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Hematology Reports

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https://www.readbyqxmd.com/read/30046415/impact-of-voxelotor-gbt440-on-unconjugated-bilirubin-and-jaundice-in-sickle-cell-disease
#1
Paul Telfer, Irene Agodoa, Kathleen M Fox, Laurie Burke, Timothy Mant, Marzena Jurek, Margaret Tonda, Josh Lehrer-Graiwer
For many patients with sickle cell disease (SCD), jaundice is a significant clinical disease manifestation that impacts on patient well-being. We report a case of a patient with SCD and chronic jaundice treated with voxelotor (GBT440), a novel small molecule hemoglobin oxygen affinity modulator and potential disease-modifying therapy for SCD. The case patient is a 27-year-old Black male with a long history of SCD with clinical jaundice and scleral icterus. After starting voxelotor, the patient reported that his jaundice cleared within one week, and that he felt much better with more energy, and was relieved after his eyes cleared...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046414/generalized-lymphadenopathy-secondary-to-isolated-extramedullary-hematopoiesis-as-an-initial-manifestation-of-primary-myelofibrosis
#2
Mansour S Aljabry, Shuaa Asiri, Tayseer Elsafi, Ghaleb Elyamany
Extramedullary hematopoiesis (EMH) is a presence of hematopoietic activity in the extramedullary sites. EMH can occur in both benign and malignant hematologic diseases. The liver and spleen are the most common sites, but may also occur infrequently at other sites. EMH often occurs in more than one site and quite rare in an isolated organ. In this study we describe an unusual case of generalized lymphadenopathy secondary to isolated extramedullary hematopoiesis as an initial manifestation of primary myelofibrosis...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046413/the-shortcut-strategy-for-beta-thalassemia-prevention
#3
Narutchala Suwannakhon, Khajohnsilp Pongsawatkul, Teerapat Seeratanachot, Khwanruedee Mahingsa, Arunee Pingyod, Wanwipa Bumrungpakdee, Torpong Sanguansermsri
We propose antenatal blood tests using high-resolution DNA melting (HRM) analysis for beta thalassemia mutation detection after hemoglobin A2 estimation as a modified strategy for the identification of beta thalassemia at-risk couples. Antenatal blood samples of 1,115 couples were transferred from the antenatal care clinic. Hemoglobin A2 was quantified, and proportions ≥3.5% were further assessed for beta thalassemia mutation using HRM analysis. Twelve types of beta thalassemia mutations, including hemoglobin E, were identified...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046412/blastic-epstein-barr-virus-associated-post-transplant-lymphoproliferative-disorder-after-allogeneic-stem-cell-transplantation-for-severe-aplastic-anemia
#4
Masaaki Hotta, Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Akiko Konishi, Hideaki Yoshimura, Tomoki Ito, Kazuyoshi Ishii, Shosaku Nomura
Post-transplant lymphoproliferative disorder (PTLD) is a well-recognized complication of organ transplantation. Progress has recently been made in the pathological classification of PTLD. However, the clinical course has not been clarified because of the rarity of this disease. We experienced a case of PTLD with a fulminant clinical course. The patient had been under longterm immunosuppressive treatment for aplastic anemia. He received related allogeneic hematopoietic stem cell transplantation. Soon after transplantation, he developed PTLD...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046411/gbt440-reverses-sickling-of-sickled-red-blood-cells-under-hypoxic-conditions-in-vitro
#5
Kobina Dufu, Donna Oksenberg
Sickle cell disease is characterized by hemolytic anemia, vasoocclusion and early mortality. Polymerization of hemoglobin S followed by red blood cell sickling and subsequent vascular injury are key events in the pathogenesis of sickle cell disease. Sickled red blood cells are major contributors to the abnormal blood rheology, poor microvascular blood flow and endothelial injury in sickle cell disease. Therefore, an agent that can prevent and or reverse sickling of red blood cells, may provide therapeutic benefit for the treatment of sickle cell disease...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046410/myeloid-sarcoma-without-circulating-leukemia-mimicking-gastrointestinal-malignancy-and-lymphoma
#6
Sravanthi Ravulapati, Craig Siegel, Ameesh Dara, Jack M Lionberger
We present an unusual case of myeloid sarcoma with ascites and abdominal pain in which initial clinical, laboratory, and imaging studies suggested a gastrointestinal malignancy or lymphoma. Subsequent detection of leukemic ascites and blasts in a gastric, small bowel, and skin biopsy supported a diagnosis of myeloid sarcoma. Bone marrow biopsy revealed 15% blasts, and cytogenetics with an inversion 16 rearrangement was diagnostic of acute myeloid leukemia (AML). Positron emission tomography-computed tomography performed at presentation to stage a presumptive lymphoma found later utility in following the burden of extramedullary disease...
May 14, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721255/paroxysmal-nocturnal-hemoglobinuria-when-delay-in-diagnosis-and-long-therapy-occurs
#7
Salvatrice Mancuso, Giuseppe Sucato, Melania Carlisi, Marco Santoro, Giuseppe Tarantino, Emilio Iannitto, Mariasanta Napolitano, Sergio Siragusa
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by hemolytic anemia, bone marrow failure and thrombosis, caused by a somatic mutation in PIG-A gene that results in the absence of CD55 and CD59, two important complement regulatory proteins. In this paper, a case of PNH is retrospectively examined looking for clinical and laboratory features, and the entire course of the disease from the onset of the symptoms is described, together with an adequate follow- up over a 7-years treatment period...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721254/chromosome-positioning-in-interphase-nuclei-of-hematopoietic-stem-cell-and-myeloid-precursor
#8
Mariana Lomiento, Fabiana Mammoli, Emilia Maria Cristina Mazza, Silvio Bicciato, Sergio Ferrari
Human myelopoiesis is an intriguing biological process during which multipotent stem cells limit their differentiation potential generating precursors that evolve into terminally differentiated cells. The differentiation process is correlated with differential gene expression and changes in nuclear architecture. In interphase, chromosomes are distinct entities known as chromosome territories and they show a radial localization that could result in a constrain of inter-homologous distance. This element plays a role in genome stability and gene expression...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721253/neurofibromatosis-type-i-and-multiple-myeloma-coexistence-a-possible-link
#9
Fabrizio Accardi, Valentina Marchica, Cristina Mancini, Elena Maredi, Costantina Racano, Laura Notarfranchi, Davide Martorana, Paola Storti, Eugenia Martella, Benedetta Dalla Palma, Luisa Craviotto, Massimo De Filippo, Antonio Percesepe, Franco Aversa, Nicola Giuliani
The association between Neurofibromatosis type I (NF1) and multiple myeloma (MM), a plasma cell, dyscrasia is very rare. Here we put to the attention of the scientific community two new cases. The first one is a patient with active MM whereas the second with smoldering MM. Both patients present typical features of NF1 but skeletal alterations were present only in the second case including dysplasia, marked scoliosis and osteoporosis. MM osteolytic lesions were absent in both patients. In addition to the clinical diagnosis of NF1, a molecular testing for NF1 gene mutations has been performed finding that patient one was heterozygous for the c...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721252/acute-monocytic-leukemia-diagnosed-by-flow-cytometry-includes-acute-myeloid-leukemias-with-weakly-or-faintly-positive-non-specific-esterase-staining
#10
Yuriko Zushi, Miho Sasaki, Ayano Mori, Toshiharu Saitoh, Takae Goka, Yumi Aoyama, Yuta Goto, Hiroko Tsunemine, Taiichi Kodaka, Takayuki Takahashi
A diagnosis of acute monocytic leukemia (AML-M5) based on α-naphthyl butyrate esterase (α-NB) staining has some problems, because AML-M5 leukemic cells often show weak or faint positivity on α-NB staining. In these situations, some cases of AML-M5 tend to be misdiagnosed as AML-M0. Therefore, we evaluated the significance of weak or faint α-NB staining in AML-M5 diagnosed by flow cytometry (FCM). Nineteen AML cases in which leukemic cells were negative for naphthol AS-D chloroacetate esterase staining were studied...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721251/bone-targeted-agents-in-multiple-myeloma
#11
Hiroko Nishida
Osteolytic bone disease, characterized by bone pain, increased risk of pathologic fractures, tumor-induced hypercalcemia known as skeletal-related events (SREs), is a frequent complication of patients with multiple myeloma (MM) and persists even in the absence of active disease, resulting in a major cause of morbidity and mortality. The interaction between myeloma cells and their surrounding cells in the bone marrow (BM) microenvironment promotes both myeloma cell growth and bone destruction and forms the vicious cycle of MM bone disease...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721250/congenital-methemoglobinemia-misdiagnosed-as-polycythemia-vera-case-report-and-review-of-literature
#12
Dina Sameh Soliman, Mohamed Yassin
Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29721249/extranodal-diffuse-large-b-cell-lymphomas-a-retrospective-case-series-and-review-of-the-literature
#13
Stergios Boussios, Ioannis Zerdes, Amalia Vassou, Eleni Bareta, Esmeralda Seraj, Alexandra Papoudou-Bai, Nicholas Pavlidis, Anna Batistatou, George Pentheroudakis
Non-Hodgkin lymphomas commonly show extranodal involvement (25-30%) but primary diffuse large B-cell lymphomas (DLBCL) with extranodal localization represent clinically and molecularly distinct entities. The present study involved retrospective analysis of case records of 4 patients who were diagnosed with extranodal DLBCL between 2010 and 2016 at the Medical Oncology and Hematology Departments of the Ioannina University Hospital, Greece. Median age of presentation was 69 years (range 60-77 years). There were 2 males and 2 females...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29383226/double-heterozygocity-for-hemoglobin-c-and-beta-thalassemia-dominant-a-rare-case-of-thalassemia-intermedia
#14
Alexandra Agapidou, Paul King, Cecilia Ng, Dimitris A Tsitsikas
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29383225/a-rare-case-of-de-novo-cd5-diffuse-large-b-cell-lymphoma-in-leukemic-phase-and-positive-for-cd13
#15
Giovanni Carulli, Eugenio Mario Ciancia, Francesco Caracciolo, Paola Sammuri, Cristiana Domenichini, Maria Immacolata Ferreri, Alessia Di Vita, Virginia Ottaviano, Martina Rousseau, Mario Petrini
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29383224/haploidentical-hematopoietic-stem-cell-transplantation-in-a-myelofibrosis-patient-with-primary-graft-failure
#16
Cristina Tecchio, Angelo Andreini, Claudio Costantini, Alberto Zamò, Donata de Sabata, Fiorenza Aprili, Roberta Galavotti, Emanuele Guardalben, Fabio Benedetti
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29333227/dasatinib-induced-hemorrhagic-colitis-complicated-with-cytomegalovirus-infection
#17
Aya Nakaya, Yoshiko Azuma, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yukie Tsubokura, Akiko Konishi, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito, Shosaku Nomura
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29333226/expression-of-some-atp-binding-cassette-transporters-in-acute-myeloid-leukemia
#18
Antonella Maria Salvia, Flavia Cuviello, Sabrina Coluzzi, Roberta Nuccorini, Immacolata Attolico, Sara Pasquina Pascale, Faustino Bisaccia, Michele Pizzuti, Angela Ostuni
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29333225/a-rare-cause-of-pericardial-effusion-and-ascites-poems-syndrome
#19
Bilal Katipoglu, Zeynep Katipoğlu, Ihsan Ates, Gokhan Yirgin, Burak Furkan Demir, Fatih Acehan
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29333224/prdi-bf1-and-prdi-bf1p-isoform-expressions-correlate-with-disease-status-in-multiple-myeloma-patients
#20
Gabriele Buda, Francesca Guerrini, Sara Galimberti, Enrico Orciuolo, Simone Pacini, Elisa Mazzantini, Mario Petrini
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
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