keyword
MENU ▼
Read by QxMD icon Read
search

hematologic disorders

keyword
https://www.readbyqxmd.com/read/29156642/is-the-benefit-risk-ratio-for-patients-with-transfusion-dependent-thalassemia-treated-by-unrelated-cord-blood-transplantation-favorable
#1
REVIEW
Tang-Her Jaing
Transfusion-dependent thalassemia (TDT) is an inherited disorder characterized by absent or defective production of α- or β-hemoglobin chains. If untreated, the disease invariably culminates in death in early infancy due to cardiac failure or overwhelming infection. Although there is clear evidence of good health-related quality of life and return to normal life style, the choice to undergo hematopoietic stem cell transplantation (HSCT) remains a challenge because of the potential risk of transplant-related mortality (TRM) in TDT...
November 20, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29156497/melanoma-in-patients-with-gata2-deficiency
#2
Jannett Nguyen, Tiffany Alexander, Hong Jiang, Natasha Hill, Zied Abdullaev, Svetlana D Pack, Amy P Hsu, Steven M Holland, Dennis D Hickstein, Eric A Engels, Isaac Brownell
GATA2 deficiency is a recently described genetic disorder affecting hematopoietic stem cells and is associated with immunodeficiency, cutaneous findings, and hematologic malignancy. The risk of non-hematologic malignancies is unclear. To explore the incidence and clinical course of melanoma in GATA2 deficient patients we conducted a retrospective chart review of 71 patients with GATA2 deficiency. We identified two patients with melanoma, including an invasive melanoma presenting as a graft-versus-tumor effect following bone marrow transplantation...
November 20, 2017: Pigment Cell & Melanoma Research
https://www.readbyqxmd.com/read/29154038/dna-damage-and-neurodegenerative-phenotypes-in-aged-ciz1-null-mice
#3
Mohammad Moshahid Khan, Jianfeng Xiao, Damini Patel, Mark S LeDoux
Cell-cycle dysfunction and faulty DNA repair are closely intertwined pathobiological processes that may contribute to several neurodegenerative disorders. CDKN1A interacting zinc finger protein 1 (CIZ1) plays a critical role in DNA replication and cell-cycle progression at the G1/S checkpoint. Germline or somatic variants in CIZ1 have been linked to several neural and extra-neural diseases. Recently, we showed that germline knockout of Ciz1 is associated with motor and hematological abnormalities in young adult mice...
November 16, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29153343/prevalence-of-anemia-in-hospitalized-internal-medicine-patients-correlations-with-comorbidities-and-length-of-hospital-stay
#4
Carlo Zaninetti, Catherine Klersy, Concetta Scavariello, Raffaella Bastia, Carlo L Balduini, Rosangela Invernizzi
BACKGROUND: Several studies have documented a linear growth of the prevalence of anemia with aging. Especially in hospitalized elderly patients, anemia can aggravate the course of chronic disorders, and ultimately get worse the clinical outcomes. METHODS: To evaluate the distribution, the main causes, and the possible correlations of anemia with comorbidities and length of hospitalization in a population of internal medicine inpatients, we carried out an observational study on a cohort of 923 consecutive admissions relative to 856 subjects...
November 16, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29149370/-hematological-diagnosis-in-the-corneal-consultation
#5
J Wasielica-Poslednik, A Gericke, M Munder, N Pfeiffer, W Lisch
This case report describes a diagnostic chain in a patient with atypical bilateral corneal opacity, which led to the diagnosis of a hematological disorder. The patient's medical history, clinical appearance and findings in confocal microscopy gave rise to the suspicion of a paraproteinemic keratopathy. The hematological laboratory diagnostics revealed a monoclonal gammopathy of the IgG kappa type. The bone marrow puncture led to the diagnosis of a lymphoplasmacytic lymphoma, which belongs to the group of B‑cell non-Hodgkin's lymphomas...
November 17, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29147753/defective-wnt-signaling-associates-with-bone-marrow-fibrosis-a-cross-sectional-cohort-study-in-a-family-with-wnt1-osteoporosis
#6
R E Mäkitie, R Niinimäki, S Kakko, T Honkanen, P E Kovanen, O Mäkitie
This study explores bone marrow function in patients with defective WNT1 signaling. Bone marrow samples showed increased reticulin and altered granulopoiesis while overall hematopoiesis was normal. Findings did not associate with severity of osteoporosis. These observations provide new insight into the role of WNT signaling in bone marrow homeostasis. INTRODUCTION: WNT signaling regulates bone homeostasis and survival and self-renewal of hematopoietic stem cells. Aberrant activation may lead to osteoporosis and bone marrow pathology...
November 16, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/29147590/paroxysmal-nocturnal-hemoglobinuria-in-pregnancy-a-dilemma-in-treatment-and-thromboprophylaxis
#7
Arpan Patel, Athira Unnikrishnan, Martina Murphy, Robert Egerman, Sarah Wheeler, Ashley Richards, John Wingard
Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Treatment frequently poses conundrum. The safety and efficacy of eculizumab during pregnancy and breast feeding have not been extensively studied and contraception has been recommended due to potential for teratogenicity...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29145678/-thrombocytosis-and-thrombocytopenia-background-and-clinical-relevance
#8
Kai Wille, Parvis Sadjadian, Martin Griesshammer
Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET)...
November 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29143804/telomeres-and-telomerase-in-hematopoietic-dysfunction-prognostic-implications-and-pharmacological-interventions
#9
REVIEW
Theresa Vasko, Andrea Kaifie, Matthias B Stope, Thomas Kraus, Patrick Ziegler
Leukocyte telomere length (TL) has been suggested as a marker of biological age in healthy individuals, but can also reflect inherited and acquired hematopoietic dysfunctions or indicate an increased turnover of the hematopoietic stem and progenitor cell compartment. In addition, TL is able to predict the response rate of tyrosine kinase inhibitor therapy in chronic myeloid leukemia (CML), indicates clinical outcomes in chronic lymphocytic leukemia (CLL), and can be used as screening tool for genetic sequencing of selected genes in patients with inherited bone marrow failure syndromes (BMFS)...
October 28, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29142442/debates-regarding-lean-patients-with-polycystic-ovary-syndrome-a-narrative-review
#10
REVIEW
Manu Goyal, Ayman S Dawood
Polycystic ovary syndrome (PCOS) is a complex syndrome showing the clinical features of an endocrine/metabolic disorder, including hyperinsulinemia and hyperandrogenism. Two phenotypes are present, either lean or obese, with different biochemical, hormonal, and metabolic profiles. Evidence suggests many treatment modalities that can be applied. However, many of these modalities were found to be not suitable for the lean phenotype of PCOS. Much contradictory research was found regarding lean patients with PCOS...
July 2017: Journal of Human Reproductive Sciences
https://www.readbyqxmd.com/read/29135393/assessing-psychological-distress-in-cancer-patients-the-use-of-distress-thermometer-in-an-outpatient-cancer-hematology-treatment-center
#11
Kareen N Tonsing, Martha Vungkhanching
The purpose of this study is to assess the feasibility of the distress thermometer (DT) and the accompanying problem checklist (PC) as a screening tool for psychological distress and the sources of those distress at an outpatient cancer treatment center in Central California. Forty-three patients completed the DT and the PC. Based on a recommended DT cutoff score of 4, patients were classified as "distressed" (>4) and "not-distressed" (<4). Respondents ranged in age from 34 to 87 years (mean = 60.44, SD = 12...
November 14, 2017: Social Work in Health Care
https://www.readbyqxmd.com/read/29128013/bronchiolitis-obliterans-syndrome-and-other-late-pulmonary-complications-after-allogeneic-hematopoietic-stem-cell-transplantation
#12
REVIEW
Anne Bergeron, Guang-Shing Cheng
As more individuals survive their hematologic malignancies after allogeneic hematopoietic stem cell transplantation (HSCT), there is growing appreciation of the late organ complications of this curative procedure for malignant and nonmalignant hematologic disorders. Late noninfectious pulmonary complications encompass all aspects of the bronchopulmonary anatomy. There have been recent advances in the diagnostic recognition and management of bronchiolitis obliterans syndrome, which is recognized as a pulmonary manifestation of chronic graft-versus-host disease...
December 2017: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29127275/allogeneic-stem-cell-transplantation-in-fully-mhc-matched-mauritian-cynomolgus-macaques-recapitulates-diverse-human-clinical-outcomes
#13
Benjamin J Burwitz, Helen L Wu, Shaheed Abdulhaqq, Christine Shriver-Munsch, Tonya Swanson, Alfred W Legasse, Katherine B Hammond, Stephanie L Junell, Jason S Reed, Benjamin N Bimber, Justin M Greene, Gabriela M Webb, Mina Northrup, Wolfram Laub, Paul Kievit, Rhonda MacAllister, Michael K Axthelm, Rebecca Ducore, Anne Lewis, Lois M A Colgin, Theodore Hobbs, Lauren D Martin, Betsy Ferguson, Charles R Thomas, Angela Panoskaltsis-Mortari, Gabrielle Meyers, Jeffrey J Stanton, Richard T Maziarz, Jonah B Sacha
Allogeneic hematopoietic stem cell transplantation (HSCT) is a critically important therapy for hematological malignancies, inborn errors of metabolism, and immunodeficiency disorders, yet complications such as graft-vs.-host disease (GvHD) limit survival. Development of anti-GvHD therapies that do not adversely affect susceptibility to infection or graft-vs.-tumor immunity are hampered by the lack of a physiologically relevant, preclinical model of allogeneic HSCT. Here we show a spectrum of diverse clinical HSCT outcomes including primary and secondary graft failure, lethal GvHD, and stable, disease-free full donor engraftment using reduced intensity conditioning and mobilized peripheral blood HSCT in unrelated, fully MHC-matched Mauritian-origin cynomolgus macaques...
November 10, 2017: Nature Communications
https://www.readbyqxmd.com/read/29125137/red-blood-cell-distribution-width-relative-lymphocyte-count-and-type-2-diabetes-mellitus-predict-all-cause-mortality-in-patients-with-advanced-heart-failure
#14
Bożena Szyguła-Jurkiewicz, Łukasz Siedlecki, Łukasz Pyka, Ewa Romuk, Piotr Przybyłowski, Mariusz Gąsior
INTRODUCTION    Early identification of patients with advanced heart failure (HF) who are at a higher risk for poor outcome is an important element of patient management, both from the medical and the economic point of view. OBJECTIVES    In the present study, we sought to determine the association between hematologic parameters assessed on admission and within the 3-year mortality period in patients consecutively admitted with advanced HF.  We also investigated the association between baseline demographic and clinical data and mortality...
November 9, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29115073/assessment-of-competence-in-emergency-medicine-among-healthcare-professionals-in-cameroon
#15
Sang Chul Kim, Young Sun Ro, Sang Do Shin, Dae Han Wi, Joongsik Jeong, Ju Ok Park, Kyong Min Sun, Kwangsoo Bae
Development of a competence-based curriculum is important. This study aimed to develop competence assessment tools in emergency medicine and use it to assess competence of Cameroonian healthcare professionals. This was a cross-sectional, descriptive study. Through literature review, expert survey, and discrimination tests, we developed a self-survey questionnaire and a scenario-based competence assessment tool for assessing clinical knowledge and self-confidence to perform clinical practices or procedures. The self-survey consisted of 23 domains and 94 questionnaires on a 5-point Likert scale...
December 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29112242/a-randomized-study-of-cyclosporine-and-methotrexate-with-or-without-methylprednisolone-for-the-prevention-of-graft-versus-host-disease-improved-long-term-survival-with-triple-prophylaxis
#16
Tapani Ruutu, Anne Nihtinen, Riitta Niittyvuopio, Eeva Juvonen, Liisa Volin
BACKGROUND: In a previously published study, the authors randomized 108 adult patients with a malignant hematologic disorder undergoing allogeneic bone marrow transplantation from a human leukocyte antigen-identical sibling to receive methylprednisolone (53 patients; MP+) or not to receive methylprednisolone (55 patients; MP-) as a part of graft-versus-host disease (GVHD) prophylaxis. All patients received cyclosporine and methotrexate. The cumulative incidence of acute GVHD was found to be significantly lower among the patients given MP...
November 7, 2017: Cancer
https://www.readbyqxmd.com/read/29112013/cancer-therapy-associated-lymphoproliferative-disorders-an-under-recognized-type-of-immunodeficiency-associated-lymphoproliferative-disorder
#17
Sergio Pina-Oviedo, Roberto N Miranda, L Jeffrey Medeiros
We describe the clinicopathologic features of 17 patients who had a hematologic malignancy of various types, were treated, and subsequently developed a lymphoproliferative disorder (LPD). There were 10 men and 7 women with a median age of 59 years (range, 36 to 83 y). The primary hematologic neoplasms included: 5 chronic lymphocytic leukemia/small lymphocytic lymphoma, 3 plasma cell myeloma, 2 acute monoblastic leukemia, and 1 case each of mixed-phenotype acute leukemia, chronic myeloid leukemia, splenic marginal zone lymphoma, follicular lymphoma, mantle cell lymphoma, T-cell prolymphocytic leukemia, and peripheral T-cell lymphoma...
November 3, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29111429/megakaryocyte-and-polyploidization
#18
REVIEW
Stefania Mazzi, Larissa Lordier, Najet Debili, Hana Raslova, William Vainchenker
In mammals, platelets are produced in the blood by cytoplasmic fragmentation of megakaryocytes (MKs). Platelet production is thus dependent on both the MK number and size. During differentiation, MKs switch from a division by mitosis to polyploidization by endomitosis to increase their size. The endomitotic process includes several successive rounds of DNA replication with an entry in mitosis with a failure in late cytokinesis and a defect in karyokinesis. This leads to a giant cell with a modal ploidy at 16N and one multilobulated nucleus...
October 27, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/29109595/the-role-of-hematological-indices-in-patients-with-acute-coronary-syndrome
#19
REVIEW
Jan Budzianowski, Konrad Pieszko, Paweł Burchardt, Janusz Rzeźniczak, Jarosław Hiczkiewicz
An increased systemic and local inflammation plays a key role in the pathophysiology of acute coronary syndrome (ACS). This review will discuss the role of hematological indices: white blood cells (WBC), neutrophil to lymphocyte ratio (NLR), red cell distribution width (RDW), and platelet indices, that is, platelet to lymphocyte ratio (PLR), mean platelet volume (MPV), and platelet distribution width (PDW) in the case of ACS. In recent years, a strong interest has been drawn to these indices, given that they may provide independent information on pathophysiology, risk stratification, and optimal management...
2017: Disease Markers
https://www.readbyqxmd.com/read/29107383/alteration-in-ikaros-expression-promotes-b-1-cell-differentiation-into-phagocytes
#20
Vivian Cristina de Oliveira, Ana Clara Pires Sodré, Caio Perez Gomes, Nilmar Silvio Moretti, João Bosco Pesquero, Ana Flavia Popi
Ikaros is a broad transcription factor pointed as a critical regulator of lymphocyte development. Recent reports have emphasized that distinct isoforms of Ikaros control the dichotomy of the hematopoietic system into lymphoid and myeloid lineages. In addition, expression of dominant-negative isoforms of Ikaros is linked to abnormal hematopoiesis, which could culminate in hematological disorders due to loss of function of the protein. B-1 cells are an intriguing subtype of B-lymphocytes that preserves some myeloid characteristics...
October 6, 2017: Immunobiology
keyword
keyword
101178
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"