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Hematology Reports

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https://www.readbyqxmd.com/read/30542528/transfusion-associated-graft-versus-host-disease-a-concise-review
#1
Palma Manduzio
Transfusion-associated graft-versushost disease (TA-GVHD) represents a rare fatal event observed in immunocompromised patients and immunocompetent individuals. The main clinical features of this transfusion reaction are pancitopenia and multiorgan failure (skin, liver, gut). The possible pathogenesis includes donor T lymphocyte proliferation in blood, their engraftment and host tissue attack. The purpose of this narrative review was analyzing the international guidelines for irradiation of cellular blood components to prevent TA-GVHD...
November 6, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30542527/immunosuppression-associated-posterior-reversible-encephalopathy-syndrome-in-an-acute-leukemia-case
#2
Umit Y Malkan, Gursel Gunes, Haluk Demiroglu, Hakan Goker
Posterior reversible encephalopathy syndrome (PRES) was described in 1996. Herein, we aimed to report an immunosuppression- related PRES case. A 34-year-old woman was diagnosed as t-cell acute lymphoblastic leukemia and allogeneic hematopoietic stem cell transplantation (HSCT) was performed. Cyclosporine was given for GVHD prophylaxis in addition to the other routine medications of HSCT. She was hospitalized for acute renal failure and due to the possible contribution of acute renal failure cyclosporine was stopped...
November 6, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30344988/notch-activation-promotes-glycosyltransferase-expression-in-human-myeloid-leukemia-cells
#3
Shichun Wang, Mai Itoh, Erika Shiratori, Mika Ohtaka, Shuji Tohda
NOTCH signaling diversely regulates the growth of acute myeloid leukemia (AML) cells. It is known that glycosylation of NOTCH receptors modulates NOTCH activation. However, little is known about glycosylation of NOTCH in AML cells. We examined the effects of ligand-induced NOTCH activation on the expression of NOTCHmodifying glycosyltransferases in two AML cell lines, THP-1 and TMD7. The cells were stimulated with recombinant NOTCH ligands JAGGED1 and DELTA1, and subjected to immunoblot analysis to evaluate the expression levels of glycosyltransferases...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30344987/an-adult-case-of-atypical-hemolytic-uremic-syndrome-presented-with-posterior-reversible-encephalopathy-syndrome-successful-response-to-late-onset-eculizumab-treatment
#4
Serife Solmaz Medeni, Sinem Namdaroglu, Tugba Cetintepe, Can Ozlu, Funda Tasli, Zehra Hilal Adibelli, Oktay Bilgir, Erhan Tatar
Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). After the diagnosis of atypical hemolytic uremic syndrome (aHUS), she was treated with plasma exchange (PE) and hemodialysis (HD)...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30344986/essential-thrombocythemia-hemolytic-anemia-and-hepatic-cirrhosis-could-there-be-an-association
#5
Nata Pratama Hardjo Lugito, Andree Kurniawan, Yohanes Chandra Kurniawan, Enny Yacobus, Edwin Halim
Vascular events are the most common clinical complication of essential thrombocythemia, leading to sign and symptoms of this disease. There are various sign and symptoms of essential thrombocythemia, such as thrombosis in artery or vein, and enlarged spleen. Portal hypertension and hepatic cirrhosis could be caused by essential thrombocythemia via intrahepatic thrombus. Anemia in essential thrombocythemia patient treated with hydroxyurea could be the side effect of bone marrow supression and also hydroxyurea induced hemolytic anemia...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30344985/acquired-factor-vii-deficiency-causing-severe-bleeding-disorder-secondary-to-al-amyloidosis-of-the-liver
#6
Anthony L Nguyen, Muhammad Kamal, Ravi Raghavan, Gayathri Nagaraj
A 52 year-old male presented with neck pain after undergoing thyroidectomy for a goiter three weeks prior which was complicated by a neck hematoma requiring evacuation. Computed tomography (CT) scan showed a neck hematoma requiring evacuation and he received desmopressin with cessation of bleeding. Coagulation studies were normal. He returned eighteen months later with severe oral mucosal bleeding after a dental procedure and required transfusions with red blood cells, platelets, and fresh frozen plasma (FFP) in addition to desmopressin, Humate-P, aminocaproic acid, and surgical packing...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30344984/human-hemoglobin-g-makassar-variant-masquerading-as-sickle-cell-anemia
#7
Ahmad Sabry Mohamad, Roszymah Hamzah, Veena Selvaratnam, Subramanian Yegapan, Jameela Sathar
Human hemoglobin of G-Makassar variant has been reported very rarely with Beta Thalassemia. In year 1969 Hb GMakassar was first identified in Makassar, Sulawesi (Celebes), Republic of Indonesia. The disease was first published in 1969 and 33 years later it has been reported at a family of Thailand origin. We report a 45-yearold Malay man who was investigated for anemia and thrombocytopenia then diagnosed with Hb G-Makassar. This finding describes as a new Hemoglobin GMakassar discovered in Malaysia after 14 years diagnosed in Thailand...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283622/the-progression-of-severe-aplastic-anemia-to-hypoplastic-leukemia-in-a-long-term-observation-after-the-administration-of-pegylated-rhumgdf
#8
Maho Ishikawa, Akira Matsuda, Daisuke Okamura, Tomoya Maeda, Nobutaka Kawai, Norio Asou, Masami Bessho
Thrombopoietin (TPO) is a critical regulator of hematopoiesis. We previously reported that a severe aplastic anemia (SAA) who received a short-term administration of pegylated recombinant human megakaryocyte growth and development factor (rHuMGDF). A trilineage hematologic response was induced, however the patient was diagnosed with leukemia after nine years and eight months from administration of rHuMGDF. In recent reports, somatic mutations in myeloid cancer candidate genes were present in one-third of the AA...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283621/myelodysplasia-related-acute-myeloid-leukemia-and-acute-promyelocytic-leukemia-concomitant-occurrence-of-two-molecularly-distinct-diseases
#9
Yenny Alejandra Moreno Vanegas, Abdel-Ghani M Azzouqa, David M Menke, James M Foran, Prakash Vishnu
Concurrent presentation of acute promyelocytic leukemia (APL) with other hematologic diseases in the absence of previous chemotherapy or ionizing radiotherapy treatment is very rare. We present a case of simultaneous occurrence of APL with myelodysplastic syndrome (MDS)-related acute myeloid leukemia (AML). A 43-yearold female presented with 3 month of history fatigue, night sweats, chills and pancytopenia. Bone marrow aspirate and biopsy demonstrated 20% myeloid blasts with dysplastic changes admixed with abnormal promyelocytes...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283620/the-role-of-red-cell-distribution-width-in-the-differential-diagnosis-of-iron-deficiency-anemia-and-non-transfusiondependent-thalassemia-patients
#10
Pokpong Piriyakhuntorn, Adisak Tantiworawit, Thanawat Rattanathammethee, Chatree Chai-Adisaksopha, Ekarat Rattarittamrong, Lalita Norasetthada
This study aims to find the cut-off value and diagnostic accuracy of the use of RDW as initial investigation in enabling the differentiation between IDA and NTDT patients. Patients with microcytic anemia were enrolled in the training set and used to plot a receiving operating characteristics (ROC) curve to obtain the cut-off value of RDW. A second set of patients were included in the validation set and used to analyze the diagnostic accuracy. We recruited 94 IDA and 64 NTDT patients into the training set. The area under the curve of the ROC in the training set was 0...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283619/long-term-complete-remission-of-early-hematological-relapse-after-discontinuation-of-immunosuppressants-following-allogeneic-transplantation-for-sezary-syndrome
#11
Hiroki Hosoi, Kazuo Hatanaka, Shogo Murata, Toshiki Mushino, Kodai Kuriyama, Akinori Nishikawa, Nobuyoshi Hanaoka, Shinobu Tamura, Hideki Nakakuma, Takashi Sonoki
Sezary syndrome (SS) is a leukemic form of cutaneous T-cell lymphoma and is chemo-resistant. Allogeneic hematopoietic stem cell transplantation is a promising therapy for SS; however, relapse is common. Therapeutic options after relapse have not been established. We managed an SS patient with hematological relapse within one month after transplantation. After discontinuation of immunosuppressants, she achieved complete remission and remained relapse-free. The chimeric analyses of Tcells showed that the full recipient type became complete donor chimera after immunological symptoms...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283618/clinical-significance-of-dasatinib-induced-pleural-effusion-in-patients-with-de-novo-chronic-myeloid-leukemia
#12
Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito, Shosaku Nomura
Dasatinib is currently approved for clinical use as a first-line treatment agent for newly diagnosed chronic myeloid leukemia (CML). However, only a few clinical trials have been performed to evaluate dasatinibinduced PE following first-line therapy. We investigated the incidence and clinical features of dasatinib-induced PE following first-line therapy in Japanese CML patients of real world clinical practice settings. Among 22 patients, the median age of PE-positive patients was higher than that of PE-negative patients...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30283617/acquired-hemophilia-a-developing-cerebral-infarction-36-days-after-the-frequent-administration-of-bypass-hemostatic-agents
#13
Makoto Saito, Hajime Senjo, Minoru Kanaya, Koh Izumiyama, Akio Mori, Masanori Tanaka, Masanobu Morioka, Masahiro Ieko
A 74-years-old male who was a smoker and received treatment for hypertension, dyslipidemia, peripheral arterial disease and idiopathic interstitial pneumonia complained of subcutaneous hemorrhage of the right lower thigh. Marked anemia (hemoglobin 5.5 g/dL) and prolonged activated partial thromboplastin time (≥130 seconds) were noted. The factor VIII activity level was reduced to 1.2%, and the factor VIII inhibitor titer was 285.3 BU/mL, a diagnosis of acquired hemophilia A (AHA) was made. Then, hematomas of 5 intra-muscles were recurred...
September 5, 2018: Hematology Reports
https://www.readbyqxmd.com/read/30046415/impact-of-voxelotor-gbt440-on-unconjugated-bilirubin-and-jaundice-in-sickle-cell-disease
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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