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Sabrina Giammarco, Patrizia Chiusolo, Nicola Piccirillo, Alessia Di Giovanni, Elisabetta Metafuni, Luca Laurenti, Simona Sica, Livio Pagano
Hyperleukocytosis is defined as a white blood cell count greater than 100,000/mL in patients affected by acute leukemia and often it is associated with increased morbidity and mortality, that can be up to 40% if unrecognized. Areas covered: Risk factors include younger age, myelomonocytic or monocytic/monoblastic morphology, microgranular variant of acute promyelocitic leukemia and T-cell ALL, and some cytogenetic abnormalities. Poor prognosis due to high early death rate secondary to leukostasis. The mechanisms at the origin of leukostasis are still poorly understood...
December 26, 2016: Expert Review of Hematology
S A Kocheva, K Martinova, Z Antevska-Trajkova, B Coneska-Jovanova, A Eftimov, A J Dimovski
Nijmegen breakage syndrome (NBS) is a rare autosomal recessive chromosomal instability disorder characterized by microcephaly, immunodeficiency, radiosensitivity and a very high predisposition to malignancy. The gene responsible for the disease, NBS1, is located on chromosome 8q21 and encodes a protein called nibrin. After identification of the gene, a truncating 5 bp deletion, 657-661delACAAA, was identified as the disease-causing mutation in patients with the NBS. In this report, we describe two patients with NBS and T-lymphoblastic leukemia/lymphoma in a Macedonian family...
July 1, 2016: Balkan Journal of Medical Genetics: BJMG
Thomas Grellety, Sophie Cousin, Louis Letinier, Pauline Bosco-Lévy, Stéphanie Hoppe, Damien Joly, Nicolas Penel, Simone Mathoulin-Pelissier, Antoine Italiano
BACKGROUND: Optimizing patient selection is a necessary step to design better clinical trials. 'Life expectancy' is a frequent inclusion criterion in phase II trial protocols, a measure that is subjective and often difficult to estimate. The aim of this study was to identify factors associated with early death in patients included in phase II studies. METHODS: We retrospectively collected medical records of patients with advanced solid tumors included in phase II trials in two French Comprehensive Cancer Centers (Bordeaux, Center 1 set; Lille, Center 2 set)...
October 4, 2016: BMC Cancer
Shyam A Patel
Acute myeloid leukemia (AML) involvement of the central nervous system is relatively rare, and detection of leptomeningeal disease typically occurs only after a patient presents with neurological symptoms. The case herein describes a 48-year-old man with relapsed/refractory AML of the mixed lineage leukemia rearrangement subtype, who presents with monocular vision loss due to leukemic eye infiltration. MRI revealed right optic nerve sheath enhancement and restricted diffusion concerning for nerve ischemia and infarct from hypercellularity...
2016: Case Reports in Hematology
Miguel Pinto da Costa, Henrique Pimenta Coelho
The authors present a case of a 60-year-old male patient, previously diagnosed with B-cell chronic lymphocytic leukemia, who was admitted to the Emergency Room with dyspnea. The initial evaluation revealed severe anemia (Hgb = 5.0 g/dL) with hyperleukocytosis (800.000/µL), nearly all of the cells being mature lymphocytes, a normal chest X-ray and a low arterial oxygen saturation (89%; pulse oximetry). After red blood cell transfusion, Hgb values rose (9.0 g/dL) and there was a complete reversion of the dyspnea...
May 2016: Acta Médica Portuguesa
(no author information available yet)
No abstract text is available yet for this article.
July 2016: MLO: Medical Laboratory Observer
Hajime Maeda, Hayato Go, Takashi Imamura, Maki Sato, Nobuo Momoi, Mitsuaki Hosoya
Infants with Down syndrome (DS) are at risk of developing a transient myeloproliferative disorder during the neonatal period, known as transient abnormal myelopoiesis (TAM). It is characterized by clonal myeloproliferation and is typically self-limiting. However, TAM can be a life-threatening disorder, when complicated by liver fibrosis. Here, we evaluated cytokine profiles in two male DS infants having TAM with or without liver dysfunction. The first patient, Patient 1, had hyperleukocytosis with cholestatic liver dysfunction, coagulopathy, and increased counts of blasts and was treated with exchange transfusion (ExT) due to the serious general condition...
2016: Tohoku Journal of Experimental Medicine
Mariam Doumbia, Jean Uwingabiye, Aboubacar Bissan, Razine Rachid, Souad Benkirane, Azlarab Masrar
The aim of this study was to describe epidemiological, cytologic and immunophenotypic aspects of acute leukemias (AL) in children diagnosed at IBN SINA University Hospital Center and to determine the concordance between cytology and immunophenotyping results. This is a cross-sectional study conducted in the hematology laboratory of IBN SINA University Hospital Center between June 2012 and May 2014. Among the 104 cases with diagnosed AL, 52% were boys with a sex-ratio H/F= 1.32, the average age was 5.7 years...
2016: Pan African Medical Journal
Chrystèle Bilhou-Nabéra, Audrey Bidet, Virginie Eclache, Eric Lippert, Marie-Joëlle Mozziconacci
The recent years have witnessed tremendous progress in the molecular characterization of Philadelphia-negative myeloproliferative neoplasms (MPN). Beside a better understanding of pathophysiology, these abnormalities often constitute very useful diagnostic markers in diseases where exclusion of reactive states used to be the strongest argument. However, conventional and molecular cytogenetics keep a major interest in MPN, either as a second line exploration, in cases where no molecular marker is available, for differential diagnosis or as a proof of clonality or in first line for cases with hyperleukocytosis, for differential diagnosis (CML), to evidence druggable targets (ABL1, RET, PDGFR…) or as a prognosis marker...
October 1, 2016: Annales de Biologie Clinique
Vipin Villgran, Mounzer Agha, Anastasios Raptis, Jing-Zhou Hou, Rafic Farah, Seah H Lim, Robert L Redner, Annie Im, Alison Sehgal, Kathleen A Dorritie, Joseph E Kiss, Daniel Normolle, Michael Boyiadzis
Hyperleukocytosis is present in 5 to 20 percent of patients with newly diagnosed acute myeloid leukemia (AML). The management of hyperleukocytosis, when symptoms of leukostasis occur, includes intensive supportive care and interventions for rapid cytoreduction. Leukapheresis is a rapid and effective means of cytoreduction and has been used in AML patients. In the current study, we evaluated the outcomes of 68 newly diagnosed AML patients that underwent leukapheresis and the effects of leukapheresis on various laboratory parameters...
October 2016: Transfusion and Apheresis Science
Kunimoto Ichikawa, Yoko Edahiro, Akihiko Gotoh, Kazuhide Iiduka, Norio Komatsu, Michiaki Koike
Early intracranial hemorrhage (eICH) is a potentially fatal complication of acute leukemia. We analyzed risk factors for eICH in patients with de novo acute leukemia. Ninety-one de novo acute leukemia patients at our institution between September 2003 and June 2014 were included. Of the 91 patients, eight (8.8 %) and 83 were included in the eICH and non-eICH groups, respectively. Univariate analysis demonstrated that white blood cell (WBC) count (P = 0.018), fibrin-fibrinogen degradation product (FDP) level (P = 0...
November 2016: International Journal of Hematology
Weerapat Owattanapanich, Chirayu U Auewarakul
BACKGROUND: Intracranial hemorrhage (ICH) is an uncommon complication in patients with hematologic disorders although high fatality rates have been shown in these patients. At present, no epidemiological data regarding ICH in patients with hematologic disorders has been collected and/or reported in Thailand. OBJECTIVE: The purpose of this study was to determine the incidence of ICH in hospitalized patients with hematologic disorders and to identify predictive factors associated with ICH in these patients...
January 2016: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
Rosa Nguyen, Sima Jeha, Yinmei Zhou, Xueyuan Cao, Cheng Cheng, Deepa Bhojwani, Patrick Campbell, Scott C Howard, Jeffrey Rubnitz, Raul C Ribeiro, John T Sandlund, Tanja Gruber, Hiroto Inaba, Ching-Hon Pui, Monika L Metzger
BACKGROUND: Hyperleukocytosis in children with acute lymphoblastic leukemia (ALL) has been associated with early morbidity and mortality. The use of leukapheresis in these children treated with contemporary therapy remains controversial. PROCEDURE: We analyzed clinical data from patients enrolled onto frontline protocols for ALL (Total Therapy XV and XVI) between 2003 and 2014. We documented adverse events within the first 14 days in patients with a white blood cell (WBC) count ≥200 × 10(9) /l and reviewed their management...
September 2016: Pediatric Blood & Cancer
Sherif M Badawy
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Hematology/oncology
Takeo Sarashina, Haruko Iwabuchi, Naoyuki Miyagawa, Masahiro Sekimizu, Tomoko Yokosuka, Kunio Fukuda, Satoshi Hamanoue, Fuminori Iwasaki, Shoko Goto, Masae Shiomi, Chihaya Imai, Hiroaki Goto
Mature B-cell acute lymphoblastic leukemia (B-ALL) is typically associated with French-American-British (FAB)-L3 morphology and MYC gene rearrangement. However, rare cases of mature B-ALL with non-L3 morphology and MLL-AF9 fusion have been reported, and such cases are characterized by a rapid and aggressive clinical course. We here report three such cases of pediatric mature B-ALL in female patients respectively aged 15 months, 4 years, and 4 months. Bone marrow smears at diagnosis showed FAB-L1 morphology in all patients...
July 2016: International Journal of Hematology
A Chaturvedi, M M Araujo Cruz, N Jyotsana, A Sharma, R Goparaju, A Schwarzer, K Görlich, R Schottmann, E A Struys, E E Jansen, C Rohde, C Müller-Tidow, R Geffers, G Göhring, A Ganser, F Thol, M Heuser
Canonical mutations in IDH1 and IDH2 produce high levels of the R-enantiomer of 2-hydroxyglutarate (R-2HG), which is a competitive inhibitor of α-ketoglutarate (αKG)-dependent enzymes and a putative oncometabolite. Mutant IDH1 collaborates with HoxA9 to induce monocytic leukemia in vivo. We used two mouse models and a patient-derived acute myeloid leukemia xenotransplantation (PDX) model to evaluate the in vivo transforming potential of R-2HG, S-2HG and αKG independent of the mutant IDH1 protein. We show that R-2HG, but not S-2HG or αKG, is an oncometabolite in vivo that does not require the mutant IDH1 protein to induce hyperleukocytosis and to accelerate the onset of murine and human leukemia...
August 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Alaa M Ali, Aibek E Mirrakhimov, Camille N Abboud, Amanda F Cashen
Leukostasis is a poorly understood and life-threatening complication of acute hyperleukocytic leukemia. The incidence of hyperleukocytosis and leukostasis differs among various subtypes of leukemias. While the pathophysiology of leukostasis is not fully understood, recent research has elucidated many novel pathways that may have therapeutic implications in the future. Respiratory and neurological compromise represents the classical clinical manifestations of leukostasis. If it is not diagnosed and treated rapidly, the one-week mortality rate is approximately 40%...
June 2016: Hematological Oncology
Tiejun Qin, Zefeng Xu, Yue Zhang, Yani Lin, Kun Ru, Liwei Fang, Hongli Zhang, Lijuan Pan, Naibo Hu, Shiqiang Qu, Jingya Wang, Ruixian Xing, Zhijian Xiao
OBJECTIVE: To estimate the long-term outcomes and the prognostic factors of homoharringtonine, cytarabine, daunorubicin or idarubicin (HAD/HAI) as induction chemotherapy in de novo acute myeloid leukemia (AML). METHODS: The CR rate, overall survival (OS) rate, relapse free survival (RFS) rate were retrospectively assayed in 143 de novo AML patients who received the HAD/HAI induction chemotherapy. The outcomes were compared among prognostic groups according to world health organization (WHO) classification, genetic prognosis and initial white blood cell (WBC) count...
February 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Takuya Miyazaki, Nana Abe, Etsuko Yamazaki, Satoshi Koyama, Kazuho Miyashita, Hiroyuki Takahashi, Yuki Nakajima, Takayoshi Tachibana, Aki Kamijo, Naoto Tomita, Yoshiaki Ishigastubo
Adequate management of hyperleukocytosis in patients with acute myeloid leukemia (AML) is essential for the prevention of life-threatening complications related to leukostasis and tumor lysis syndrome, but the optimal therapeutic strategy remains unclear. We report a 15-year-old girl with newly diagnosed AML who had extreme hyperleukocytosis (leukocyte count at diagnosis, 733,000/μl) leading to a brain hemorrhage. She was initially treated with hydroxyurea, but presented with brain hemorrhage due to leukostasis and underwent leukapheresis emergently with intensive care and mechanical ventilation...
February 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Kaiqi Liu, Xingli Zhao, Hui Wei, Dong Lin, Ying Wang, Chunlin Zhou, Bingcheng Liu, Wei Li, Huijun Wang, Chengwen Li, Qinghua Li, Benfa Gong, Shuning Wei, Yan Li, Yuntao Liu, Xiaoyuan Gong, Yingchang Mi, Jianxing Wang
OBJECTIVE: To explore the clinical and laboratory characteristics, treatment, prognostic factors of acute lymphoblastic leukemia (ALL) in adolescents. METHODS: Adolescents de novo ALL patients in Blood Disease Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences from September 1999 to September 2013 were enrolled in this study. Clinical data, therapeutic effect and prognostic factors were retrospectively analyzed. RESULTS: Of all 94 patients, 91 patients were treated in our center...
February 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
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