Editorial Board
Publication date: February 2020Source: Blood Cells, Molecules, and Diseases, Volume 80Author(s): (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - November 14, 2019 Category: Hematology Source Type: research

Czech and Slovak Diamond-Blackfan Anemia (DBA) Registry update: Clinical data and novel causative genetic lesions
Publication date: Available online 11 November 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Jana Volejnikova, Petr Vojta, Helena Urbankova, Renata Mojzíkova, Monika Horvathova, Ivana Hochova, Jaroslav Cermak, Jan Blatny, Martina Sukova, Eva Bubanska, Jaroslava Feketeova, Daniela Prochazkova, Julia Horakova, Marian Hajduch, Dagmar PospisilovaAbstractDiamond-Blackfan anemia (DBA) is a rare congenital erythroid aplasia, underlied by haploinsufficient mutations in genes coding for ribosomal proteins (RP) in approximately 70% of cases. DBA is frequently associated with somatic malformations, endocrine dysfu...
Source: Blood Cells, Molecules, and Diseases - November 13, 2019 Category: Hematology Source Type: research

Plasma adiponectin is a potential biomarker for organ involvement in male Fabry disease patients
Publication date: Available online 30 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Marina Hovakimyan, Venakta Ajay Narendra Talabattula, Claudia Cozma, Christian Beetz, Arndt Rolfs, Deborah ElsteinAbstractFabry disease is an X-linked lysosomal storage disorder caused by pathogenic variants in GLA. It manifests in hemizygous males and in many heterozygous females. Cardiovascular and renal involvement are frequent. Adiponectin is a circulating hormone that has been linked to numerous disease conditions including heart and kidney failure. In the present pilot study, we investigated plasma adiponectin lev...
Source: Blood Cells, Molecules, and Diseases - November 1, 2019 Category: Hematology Source Type: research

Urinary cross-linked carboxyterminal telopeptide, a bone resorption marker, decreases after vaso-occlusive crises in adults with sickle cell disease
Publication date: Available online 24 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Oyebimpe O. Adesina, Isaac C. Jenkins, Qian V. Wu, Ellen B. Fung, Radhika R. Narla, Edward W. Lipkin, Kanika Mahajan, Barbara A. Konkle, Rebecca Kruse-JarresAbstractPeople with sickle cell disease often report severe bone pain with repeated bouts of vaso-occlusive crises, but the extent of skeletal injury incurred during these painful episodes remain unclear. We sought to quantify bone degradation by comparing urinary concentrations of carboxyterminal cross-linked telopeptide of type I collagen (CTX-1), a well-described...
Source: Blood Cells, Molecules, and Diseases - October 26, 2019 Category: Hematology Source Type: research

Polymorphisms in genes that affect the variation of lipid levels in a Brazilian pediatric population with sickle cell disease: rs662799 APOA5 and rs964184 ZPR1
Publication date: Available online 22 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Thaisa Netto Souza Valente-Frossard, Nilcemar Rodrigues Carvalho Cruz, Fernanda Oliveira Pereira, Andre Rolim Belisario, Brisa Machado Pereira, Antônio Frederico de Freitas Gomides, Glaucia Aparecida Domingos Resende, Aline Menezes Carlos, Helio Moraes-Souza, Cibele Velloso-RodriguesAbstractThis cross-sectional study investigated associations between SNPs in metabolizing lipid genes, alpha-thalassemia and laboratory parameters in two forms of sickle cell disease (SCD), sickle cell anemia (SCA) and hemoglobin SC di...
Source: Blood Cells, Molecules, and Diseases - October 23, 2019 Category: Hematology Source Type: research

Hereditary xerocytosis - spectrum and clinical manifestations of variants in the PIEZO1 gene, including co-occurrence with a novel β-globin mutation
Publication date: Available online 22 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Karolina Maciak, Anna Adamowicz-Salach, Alicja Siwicka, Jaroslaw Poznanski, Tomasz Urasinski, Danuta Plochocka, Monika Gora, Beata BurzynskaAbstractHereditary xerocytosis (HX) is a rare, autosomal dominant congenital hemolytic anemia (CHA) characterized by erythrocyte dehydration with presentation of various degrees of hemolytic anemia. HX is often misdiagnosed as hereditary spherocytosis or other CHA. Here we report three cases of suspected HX and one case of HX associated with β-thalassemia.Sanger method was used...
Source: Blood Cells, Molecules, and Diseases - October 23, 2019 Category: Hematology Source Type: research

High red blood cell distribution width might predict thrombosis in essential thrombocythemia and polycythemia vera
Publication date: Available online 21 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Ivan Krečak, Filip Krečak, Velka Gverić-Krečak (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - October 23, 2019 Category: Hematology Source Type: research

White blood cell labeling with Technetium-99m (99mTc) using red blood cell extracellular vesicles-mimetics
Publication date: Available online 20 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Seung Hyun Son, Ji Min Oh, Prakash Gangadaran, Hyun Dong Ji, Ho Won Lee, Ramya Lakshmi Rajendran, Se Hwan Baek, Arunnehru Gopal, Senthilkumar Kalimuthu, Shin Young Jeong, Sang-Woo Lee, Jaetae Lee, Byeong-Cheol AhnAbstractBackgroundExtracellular vesicles, have gained increasing attention for their application in drug delivery. Here, we developed a novel method for radiolabeling WBCs with 99mTc using RBC-derived extracellular vesicles -mimetics (EVMs), and monitored in vivo inflammation tracking of 99mTc-WBC using gamma c...
Source: Blood Cells, Molecules, and Diseases - October 21, 2019 Category: Hematology Source Type: research

Allergy and inhibitors in hemophilia - a rare complication with potential novel solutions
DiscussionOur case series illustrates the difficulty in managing patients with anaphylaxis to replacement therapy. In the era of novel therapies, such as emicizumab, the management of HA patients who experience anaphylaxis to replacement therapy is becoming easier and may obviate the need for ITI. Current treatment strategies for HB patients with such anaphylaxis, however, are limited to rFVIIa, and it continues to pose a challenge. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - October 21, 2019 Category: Hematology Source Type: research

The role of a low erythropoietin level in the diagnosis of JAK2 exon 12-mutated polycythemia vera
Publication date: Available online 20 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Stephen E. Langabeer (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - October 21, 2019 Category: Hematology Source Type: research

Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia
This study was aimed to identify the predictors of splenic sequestration crisis (SSC) among pediatric patients with sickle cell disease (SCD).This prognosis study was carried out in the pediatric immuno-hematology unit, over 20 years (1998 to 2017), enrolling patients with SCD. The cox model was used in multivariate analysis.Among 423 patients with SCD (240 S/S phenotype, 128 S/B0, 30 S/B+, 14 S/O arab and 11 S/C), 150(35.4%) had at least one episode of SSC. The average age of patients at the first episode was 48.3 months ± 32.4(2–168). Recurrence of SSC was observed in 117 patients (78%). Spleen size ≥...
Source: Blood Cells, Molecules, and Diseases - October 20, 2019 Category: Hematology Source Type: research

Analysis of platelets by flow cytometry in patients with Paroxysmal Nocturnal Hemoglobinuria (PNH)
Publication date: Available online 18 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): David J. Araten, Daniel Boxer, Leah Zamechek, Erik Sherman, Michael NardiAbstractThe marked pro-thrombotic tendency in PNH is likely to be at least partly due to the population of platelets derived from the abnormal stem cell clone. However, identification of GPI (−) platelets by flow cytometry can be technically difficult. Here we describe a technique that involves the addition of aspirin immediately after the separation of platelet rich plasma and the use of gel filtration to isolate platelets away from plasma p...
Source: Blood Cells, Molecules, and Diseases - October 18, 2019 Category: Hematology Source Type: research

Platelet function defects in patients with Gaucher disease on long term ERT- implications for evaluation at bleeding challenges
Publication date: Available online 18 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Komninaka Veroniki, Repa Konstantina, Marinakis Theodoros, Pouliakis Abraham, Koutsouri Theodora, Tsokanas Dimitrios, Pagona Flevary, Ersi Voskaridou, Politou Marianna (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - October 18, 2019 Category: Hematology Source Type: research

Gaucher disease, myelodysplastic syndrome and ICUS
Publication date: Available online 18 October 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Rosa Ruchlemer, Moshe Mittelman, Ari ZimranAbstractGaucher disease is an inherited lysosomal storage disease commonly associated with hepatosplenomegaly and cytopenias. Progressive cytopenias may be interpreted as an indication of advanced disease and suggest the need to start Gaucher disease specific treatment. As bone marrow evaluation is not routinely performed in Gaucher disease, other causes of cytopenias such as myelodysplastic syndrome a stem cell disorder may be missed. Six patients are described who suffered si...
Source: Blood Cells, Molecules, and Diseases - October 18, 2019 Category: Hematology Source Type: research

Editorial Board
Publication date: November 2019Source: Blood Cells, Molecules, and Diseases, Volume 79Author(s): (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - September 11, 2019 Category: Hematology Source Type: research

The role of a low erythropoietin level for the polycythemia vera diagnosis
Publication date: Available online 7 September 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Oleksandra Lupak, Xiaoxia Han, Peter Xie, Sharmeen Mahmood, Hadi Mohammed, Vijayalakshmi DonthireddyAbstractA low erythropoietin (EPO) level is a minor diagnostic criterion for polycythemia vera (PV). Controversies exist regarding the diagnostic value of a low EPO level when considering increasing availability of advanced molecular testing. We assessed the role of low EPO level for PV diagnosis in the context of positive JAK2 mutation status as well as other diagnostic parameters. Of 138 patients, 75 patients had PV an...
Source: Blood Cells, Molecules, and Diseases - September 8, 2019 Category: Hematology Source Type: research

BRAF V600E mutation in childhood Langerhans cell histiocytosis correlates with multisystem disease and poor survival
Publication date: Available online 30 August 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Prateek Bhatia, Minu Singh, Madhulika Sharma, Ajay Kumar, Nandita Kakkar, S. Radhika, Amita Trehan, Deepak BansalAbstractLangerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia of children with systemic involvement and poor outcome. The altered RAS-RAF-MEK-ERK cell signalling pathway due to somatic mutation of BRAF V600E is the most common genetic abnormality associated with the disease. In the current study, we highlight the frequency of BRAF V600E in our cohort of LCH cases (n = 31) and its relatio...
Source: Blood Cells, Molecules, and Diseases - August 31, 2019 Category: Hematology Source Type: research

Reflections on the crooked timber of red blood cell physiology1
Publication date: Available online 17 August 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Joseph F. Hoffman (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - August 18, 2019 Category: Hematology Source Type: research

High erythropoietin may be associated with vascular complications in patients with secondary erythrocytosis caused by high oxygen affinity variant hemoglobin Coimbra
Publication date: Available online 15 August 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Bruna Cunha Santos, Susan Elisabeth Jorge, Dulcinéia Martins de Albuquerque, Simone Cristina Olenscki Gilli, Maria de Fátima Sonati, Kleber Yotsumoto Fertrin, Fernando Ferreira Costa (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - August 17, 2019 Category: Hematology Source Type: research

Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia
We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32 mm2/unit area (range 5–5248) rising to 3268 RBCs per 32 mm2/unit area (range 49–18,368, P = 0.004), under ambient and hypoxic conditions, respectively (n = 14). This was not seen in RBCs from men without a history of priapism (median 402 (range 14–785) and 122 (range 31–4112) RBCs per 32 mm2/unit area), ambient and hypoxic conditions, respective...
Source: Blood Cells, Molecules, and Diseases - August 7, 2019 Category: Hematology Source Type: research

Comprehensive analysis the potential biomarkers for the high-risk of childhood acute myeloid leukemia based on a competing endogenous RNA network
Publication date: Available online 2 August 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Nan Zhang, Ying Chen, Yan Shen, Shifeng Lou, Jianchuan DengAbstractAcute myeloid leukemia (AML) is a common form of hematological malignancies, the discovery of non-coding RNA (ncRNA) plays an important role in diverse biological processes including hematopoietic differentiation and proliferation. However, the interaction mechanism of key RNAs and their regulatory network in childhood AML are still to be elucidated. RNA profiles were downloaded from the Therapeutically Applicable Research to Generate Effective Treatment (...
Source: Blood Cells, Molecules, and Diseases - August 4, 2019 Category: Hematology Source Type: research

Factors affecting thrombohemorrhagic early death in patients with acute promyelocytic leukemia treated with arsenic trioxide alone
This study identified risk factors for TH-ED in a large cohort of patients with APL, which enriched clinical information on identifying patients at high risk of TH-ED. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - August 2, 2019 Category: Hematology Source Type: research

Elevated surface-bound complement FH alters the function of platelets and monocytes in FHR1/3 null healthy individuals
Publication date: Available online 22 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Angika Bhasym, Teena Bhakuni, Prasenjit GuchhaitAbstractComplement factor H (FH) and FH-related proteins (FHRs), structurally similar proteins are involved in the regulation of complement activation. Homozygous deletion of FHR 1 and 3 proteins (FHR1/3−/−) is known as a risk factor for disorders such as aHUS and SLE, characterised by thrombo-inflammatory complications. Interestingly, FHR1/3−/− genotype also exists as polymorphism in healthy population of various ethnicities around the world including...
Source: Blood Cells, Molecules, and Diseases - July 22, 2019 Category: Hematology Source Type: research

Combined genetic disruption of K-Cl cotransporters and Gardos channel KCNN4 rescues erythrocyte dehydration in the SAD mouse model of sickle cell disease
We describe here generation of mice genetically deficient in the three major red cell volume regulatory gene products, Kcnn4, Kcc3 and Kcc1 in C57BL6 non-sickle and SAD sickle backgrounds. We show that combined loss-of-function of all three gene products in SAD mice leads to incrementally increased MCV, decreased CHCM and % hyperchromic cells, decreased red cell density (phthalate method), increased resistance to hypo-osmotic lysis, and increased cell K content. The data show that combined genetic deletion of the Gardos channel and K-Cl cotransporters in a mouse SCD model decreases red cell density and improves several hem...
Source: Blood Cells, Molecules, and Diseases - July 19, 2019 Category: Hematology Source Type: research

Editorial Board
Publication date: September 2019Source: Blood Cells, Molecules, and Diseases, Volume 78Author(s): (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - July 16, 2019 Category: Hematology Source Type: research

Pediatric cerebral sinovenous thrombosis: Optimal treatment may differ by etiology
Publication date: Available online 11 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Adriana Gonzalez Torriente, Lisa R. Sun (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - July 13, 2019 Category: Hematology Source Type: research

Co-inheritance of Southeast Asian Ovalocytosis (SAO) and G6PD deficiency associated with acute hemolysis in a Thai patient
Publication date: Available online 11 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Kannadit Prayongratana, Vip Viprakasit (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - July 13, 2019 Category: Hematology Source Type: research

Conjugate prodrug AN-233 induces fetal hemoglobin expression in sickle erythroid progenitors and β-YAC transgenic mice
Publication date: Available online 9 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Aluya R. Oseghale, Xingguo Zhu, Biaoru Li, Kenneth R. Peterson, Abraham Nudelman, Ada Rephaeli, Hongyan Xu, Betty S. PaceAbstractPharmacologic induction of fetal hemoglobin (HbF) is an effective strategy for treating sickle cell disease (SCD) by ameliorating disease severity. Hydroxyurea is the only FDA-approved agent that induces HbF, but significant non-responders and requirement for frequent monitoring of blood counts for drug toxicity limit clinical usefulness. Therefore, we investigated a novel prodrug conjugate of but...
Source: Blood Cells, Molecules, and Diseases - July 10, 2019 Category: Hematology Source Type: research

Phenazine methosulphate-treated red blood cells activate NF-κB and upregulate endothelial ICAM-1 expression
Publication date: Available online 5 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Rani Kaliyaperumal, Jianhe Wang, Herbert J. Meiselman, Björn NeuAbstractAlthough enhanced Red Blood Cell (RBC) - Endothelial Cell (EC) interaction, as well as RBC induced EC activation, have been extensively studied in several RBC-linked pathologies, the specific individual effects of oxidatively modified RBC on EC activation has not yet been documented. However, increasing evidence in both experimental and clinical studies suggests that oxidatively modified RBC could be considered potential pathogenic determinants in ...
Source: Blood Cells, Molecules, and Diseases - July 7, 2019 Category: Hematology Source Type: research

Serum calprotectin: A circulating biomarker of the inflammatory state in Philadelphia-negative myeloproliferative neoplasms
Publication date: Available online 6 July 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Ivan Krečak, Filip Krečak, Velka Gverić-Krečak, Iva Bilandžija, Pavle Rončević, Ksenija Fumić, Josip Batinić, Nadira Duraković, Maja Radman (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - July 7, 2019 Category: Hematology Source Type: research

Predictors of autoimmune hemolytic anemia in beta-thalassemia patients with underlying red blood cells autoantibodies
Publication date: Available online 29 June 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Monia Ben Khaled, Monia Ouederni, Nessrine Sahli, Nawel Dhouib, Ahmed Ben Abdelaziz, Samia Rekaya, Ridha Kouki, Houda Kaabi, Hmida Slama, Fethi Mellouli, Mohamed BejaouiAbstractIn beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia patients with positive Direct Antiglobulin Test (DAT), in Tunisia.This longitudinal prognosis study was carried out on beta-thalassemia patients with a p...
Source: Blood Cells, Molecules, and Diseases - July 1, 2019 Category: Hematology Source Type: research

Evaluation of double heptamer-type sgRNA as a potential therapeutic agent against multiple myeloma
Publication date: Available online 12 June 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Tatsuya Ishikawa, Arisa Haino, Takashi Ichiyanagi, Mineaki Seki, Masayuki NashimotoAbstractEmergence of drug-resistant mutations in the course of myeloma cell evolution and subsequent relapse of myeloma appears to be currently inevitable in most patients. To remedy this situation, we are trying to develop therapeutic small guide RNAs (sgRNAs) based on tRNase ZL-utilizing efficacious gene silencing (TRUE gene silencing), an RNA-mediated gene expression control technology. We designed two sets of double heptamer-type sgRNA, ...
Source: Blood Cells, Molecules, and Diseases - June 13, 2019 Category: Hematology Source Type: research

Mechanism underlying N-(3-oxo-dodecanoyl)-L-homoserine lactone mediated intracellular calcium mobilization in human platelets
In this study, we explored the mechanism of AHLs induced calcium mobilization in human platelets. We found that 3-oxo-C12 HSL but not C4-HSL induces intracellular calcium release. 3-oxo-C12 HSL induced calcium mobilization was majorly contributed from the dense tubular system (DTS). Furthermore, 3-oxo-C12 HSL also stimulates the store-operated Ca2+ entry (SOCE) in platelet. Intracellular calcium rise was significantly lowered in rotenone, and bafilomycin pre-treated platelets suggesting partial involvement of mitochondria and acidic vacuoles. The significant effect of 3-oxo-C12 HSL on calcium mobilization can alter the pla...
Source: Blood Cells, Molecules, and Diseases - May 22, 2019 Category: Hematology Source Type: research

Editorial Board
Publication date: July 2019Source: Blood Cells, Molecules, and Diseases, Volume 77Author(s): (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - May 12, 2019 Category: Hematology Source Type: research

No increased cerebrovascular involvement in adult beta-thalassemia by advanced MRI analyses
Publication date: Available online 2 May 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Andrea Gerardo Russo, Sara Ponticorvo, Immacolata Tartaglione, Martina Caiazza, Domenico Roberti, Andrea Elefante, Maddalena Casale, Rosanna Di Concilio, Angela Ciancio, Elisa De Michele, Antonietta Canna, Mario Cirillo, Silverio Perrotta, Fabrizio Esposito, Renzo ManaraAbstractBeta-thalassemia-related anemia and chronic hypercoagulative state are supposed to cause cumulative cerebrovascular damage with consequent parenchymal/vascular changes and functional impairment. However, recent conventional MRI/MR-angiography investig...
Source: Blood Cells, Molecules, and Diseases - May 3, 2019 Category: Hematology Source Type: research

EPO-R+ myelodysplastic cells with ring sideroblasts produce high erythroferrone levels to reduce hepcidin expression in hepatic cells
In this study, we examined the level of ERFE mRNA during ex vivo erythroid differentiation using cord blood CD34+ cells and we further analyzed whether ERFE could be produced by MDS cells using a public database (GSE58831). ERFE mRNA was increased during normal erythroid differentiation. An analysis of GSE58831 indicated that ERFE expression in bone marrow (BM) MDS cells was higher than that in healthy volunteer (HV)–derived BM cells. ERFE expression significantly and positively correlated with the expression of erythropoietin (EPO) receptors (EPO-R), ALAS2 (5’-Aminolevulinate Synthase 2), STEAP3 (STEAP family ...
Source: Blood Cells, Molecules, and Diseases - April 30, 2019 Category: Hematology Source Type: research

Autophagy-deficient mice are more susceptible to engrafted leukemogenesis
In this study, conditional knockout mice Atg7f/f;Ubc-Cre, in which an autophagy-essential gene Atg7 is universally deleted, were used as recipients, B-ALL cell line 697 was used as donor cells to generate leukemia mouse model. Compared to wild-type mice, Atg7 knockout mice were more susceptible to engrafted leukemogenesis, shown by increase in white blood cells, lymphocytes, and platelets, decrease in HSPC number and its colony-forming unit (CFU). The liver and spleen displayed hepatosplenomegaly and inflammatory cell infiltration. Furthermore, second competitive transplantation revealed dysfunction of the HSPC in Atg7-kno...
Source: Blood Cells, Molecules, and Diseases - April 29, 2019 Category: Hematology Source Type: research

CD133+ bone marrow stem cells (BMSC) control platelet activation – Role of ectoNTPDase-1 (CD39)
ConclusionWe provide evidence that CD133+BMSC are capable of controlling ADP-dependent platelet aggregation and activation by direct interaction dependent on cellular expression of ectoNTPDase-1. Whether different capacities of BMSC modulate platelet-depending thrombogenicity at sites of regeneration impact effectiveness and adverse event profiles of regenerative treatment requires further evaluation. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 27, 2019 Category: Hematology Source Type: research

The Growth Differentiation Factor-15 (GDF-15) levels are increased in patients with compound heterozygous sickle cell and beta-thalassemia (HbS/βthal), correlate with markers of hemolysis, iron burden, coagulation, endothelial dysfunction and pulmonary hypertension
Publication date: Available online 23 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Katerina Larissi, Marianna Politou, Alexandra Margeli, Christos Poziopoulos, Pagona Flevari, Evangelos Terpos, Ioannis Papassotiriou, Ersi VoskaridouAbstractThe clinical manifestations of Sickle Cell Disease (SCD) include episodes of vascular occlusion, chronic hemolytic anemia and frequent infections. GDF-15, a multifactorial cytokine, is a member of the transforming growth factor- superfamily. Expression of the GDF-15 gene in cardiomyocytes, vascular smooth muscle cells, and endothelial cells is strongly upregulated in ...
Source: Blood Cells, Molecules, and Diseases - April 24, 2019 Category: Hematology Source Type: research

Reversal of acquired von Willebrand syndrome with allogeneic stem cell transplant for chronic lymphocytic leukemia
Publication date: Available online 18 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Livia Hegerova, Fiona He, Nicole D. Zantek, Gregory M. Vercellotti, Shernan G. Holtan, Mark T. RedingAbstractAcquired von Willebrand syndrome (AVWS) is a rare, potentially fatal bleeding disorder caused by low activity of von Willebrand factor (VWF) in patients without congenital deficiency. The majority of adult cases are associated with hematological malignancy, including lymphoproliferative (48%) or myeloproliferative (15%) disorders (Federici et al., 2000). Both qualitative and quantitative defects occur, due to antib...
Source: Blood Cells, Molecules, and Diseases - April 18, 2019 Category: Hematology Source Type: research

Identification of promising prognostic genes for relapsed acute lymphoblastic leukemia
ConclusionTranscription and focal adhesion might play important roles in ALL progression. Furthermore, genes including ALPK1, ZNF695, ACTN4, CALR, and FBXL5 might be novel prognostic genes for relapsed ALL. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 18, 2019 Category: Hematology Source Type: research

Monocytes-neutrophils-ratio as predictive marker for failure of first induction therapy in AML
ConclusionsWe propose a novel and non-invasive method for detection of failure of first induction therapy in patients with de novo AML and “7 + 3” induction therapy. The MNR is free of cost since the required cell counts are performed routinely for each patient undergoing intensive chemotherapy. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 18, 2019 Category: Hematology Source Type: research

Cytogenetic evolution in myeloproliferative neoplasms with different molecular abnormalities
Publication date: Available online 13 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Seon Young Kim, Mosae Koo, Yumi Park, Hyunjin Kim, Qute Choi, Ik-Chan Song, Deog-Yeon Jo, Jimyung Kim, Gye Cheol Kwon, Sun Hoe KooAbstractWe investigated the changes in chromosomal abnormalities in myeloproliferative neoplasm (MPN) patients during long-term follow-up. In total, 28 MPN patients (22 with primary myelofibrosis and 6 with polycythemia vera) were included. Among them, 25 patients underwent serial bone marrow (BM) biopsies during disease progression, and 3 patients had cytogenetic abnormalities at initial diagn...
Source: Blood Cells, Molecules, and Diseases - April 14, 2019 Category: Hematology Source Type: research

A retrospective study on clinical manifestations of neonates with FXIII-A deficiency
Publication date: Available online 11 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Majid Naderi, Nader Cohan, Iraj Shahramian, Ghasem Miri-Aliabad, Sezaneh Haghpanah, Mahmood Imani, Mohamad Moghadam, Abdollah Dehvari, Akbar Dorgalaleh, Mehran KarimiAbstractWe assessed clinical presentations and the rate of central nervous system (CNS) bleeding in neonates with FXIIID who exhibited bleeding diathesis in the early days of their lives. A total of 27 neonates presented bleeding or abnormal clinical symptoms, diagnosed with FXIII deficiency were evaluated. Factor XIII concentrate was initiated as the first-l...
Source: Blood Cells, Molecules, and Diseases - April 12, 2019 Category: Hematology Source Type: research

Addendum to Letter to the Editor: Safety, efficacy, and authorization of eliglustat as a first-line therapy in Gaucher disease type 1
Publication date: Available online 9 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Pramod K. Mistry, Manisha Balwani, Hagit N. Baris, Hadhami Ben Turkia, T. Andrew Burrow, Joel Charrow, Gerald F. Cox, Sumita Danda, Marta Dragosky, Guillermo Drelichman, Amal El-Beshlawy, Cristina Fraga, Selena Freisens, Sebastiaan Gaemers, Evgueniy Hadjiev, Priya S. Kishnani, Elena Lukina, Pierre Maison-Blanche, Ana Maria Martins, Gregory Pastores (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 11, 2019 Category: Hematology Source Type: research

Scurvy of modern age: Rare presentation with pancytopenia
Publication date: Available online 8 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Amos Lal, Ajay Kumar Mishra, Kamal Kant Sahu, Mohsen Noreldin (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 9, 2019 Category: Hematology Source Type: research

Screening umbilical cord blood for congenital Iron deficiency
ConclusionsAmong SGA, IDM, and VLBW neonates, maternal obesity is a risk factor for congenital iron deficiency. We speculate that elevated hepcidin levels in obese pregnant women impede iron absorption and interfere with transplacental iron transfer. (Source: Blood Cells, Molecules, and Diseases)
Source: Blood Cells, Molecules, and Diseases - April 9, 2019 Category: Hematology Source Type: research

Pagophagia in men with iron-deficiency anemia
Publication date: Available online 7 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): James C. Barton, J. Clayborn Barton, Luigi F. BertoliAbstractFew case series of pagophagia and iron deficiency include men. We performed a retrospective study of non-Hispanic white men with iron-deficiency anemia whose anemia and pagophagia, thrombocytosis, and thrombocytopenia (if present) resolved after iron replacement. Iron-deficiency anemia was defined as transferrin saturation (TS)
Source: Blood Cells, Molecules, and Diseases - April 8, 2019 Category: Hematology Source Type: research

Protective effect of the antioxidative peptide SS31 on ionizing radiation-induced hematopoietic system damage in mice
Publication date: Available online 7 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Xiaoliang Han, Ping Gao, Ying Zhang, Jinyan Wang, Fengtao Sun, Qingguo Liu, Shubo ZhangAbstractIonizing radiation (IR) causes severe damage to the hematopoietic system; thus, it is necessary to explore agents or compounds that can reduce this damage. SS31 is a mitochondria-targeted peptide that can scavenge cellular reactive oxygen species (ROS) and inhibit the production of mitochondrial ROS. Therefore, in this study, we discuss the protective effect of SS31 on IR-induced hematopoietic system damage. Our results showed th...
Source: Blood Cells, Molecules, and Diseases - April 8, 2019 Category: Hematology Source Type: research

Decitabine improves overall survival in myelodysplastic syndromes-RAEB patients aged ≥60 years and has lower toxicities: Comparison with low-dose chemotherapy
In this study, we retrospectively assessed the efficacy and toxicity of the two regimens for MDS-refractory anemia with excess blasts (MDS-RAEB) patients. A total of 112 patients with a diagnosis of MDS-RAEB are included. The overall response (OR) and complete remission (CR) rate was comparable between the two groups (OR: 64.1% vs. 66.7%, p = 0.60; CR: 23.4% vs. 31.3%, p = 0.64). The OR rates of 20 mg/m2/day and 15 mg/m2/day decitabine regimen were comparable (69.0% vs. 60.0%, p = 0.46). Overall survival (OS) did not differ significantly between the groups (20.7 vs. 13.5 months, p = 0.17). In a subgro...
Source: Blood Cells, Molecules, and Diseases - April 1, 2019 Category: Hematology Source Type: research