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Thrombocytopenia

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https://www.readbyqxmd.com/read/28436749/differential-diagnosis-of-neonatal-alloimmune-thrombocytopenia-type-2b-von-willebrand-disease
#1
Mathilde Penel-Page, Sandrine Meunier, Mathilde Fretigny, Sandra Le Quellec, Pierre Boisseau, Christine Vinciguerra, Catherine Ternisien, Lucia Rugeri
At birth, severe thrombocytopenia without context of infection should mainly suggest neonatal alloimmune thrombocytopenia (NAIT), especially in case of a platelet count below 20 GL(-1). We report two cases of severe neonatal thrombocytopenia, first suspected as being NAIT. Both had a platelet count below 20 GL(-1) with platelet clumps. The absence of alloantibodies and failure of platelet transfusion and intravenous immunoglobulins to improve the platelet count led to question the diagnosis and to evoke inherited bleeding disorders...
April 24, 2017: Platelets
https://www.readbyqxmd.com/read/28436582/phase-i-study-of-bortezomib-in-combination-with-irinotecan-in-patients-with-relapsed-refractory-high-risk-neuroblastoma
#2
Rajen Mody, Lili Zhao, Gregory Anthony Yanik, Valerie Opipari
PURPOSE: Prognosis for relapsed/refractory high-risk neuroblastoma (HR-NBL) remains poor. Bortezomib, a proteasome inhibitor, has shown preclinical activity against NBL as a single agent and in combination with cytotoxic chemotherapy including irinotecan. PATIENTS AND METHODS: Eighteen HR-NBL patients with primary refractory (n = 8) or relapsed (n = 10) disease were enrolled in a Phase I study using modified Time To Event Continual Reassessment Method. Bortezomib (1...
April 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28436299/mgmt-gene-variants-temozolomide-myelotoxicity-and-glioma-risk-a-concise-literature-survey-including-an-illustrative-case
#3
Meric A Altinoz, Ilhan Elmaci, Fatih Han Bolukbasi, Cumhur Gokhan Ekmekci, Guven Yenmis, Ramazan Sari, Aydin Sav
Temozolomide may cause thrombocytopenia or neutropenia in 3-4% of glioblastoma patients, respectively. However, pancytopenia is rarely reported. MGMT (O6-methylguanine-DNA-methyltransferase) enzyme repairs temozolomide-induced DNA mutations and associates both with antitumour efficacy and myelosuppression. Many studies on the effects of MGMT gene-methylation on temozolomide's effects exist, but much fewer publications concerning MGMT variants were documented. A full sequencing of the MGMT gene was performed in a female glioblastoma patient, who developed pancytopenia following temozolomide treatment...
April 23, 2017: Journal of Chemotherapy
https://www.readbyqxmd.com/read/28435653/paroxysmal-nocturnal-hemoglobinuria-in-the-differential-diagnosis-of-thrombocytopenia
#4
Fusun Gediz, Bahriye Kadriye Payzin, Ozlem Zekiye Cakmak, Yusuf Uzum, Damla Ernur, Fahri Sahin
Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain. On laboratory tests thrombocytopenia and iron deficiency anemia without any clinical findings were found. Flow cytometric evaluations showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28434836/retrograde-superselective-intra-arterial-chemotherapy-and-daily-concurrent-radiotherapy-for-t2-4n0-tongue-cancer-control-of-occult-neck-metastasis
#5
Shuhei Minamiyama, Kenji Mitsudo, Yuichiro Hayashi, Masaki Iida, Toshinori Iwai, Hideyuki Nakashima, Senri Oguri, Tomomichi Ozawa, Toshiyuki Koizumi, Makoto Hirota, Mitomu Kioi, Iwai Tohnai
OBJECTIVE: To evaluate the therapeutic results and control of occult neck metastasis in patients with T2-4N0 oral tongue squamous cell carcinoma treated with retrograde superselective intra-arterial chemotherapy and daily concurrent radiotherapy. STUDY DESIGN: Forty-two patients with T2-4N0 tongue cancer (17 with late T2; 13 with T3; and 12 with T4a disease, M0) were treated with intra-arterial chemoradiotherapy. Treatment consisted of retrograde superselective intra-arterial chemotherapy (docetaxel 50-70 mg/m(2), cisplatin 125-175 mg/m(2)) and daily concurrent radiotherapy (50-70 Gy) for 5-7 weeks...
February 21, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28433984/haemophagocytic-lymphohistiocytosis-presenting-as-hellp-syndrome-a-diagnostic-and-therapeutic-challenge
#6
Robert Noel Kerley, Raymond Michael Kelly, Mary Rose Cahill, Louise Clare Kenny
Haemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, haematological disorder, which can be clinically challenging to diagnose and manage. We report a case of HLH in a previously healthy 33-year-old primigravida. The patient presented at 22 weeks gestation with dyspnoea, abdominal pain, anaemia, thrombocytopenia and elevated liver enzymes suggestive of HELLP syndrome.HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets is considered a severe form of pre-eclampsia...
April 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28433732/the-clinical-significance-of-an-estimated-fetal-weight-below-the-10th-centile-a-comparison-of-outcomes-between-5th-versus-5th-9th-centile
#7
Malgorzata Mlynarczyk, Suneet P Chauhan, Hind A Baydoun, Catherine M Wilkes, Kimberly R Earhart, Yili Zhao, Christopher Goodier, Eugene Chang, Nicole M Lee Plenty, E Kaitlyn Mize, Michelle Owens, Shilpa Babbar, Dev Maulik, Emily DeFranco, David McKinney, Alfred Z Abuhamad
BACKGROUND: The association between small for gestational age (SGA; birth weight < 10(th) centile for gestational age) and neonatal morbidity is well established. Yet there is a paucity of data on the relationship between suspected SGA (sonographic estimated fetal weight <10(th) centile), at two thresholds and subsequent neonatal morbidity. OBJECTIVE: The objective of this study was to determine the relationship between sonographic estimated fetal weight (SEFW) < 5(th) centile versus 5-9(th) centile and neonatal morbidity...
April 19, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28432736/severe-primary-autoimmune-thrombocytopenia-itp-in-pregnancy-a-national-cohort-study
#8
A Care, S Pavord, M Knight, Z Alfirevic
OBJECTIVE: To quantify UK incidence of severe ITP in pregnancy, determine current treatment strategies and establish maternal and neonatal morbidity and mortality associated with severe ITP in pregnancy. DESIGN: A prospective national cohort study SETTING: United Kingdom POPULATION: Women with severe ITP; defined as platelets <50 x 10(9) /l in pregnancy or antenatal treatment of isolated low platelets. METHODS: Data collected via United Kingdom Obstetric Surveillance System (UKOSS) between 1(st) June 2013-31(st) January 2015 from all UK Consultant led obstetric units...
April 22, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28432223/fli1-level-during-megakaryopoiesis-affects-thrombopoiesis-and-platelet-biology
#9
Karen K Vo, Danuta J Jarocha, Randolph B Lyde, Vincent Hayes, Christopher S Thom, Spencer K Sullivan, Deborah L French, Mortimer Poncz
Friend Leukemia Virus Integration 1 (FLI1), a critical transcription factor (TF) during megakaryocyte differentiation, is amongst genes hemizygously deleted in Jacobsen syndrome, resulting in a macrothrombocytopenia termed Paris-Trousseau syndrome (PTSx). Recently, heterozygote human FLI1 mutations have been ascribed to cause thrombocytopenia. We studied induced-pluripotent stem cell (iPSC)-derived megakaryocytes (iMegs) to better understand these clinical disorders, beginning with iPSCs generated from a PTSx patient and iPSCs from a control line with a targeted heterozygous FLI1 knockout (FLI1(+/-))...
April 21, 2017: Blood
https://www.readbyqxmd.com/read/28432176/a-phase-i-study-of-the-cdk4-6-inhibitor-ribociclib-lee011-in-pediatric-patients-with-malignant-rhabdoid-tumors-neuroblastoma-and-other-solid-tumors
#10
Birgit Geoerger, Franck Bourdeaut, Steven G DuBois, Matthias Fischer, James I Geller, Nicholas G Gottardo, Aurélien Marabelle, Andrew D J Pearson, Shakeel Modak, Thomas Cash, Giles W Robinson, Marlyane Motta, Alessandro Matano, Suraj G Bhansali, Jason R Dobson, Sudha Parasuraman, Susan N Chi
Purpose: The cyclin-dependent kinase (CDK) 4/6 inhibitor, ribociclib (LEE011), displayed preclinical activity in neuroblastoma and malignant rhabdoid tumor (MRT) models. In this phase I study, the maximum tolerated dose (MTD) and recommended phase II dose (RP2D), safety, pharmacokinetics (PK), and preliminary activity of single-agent ribociclib were investigated in pediatric patients with neuroblastoma, MRT, or other cyclin D-CDK4/6-INK4-retinoblastoma pathway-altered tumors.Experimental Design: Patients (aged 1-21 years) received escalating once-daily oral doses of ribociclib (3-weeks-on/1-week-off)...
April 21, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28431186/unfractionated-heparin-versus-low-molecular-weight-heparins-for-avoiding-heparin-induced-thrombocytopenia-in-postoperative-patients
#11
REVIEW
Daniela R Junqueira, Liliane M Zorzela, Edson Perini
BACKGROUND: Heparin-induced thrombocytopenia (HIT) is an adverse drug reaction presenting as a prothrombotic disorder related to antibody-mediated platelet activation. It is a paradoxical immune reaction resulting in thrombin generation in vivo, which leads to a hypercoagulable state and the potential to initiate venous or arterial thrombosis. A number of factors are thought to influence the incidence of HIT including the type and preparation of heparin (unfractionated heparin (UFH) or low molecular weight heparin (LMWH)) and the heparin-exposed patient population, with the postoperative patient population at higher risk...
April 21, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28430955/measurement-of-platelet-counts-and-volume-using-magnesium-sulfate-as-an-anticoagulant-%C3%A2-comparison-of-impedance-and-light-scatter-technology
#12
Steffen Mannuß, Peter Kohlschein, Katrin Dreißiger, Peter Schuff-Werner
Objectives: Magnesium sulfate (MgSO 4 ) was recently reported as an alternative in vitro anticoagulant in pseudo-thrombocytopenia. Its suitability as an anticoagulant for the determination of reliable platelet parameters is the subject of this study. Methods: Platelet count and mean platelet volume were measured in blood samples anticoagulated with EDTA and MgSO 4 and compared. The platelet parameters were determined by impedance (XE 5000 [Sysmex, Norderstedt, Germany]; DxH 800 [Beckman-Coulter, Krefeld, Germany]) and laser light-scatter technology (Advia 120 [Siemens Healthcare Diagnostics, Eschborn, Germany])...
November 1, 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28430954/increased-multinucleated-megakaryocytes-as-an-isolated-finding-in-bone-marrow-%C3%A2-a-rare-finding-and-its-clinical-significance
#13
Majd D Jawad, Ronald S Go, Kaaren K Reichard, Min Shi
Objectives: Multinucleated megakaryocytes are a unique morphologic form of megakaryocytes characterized by multiple, distinctly separated nuclei. We investigated whether increased multinucleated megakaryocytes (≥25%) in otherwise normal-appearing bone marrow were associated with the development of a myelodysplastic syndrome (MDS). Methods: We retrospectively reviewed the medical records and bone marrow biopsy specimens of patients evaluated at our institution from 2011 to 2015 that met all of the following criteria: (1) 25% or more multinucleated megakaryocytes, (2) no other dysplastic features, (3) absence of a myeloid neoplasm, and (4) absence of neoplastic karyotypic abnormalities...
November 1, 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28429247/a-review-of-the-novel-application-and-potential-adverse-effects-of-proton-pump-inhibitors
#14
REVIEW
Li-Yuan Yu, Lu-Ning Sun, Xue-Hui Zhang, Yue-Qi Li, Lei Yu, Zi-Qing-Yun Yuan, Ling Meng, Hong-Wen Zhang, Yong-Qing Wang
Proton pump inhibitors (PPIs) are known as a class of pharmaceutical agents that target H(+)/K(+)-ATPase, which is located in gastric parietal cells. PPIs are widely used in the treatment of gastric acid-related diseases including peptic ulcer disease, erosive esophagitis and gastroesophageal reflux disease, and so on. These drugs present an excellent safety profile and have become one of the most commonly prescribed drugs in primary and specialty care. Except for gastric acid-related diseases, PPIs can also be used in the treatment of Helicobacter pylori infection, viral infections, respiratory system diseases, cancer and so on...
April 20, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28429051/induction-chemotherapy-followed-by-concurrent-chemoradiotherapy-vs-concurrent-chemoradiotherapy-for-locoregionally-advanced-nasopharyngeal-carcinoma-a-retrospective-cohort-study
#15
Jianyuan Zhang, Shaojun Chen, Guisheng Li, Weihua Zhang, Tingting Qin, Ping Yin, Haixin Huang, Hongwei Jiang
PURPOSE: To evaluate the efficacy and toxicity of induction chemotherapy followed by concurrent chemoradiotherapy vs. concurrent chemoradiotherapy for locoregionally advanced nasopharyngeal carcinoma (NPC). METHODS: We reviewed data of locoregionally advanced NPC patients who underwent 2 different treatment plans, 1 with induction chemotherapy followed by concurrent chemoradiotherapy (IC + CCRT) and the other with only concurrent chemoradiotherapy (CCRT). All patients received cisplatin 80 mg/m(2) 3 weeks one cycle concurrently with intensity-modulated radiation therapy, and three IC protocols were included for the IC + CCRT group...
April 20, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28428897/acute-myeloid-leukemia-with-basophilic-differentiation-transformed-from-myelodysplastic-syndrome
#16
Yasuhiro Tanaka, Atsushi Tanaka, Akiko Hashimoto, Kumiko Hayashi, Isaku Shinzato
Myelodysplastic syndrome (MDS) terminally transforms to acute myeloid leukemia (AML) or bone marrow failure syndrome, but acute myeloid leukemia with basophilic differentiation has been rarely reported. An 81-year-old man was referred to our department for further examination of intermittent fever and normocytic anemia during immunosuppressive treatment. Chromosomal analysis showed additional abnormalities involving chromosome 7. He was diagnosed as having MDS. At the time of diagnosis, basophils had not proliferated in the bone marrow...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28428724/hepatic-angiosarcoma-with-clinical-and-histological-features-of-kasabach-merritt-syndrome
#17
Sanya Wadhwa, Tae Hun Kim, Leah Lin, Gary Kanel, Takeshi Saito
Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome (KMS)...
April 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28428120/pathogenesis-of-immune-thrombocytopenia
#18
REVIEW
Sylvain Audia, Matthieu Mahévas, Maxime Samson, Bertrand Godeau, Bernard Bonnotte
Immune thrombocytopenia (ITP) is a rare autoimmune disease due to an abnormal T cell response, notably supported by splenic T follicular helper cells, that stimulates the proliferation and differentiation of autoreactive B cells. The antiplatelet autoantibodies they produce facilitate platelet phagocytosis by macrophages, essentially in the spleen. Macrophages contribute to the perpetuation of the auto-immune response as the main antigen-presenting cell during ITP. CD8(+) T cells also participate to thrombocytopenia by increasing platelet apoptosis...
April 17, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28427966/intravenous-immunoglobulin-for-treatment-of-severe-refractory-heparin-induced-thrombocytopenia
#19
Anand Padmanabhan, Curtis G Jones, Shannon M Pechauer, Brian R Curtis, Daniel W Bougie, Mehraboon S Irani, Barbara J Bryant, Jack B Alperin, Thomas G Deloughery, Kevin P Mulvey, Binod Dhakal, Renren Wen, Demin Wang, Richard H Aster
BACKGROUND: HIT complicated by severe thrombocytopenia and thrombosis can pose significant treatment challenges. Use of alternative anticoagulants in this setting may increase bleeding risks, especially in patients who have a protracted disease course. Additional therapies are lacking in this severely affected patient population. METHODS: We describe three HIT patients who had severe thromboembolism and prolonged thrombocytopenia refractory to standard treatment but achieved an immediate and sustained response to intravenous immunoglobulin G (IVIg) therapy...
April 17, 2017: Chest
https://www.readbyqxmd.com/read/28427514/triplet-versus-doublet-combination-regimens-for-the-treatment-of-relapsed-or-refractory-multiple-myeloma-a-meta-analysis-of-phase-iii-randomized-controlled-trials
#20
REVIEW
Zhiqiang Sun, Fang Zheng, Suwan Wu, Yanjuan Liu, Hehe Guo, Yichen Liu
During the past decades, several prospective trials had been conducted to assess the efficacy and toxicities of triplet versus doublet combination regimens for the treatment of relapsed or refractory multiple myeloma (RRMM), but the results were controversial. We thus performed a systematic literature search to identify relevant trials. Summary hazard ratios (HRs), relative risks (RRs), and 95% confidence intervals (95%CIs) were calculated. A total of 3197 RRMM patients were included for analysis. The pooled results demonstrated that triplet combination therapies significantly improve OS (HR 0...
May 2017: Critical Reviews in Oncology/hematology
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