recommendations for BTT are proposed. Srdjan Denic, Mukesh M. Agarwal, Bayan Al Dabbagh, Awad El Essa, Mohamed Takala, Saad Showqi, and Javed Yassin Copyright © 2013 Srdjan Denic et al. All rights reserved. MicroRNAs and Glucocorticoid-Induced Apoptosis in Lymphoid Malignancies Tue, 29 Jan 2013 09:39:03 +0000 The initial response of lymphoid malignancies to glucocorticoids (GCs) is a critical parameter predicting successful treatment. Although being known as a strong inducer of apoptosis in lymphoid cells for almost a century, the signaling pathways regulating the susceptibility of the cells to GCs are only partly revealed. There is still a need to develop clinical tests that can predict the outcome of GC therapy. In this paper, I discuss important parameters modulating the pro-apoptotic effects of GCs, with a specific emphasis on the microRNA world comprised of small players with big impacts. The journey through the multifaceted complexity of GC-induced apoptosis brings forth explanations for the differential treatment response and raises potential strategies for overcoming drug resistance. Ronit Vogt Sionov Copyright © 2013 Ronit Vogt Sionov. All rights reserved. CD47: A Cell Surface Glycoprotein Which Regulates Multiple Functions of Hematopoietic Cells in Health and Disease Mon, 21 Jan 2013 15:52:08 +0000 Interactions between cells and their surroundings are important for proper function and homeostasis in a multicellular organism. These interactions can either be established between the cells and molecules in their extracellular milieu, but also involve interactions between cells. In all these situations, proteins in the plasma membranes are critically involved to relay information obtained from the exterior of the cell. The cell surface glycoprotein CD47 (integrin-associated protein (IAP)) was first identified as an important regulator of integrin function, but later also was shown to function in ways that do not necessarily involve integrins. Ligation of CD47 can induce intracellular signaling resulting in cell activation or cell death depending on the exact context. By binding to another cell surface glycoprotein, signal regulatory protein alpha (SIRPα), CD47 can regulate the function of cells in the monocyte/macrophage lineage. In this spotlight paper, several functions of CD47 will be reviewed, although some functions may be more briefly mentioned. Focus will be on the ways CD47 regulates hematopoietic cells and functions such as CD47 signaling, induction of apoptosis, and regulation of phagocytosis or cell-cell fusion. Per-Arne Oldenborg Copyright © 2013 Per-Arne Oldenborg. All rights reserved.