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American Journal of Blood Research

Borhane Slama, Olivier Fain, Hervé Maisonneuve, Eric Jourdan, Jean-François Viallard, Rabye Ouaja, Ousmane Alfa-Cissé, Bertrand Godeau
[This corrects the article on p. 1 in vol. 7, PMID: 28203488.].
2017: American Journal of Blood Research
Niaz Hussain Jamali, Abdul Hakeem Jamali, Aftab Ahmed Khand, Hidayatullah Mahesar, Muhammad Iqbal Arain
Ferritin is a protein found in reticuloendothelial system and does not work only as a mid-size protein during Hemoglobin synthesis but also as storage protein for iron delivery. This cross sectional study was conducted in District Shaheed Benazirabad during the period of August 2015 to March 2016, for obtaining credible and consolidated data. In present study we investigated BMI, Hb and S.f level with relation to socioeconomic status in children and adults from total 2040 volunteers (girls n=991, boys n=1049)...
2017: American Journal of Blood Research
Ahmad T Mansour, Alaleh Esmaeili Shandiz, Michelle K Zimmerman, Trenton D Roth, Jiehao Zhou
BACKGROUND: Lymphoplasmacytic lymphoma and plasma cell myeloma are two B cell lymphoproliferative neoplasms derived from mature B-lymphocytes in different differentiation stages. The coexistence of these two tumors in the same patient is exceedingly rare and can be difficult to diagnose. CASE PRESENTATION: A 76-year-old male presented with a pathologic fracture after a fall. Radiography showed a lytic lesion in the pelvis. Serum immunofixation showed distinct IgM kappa and IgA kappa monoclonal protein bands...
2017: American Journal of Blood Research
Borhane Slama, Olivier Fain, Hervé Maisonneuve, Eric Jourdan, Jean-François Viallard, Rabye Ouaja, Ousmane Alfa-Cissé, Bertrand Godeau
PURPOSE: The present study was designed to assess the efficacy and safety of IGNG that is a new liquid, saccharose and maltose-free highly purified ready-to-use 5% intravenous immunoglobulin (IVIg), in primary immune thrombocytopenic patients with severe thrombocytopenia. METHODS: Nineteen adults with a platelet count ≤ 25 × 10(9)/L received a single dose of IGNG (1 g/kg) on Day 1, with a second identical dose on Day 3 if needed. Patients were followed for 30 days...
2017: American Journal of Blood Research
Fabian Mück, Silvia Bracharz, Rolf Marschalek
AF4/AFF1 and AF5/AFF4 are both backbones for the assembly of "super elongation complexes" (SECs) that exert 2 distinct functions after the recruitment of P-TEFb from the 7SK snRNP: (1) initiation and elongation of RNA polymerase II gene transcription, and (2) modification of transcribed gene regions by distinct histone methylation patterns. In this study we aimed to investigate one of the initial steps, namely how P-TEFb is transferred from 7SK snRNPs to the SECs. In particular, we were interested in the role of DDX6 that we have recently identified as part of the AF4 complex...
2016: American Journal of Blood Research
Fahri Sahin, Olga Meltem Akay, Mesut Ayer, Mehmet Sinan Dal, Sehmus Ertop, Osman Ilhan, Volkan Karakus, Mehmet Ali Ozcan, Vildan Ozkocaman, Hayri Ozsan, Ozan Salim, Mahmut Tobu, Anil Tombak, Tulin Firatli Tuglular, Mehmet Yilmaz, Ali Unal, Mustafa Nuri Yenerel, Guray Saydam
PNH Education and Study Group (PESG) have been established in December 2013 as a non-profit, independent, medical organization Paroxysmal Nocturnal Hemoglobinuria (PNH) is a multi-systemic disease that should be treated with a multidisciplinary approach. Patients may apply to the clinics other than the hematology due to variability and diversity of clinical findings which lower the rate of diagnosis due to low awareness about PNH. PNH might be overlooked and diagnosis might be delayed. Regarding these, PESG was established with the collaboration of Immunology, Cardiology, Thorax Diseases (Pulmonology), Neurology, Gastroenterology, General Surgery and Urology specialists in addition to hematologists dealing with PNH...
2016: American Journal of Blood Research
Brittany A Nierste, Ellen J Gunn, Kathleen R Whiteman, Robert J Lutz, Julia Kirshner
Environmental-mediated drug-resistance (EM-DR) presents a major challenge for therapeutic development. Tissue microenvironment in the form of extracellular matrix, soluble factors, and stroma contribute to EM-DR. In multiple myeloma (MM), drug-resistance has hindered treatment success with 5-year survival rates remaining <50%. Here we evaluated IMGN901, a maytansinoid immunoconjugate, for its ability to overcome EM-DR alone or in combination with lenalidomide or dexamethasone. We show that while adhesion of MM cells to the extracellular matrix reduces potency of IMGN901, it remains cytotoxic with an average LC50=43 nM...
2016: American Journal of Blood Research
Wederson M Claudino, Bradley Gibson, William Tse, Maxwell Krem, Jaspreet Grewal
Methotrexate (MTX) is a commonly used anti-metabolite agent. Increased risk of lymphoproliferative disorders (LPD) in patients with rheumatoid arthritis (RA) has been documented with the prolonged use of immunosuppressive medications such as MTX. This is thought to be the result of immune dysregulation and/or chronic immune stimulation. Most cases of LPDs regress following withdrawal of the offending immunosuppressive agent. We present an interesting and rare case of CD30 and EBV positive CD8 primary cutaneous anaplastic large cell lymphoma (PC-ALCL) in a 66-year-old African American woman...
2016: American Journal of Blood Research
Lolowa A Al Mekaini, Srdjan Denic, Omar N Al Jabri, Hassib Narchi, Abdul-Kader Souid, Suleiman Al-Hammadi
α+-Thalassemia trait and iron deficiency anemia are frequent causes of microcytosis and a common diagnostic challenge in Arabian children. In this study, their prevalences and effects on the red cell parameters were evaluated in 28,457 children aged one day to 6 years. α+-Thalassemia trait was considered to be present when mean cell volume (MCV) was <94 fL at birth and iron deficiency anemia when red cell distribution width (RDW) was >14.5%. The prevalence of α+-thalassemia trait was 15.7% (502/3,191), which was similar to previously reported values for adults (9-14%)...
2015: American Journal of Blood Research
Melda Comert Ozkan, Murat Tombuloglu, Fahri Sahin, Guray Saydam
OBJECTIVE: Multiple myeloma (MM) comprises 1% of all cancers and 10% of hematologic malignancies and known as an incurable disease. The introduction of immunomodulatory drugs (IMiDs) has brought a major shift in therapeutic paradigm in the treatment of newly diagnosed and relapsed/refractory MM patients. The aim of this study was to evaluate the relationship between response status and hematological parameters in patients with MM treated with thalidomide or lenalidomide. METHODS: Sixty-eight patients who were treated with IMiDs in Ege University, School of Medicine, Department of Hematology, between 2005 and 2012, were evaluated, retrospectively...
2015: American Journal of Blood Research
Masahiro Manabe, Katsuya Wada, Dai Momose, Yasuyoshi Sugano, Masayuki Hino, Takahisa Yamane, Eiwa Ishida, Ki-Ryang Koh
We present a case of therapy-related myelodyspastic syndrome in which the t(3;8)(q26;q24) translocation appeared, even though no chromosomal abnormalities were found at the initial diagnosis of acute myeloid leukemia. To the best of our knowledge, there have only been around 20 reported cases of myeloid malignancies involving t(3;8)(q26;q24). We discuss the characteristics of t(3;8)(q26;q24) along with a review of literature.
2015: American Journal of Blood Research
Zaher K Otrock, Nabil Chamseddine, Ziad M Salem, Tarek Wehbe, Mouna Al-Ayoubi, Moussa Dhaini, Joseph Kattan, Walid Mokaddem, Therese Abi Nasr, Oussama Jradi, Fadi S Farhat, Mahmoud Wehbe, Mohammad H Haidar, Mohamed A Kharfan-Dabaja, Nizar Bitar, Mirna El Hajj, Adel M Kadri, Francois G Kamar, Hanan Yassine, Hassan Khodr, Ali T Taher, Noha Hakime, Rami Ar Mahfouz, Wassim Serhal, Ali Bazarbachi, Hussein Z Farhat
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic disorders characterized by peripheral blood cytopenias, blood cells dysplasia, and increased risk for progression to acute leukemia.Physicians should be vigilant in diagnosing MDS and should be aware of the contemporary therapies that are always in progress. Most of the data on MDS epidemiology and management comes from developed countries. The incidence and features of MDS in the Arab countries, among them Lebanon, are not known...
2015: American Journal of Blood Research
Pritesh Patel, Dolores Mahmud, Youngmin Park, Kazumi Yoshinaga, Nadim Mahmud, Damiano Rondelli
Clinical isolation of circulating CD4(+)CD25(+) regulatory T cells (Tregs) from peripheral blood mononuclear cells is usually performed by CD4(+) cell negative selection followed by CD25(+) cell positive selection. Although G-CSF mobilized peripheral blood (G-PBSC) contains a high number of Tregs, a high number of monocytes in G-PBSC limits Treg isolation. Using a small scale device (MidiMACS, Miltenyi) we initially demonstrated that an initial depletion of monocytes would be necessary to obtaina separation of CD4(+)CD25(+)FoxP3(+)CD127(-) cells from G-PBSC (G-Tregs) with a consistent purity >70% and inhibitory activity of T cell alloreactivity in-vitro...
2015: American Journal of Blood Research
Yuan Tang, Jie Chen, Jianchao Wang, Ke Zheng, Dianying Liao, Xiaomei Liao, Weiping Liu, Lin Wang
Evaluation of immunoglobulin (Ig) gene rearrangements with BIOMED-2 multiplex PCR has become a standard detection of clonality in mature B cell malignancies. Conventionally, this method is relatively labor-intensive and time-consuming, as it requires DNA isolation from bone marrow aspirates (BM) or peripheral blood (PB) in patients with BM or PB involvement. On the other hand, fluorescence in situ hybridization (FISH) is routinely used as genetic screening in B cell malignancies, but the surplus fixed nuclei initially prepared for FISH usually turn useless afterwards...
2015: American Journal of Blood Research
Maria Zingariello, Alessandra Ruggeri, Fabrizio Martelli, Manuela Marra, Laura Sancillo, Ilaria Ceglia, Rosa Alba Rana, Anna Rita Migliaccio
Despite numerous circumstantial evidences, the pathogenic role of TGF-β in primary myelofibrosis (PMF), the most severe of the Philadelphia-negative myeloproliferative neoplasms, is still unclear because of the modest (2-fold) increases in its plasma levels observed in PMF patients and in the Gata1(low) mouse model. Whether myelofibrosis is associated with increased bioavailability of TGF-β bound to fibrotic fibres is unknown. Transmission electron-microscopy (TEM) observations identified that spleen from PMF patients and Gata1(low) mice contained megakaryocytes with abnormally high levels of TGF-β and collagen fibres embedded in their cytoplasm...
2015: American Journal of Blood Research
Fahri Sahin, Asu F Yilmaz, Melda Comert Ozkan, Nihal Mete Gokmen, Guray Saydam
Paroxysmal nocturnal hemoglobinuria (PNH) is a disease characterized by chronic persistent hemolysis, multi-organ damage and eventually multiple organ failure. PNH develops as a result of increased sensitivity to complement due to an acquired deficiency of certain glycosylphosphatidylinositol (GPI)-linked proteins. The clinical presentation of PNH varies greatly from one patient to another. We present three cases of PNH with different clinical presentations to illustrate the debilitating nature of the disease, possible fatal outcomes, and the need to timely diagnosis and targeted therapy...
2015: American Journal of Blood Research
Wederson M Claudino, Ajoy Dias, William Tse, Vivek R Sharma
Major strides have been made in improving the treatment of medical emergencies associated with malignancies. Nonetheless, metabolic emergencies in cancer patients can often times be life-threatening. Type B lactic acidosis is a rare but potentially fatal paraneoplastic phenomenon that has been described in association with hematologic and solid malignancies and portends a poor prognosis if not rapidly recognized and treated. It is believed that this occurs as a result of cancer cells switching their glucose metabolism from an oxidative oxygen- dependent pathway towards a glycolytic phenotype, also known as the "Warburg effect"...
2015: American Journal of Blood Research
Bastian Scholz, Eric Kowarz, Tanja Rössler, Khalil Ahmad, Dieter Steinhilber, Rolf Marschalek
AF4/AFF1 and AF5/AFF4 are the molecular backbone to assemble "super-elongation complexes" (SECs) that have two main functions: (1) control of transcriptional elongation by recruiting the positive transcription elongation factor b (P-TEFb = CyclinT1/CDK9) that is usually stored in inhibitory 7SK RNPs; (2) binding of different histone methyltransferases, like DOT1L, NSD1 and CARM1. This way, transcribed genes obtain specific histone signatures (e.g. H3K79me2/3, H3K36me2) to generate a transcriptional memory system...
2015: American Journal of Blood Research
Fahri Sahin, Melda Comert Ozkan, Nihal Gokmen Mete, Mumtaz Yilmaz, Nevin Oruc, Alev Gurgun, Meral Kayikcioglu, Ayse Guler, Figen Gokcay, Ferda Bilgir, Cengiz Ceylan, Oktay Bilgir, Ismail Hakan Sari, Guray Saydam
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease caused by clonal expansion of one or more hematopoietic stem cell (HSC) lines due to a somatic mutation of the phosphatidylinositol glycan anchor (PIG-A) gene located on Xp22.1. PNH incidence is 1.5-2 cases per million of the population per year. PNH can affect multiple systems in the body and requires multidisciplinary clinical management. Patients can manifest with severe pancytopenia, life-threatening thrombosis affecting the hepatic, abdominal, cerebral, and subdermal veins, and high requirements for blood transfusion due to haemolytic anemia...
2015: American Journal of Blood Research
Kosuke Obama
Concomitant chemoradiotherapy is established as the standard treatment to improve the prognosis of several types of solid tumor, but has not been the general practice for hematological malignancies. Here, I report two cases of adult T-cell leukemia (ATL) with a radiotherapy-resistant bulky disease treated with concomitant radiotherapy and two topoisomerase inhibitors: etoposide (VP-16) and irinotecan (CPT-11). Patient 1 was a 78-year-old man with chemotherapy-resistant inguinal bulky mass. Radiotherapy (total 40 Gy) for this inguinal lesion was started; however, the bulky disease was found to be resistant to radiotherapy and progressed...
2014: American Journal of Blood Research
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