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By Ronald Lands Benign hematologist
Luke Y C Chen, Andre Mattman, Michael A Seidman, Mollie N Carruthers
IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes...
March 2019: Haematologica
Victor R Gordeuk, Nigel S Key, Josef T Prchal
Here we critically evaluate the role of elevated hematocrit as the principal determinant of thrombotic risk in polycythemia and erythrocytosis, defined by an expansion of red cell mass. Since red cell volume determination is no longer readily available, in clinical practice, polycythemia and erythrocytosis are defined by elevated hemoglobin and hematocrit. Thrombosis is common in Chuvash erythrocytosis and polycythemia vera. Although the increased thrombotic risk is assumed to be due to the elevated hematocrit and an associated increase in blood viscosity, thrombosis does not accompany most types of erythrocytosis...
April 2019: Haematologica
Zin W Myint, Thein H Oo, Kyaw Z Thein, Aung M Tun, Hayder Saeed
Copper is a crucial micronutrient needed by animals and humans for proper organ function and metabolic processes such as hemoglobin synthesis, as a neurotransmitter, for iron oxidation, cellular respiration, and antioxidant defense peptide amidation, and in the formation of pigments and connective tissue. Multiple factors, either hereditary or acquired, contribute to the increase in copper deficiency seen clinically over the past decades. The uptake of dietary copper into intestinal cells is via the Ctr1 transporter, located at the apical membrane aspect of intestinal cells and in most tissues...
September 2018: Annals of Hematology
Miquel Vázquez-Santiago, Noelia Vilalta, Biel Cuevas, Joaquim Murillo, Dolors Llobet, Raquel Macho, Núria Pujol-Moix, Marina Carrasco, José Mateo, Jordi Fontcuberta, José Manuel Soria, Juan Carlos Souto
INTRODUCTION: Platelets play a role in the pathophysiology of venous thromboembolism (VTE). Some studies have not found an association between VTE and platelet aggregation. The PFA-100® analyser is an in vitro assay for assessing primary haemostasis. But, there are no studies to evaluate its association with VTE. We investigated the contribution of the global platelet function and aggregation in the development of VTE. MATERIAL AND METHODS: We analysed 800 individuals who were included in the RETROVE Study (Riesgo de Enfermedad TROmboembólica VEnosa)...
September 2018: Thrombosis Research
Neha Berry, Jahangeer Basha, Neelam Varma, Subhash Varma, Kaushal Kishor Prasad, Kim Vaiphei, Narendra Dhaka, Saroj K Sinha, Rakesh Kochhar
Background and Aims: Anemia is one of the most common extraintestinal manifestations of celiac disease (CD), with iron deficiency anemia (IDA) being the predominant cause. However, anemia in CD can have varied etiologies, including mixed nutritional deficiency. We aimed to study the prevalence and etiology of anemia in CD in a north Indian population. Methods: In this prospective observational study, consecutive patients with documented CD between January 2012 and December 2013 were included, and all patients underwent detailed clinical assessment; hematological investigations including iron profile, serum folate, and vitamin B12 levels; and esophageoduodenoscopy with duodenal biopsies for histopathological examination...
October 2018: JGH open: an open access journal of gastroenterology and hepatology
Lesha Pretorius, Douglas B Kell, Etheresia Pretorius
Alzheimer's disease and other similar dementias are debilitating neurodegenerative disorders whose etiology and pathogenesis remain largely unknown, even after decades of research. With the anticipated increase in prevalence of Alzheimer's type dementias among the more susceptible aging population, the need for disease-modifying treatments is urgent. While various hypotheses have been put forward over the last few decades, we suggest that Alzheimer's type dementias are triggered by external environmental factors, co-expressing in individuals with specific genetic susceptibilities...
2018: Frontiers in Neuroscience
Leendert Porcelijn, Elly Huiskes, Gonda Oldert, Martin Schipperus, Jaap J Zwaginga, Masja de Haas
Immune Thrombocytopenia (ITP) is diagnosed by exclusion of other causes for thrombocytopenia. Reliable detection of platelet autoantibodies would support the clinical diagnosis of ITP and prevent misdiagnosis. We optimized our diagnostic algorithm for suspected ITP using the direct monoclonal antibody immobilization of platelet antigens assay (MAIPA), which evaluates the presence of platelet autoantibodies on the glycoproteins (GP) IIb/IIIa, Ib/IX and V bound on the patient platelets. The direct MAIPA was shown to be a valuable technique for the detection of platelet autoantibodies and could possibly become a guide for optimizing therapy towards a more personalized treatment of ITP...
August 2018: British Journal of Haematology
Zahra Nikzad, Mina Iravani, Parvin Abedi, Nahid Shahbazian, Amal Saki
Iron deficiency anemia (IDA) is a common micronutrient deficiency worldwide, and an important health problem especially in women of reproductive age. This study aimed to determine the relationship between IDA and sexual satisfaction and function among reproductive-aged Iranian women. In this study, 129 women (52 with IDA and 77 non-IDA) with age 18-45 in Mahshahr, Iran were recruited. Data was gathered by a demographic questionnaire, Female Sexual Function Index (FSFI) and Larson Sexual Satisfaction Questionnaire...
2018: PloS One
Beverley J Hunt
Extremes of body weight are not uncommon in the modern world and include anorexia nervosa (AN) and obesity. Both conditions are associated with increased morbidity and mortality: AN has the highest mortality rate of all mental illnesses and unfortunately obesity has reached epidemic proportions and is a well-recognized risk factor for cardiovascular disease including venous thromboembolism (VTE). This article summarizes the current understanding of hemostatic changes of these extremes of body weight. The hemostatic changes of AN have not been well described...
October 2018: Seminars in Thrombosis and Hemostasis
Marvin T Nieman
No abstract text is available yet for this article.
November 1, 2018: Blood
Lisa N van der Vorm, Dana Huskens, Cécile H Kicken, Jasper A Remijn, Mark Roest, Bas de Laat, Adam Miszta
Physical activity is beneficial for health, for example, by lowering the risk of cardiovascular events. However, vigorous exercise is associated with the occurrence of thromboembolic events and sudden cardiac death, in particular in untrained individuals. Whereas acute exercise is known to cause a hypercoagulable state, repeated exposure to (strenuous) exercise by means of training may actually condition the hemostatic response to exercise. To date, the effects of exercise training on blood coagulability and the underlying mechanisms have yet to be fully discerned...
November 2018: Seminars in Thrombosis and Hemostasis
Mingming Qu, Qiang Liu, Hong-Guo Zhao, Jun Peng, Heyu Ni, Ming Hou, A J Gerard Jansen
Infectious complications are common and sometimes life threatening in patients with immune thrombocytopenia (ITP), mainly due to the immune-suppressive therapy. Recent evidence suggests a potential role of platelets in the inflammation process. In this clinical study, we further investigated the role of thrombocytopenia on infections in patients with primary ITP. We retrospectively evaluated data from the recently published large randomized clinical trial of a cohort of 195 patients with primary ITP, who were randomized for prednisone or high-dose dexamethasone...
September 2018: Annals of Hematology
Shruthimurthy Hassankrishnamurthy, Mayur D Mody, Vamsi K Kota
BACKGROUND Essential thrombocythemia (ET) is one of the BCR-ABL gene fusion negative chronic myeloproliferative disorders (MPDs), which also include polycythemia vera (PV), and myelofibrosis. Few clinical cases have reported the progression of ET to chronic myelogenous leukemia (CML) with the expression of the BCR-ABL gene. This report describes such a case and includes a review of other reported cases of CML co-occurring with BCR-ABL-negative chronic MPDs. CASE REPORT A 49-year-old woman was diagnosed with ET in 2007...
January 3, 2019: American Journal of Case Reports
Iuliana Vaxman, Morie Gertz
The term amyloidosis refers to a group of disorders in which protein fibrils accumulate in certain organs, disrupt their tissue architecture, and impair the function of the effected organ. The clinical manifestations and prognosis vary widely depending on the specific type of the affected protein. Immunoglobulin light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by deposition of a misfolded monoclonal light-chain that is secreted from a plasma cell clone. Demonstrating amyloid deposits in a tissue biopsy stained with Congo red is mandatory for the diagnosis...
2019: Acta Haematologica
Olivier Loréal, Thibault Cavey, François Robin, Moussa Kenawi, Pascal Guggenbuhl, Pierre Brissot
Genetic hemochromatosis is an iron overload disease that is mainly related to the C282Y mutation in the HFE gene. This gene controls the expression of hepcidin, a peptide secreted in plasma by the liver and regulates systemic iron distribution. Homozygous C282Y mutation induces hepcidin deficiency, leading to increased circulating transferrin saturation, and ultimately, iron accumulation in organs such as the liver, pancreas, heart, and bone. Iron in excess may induce or favor the development of complications such as cirrhosis, liver cancer, diabetes, heart failure, hypogonadism, but also complaints such as asthenia and disabling arthritis...
November 26, 2018: Pharmaceuticals
Joel N Buxbaum
New England Journal of Medicine, Volume 379, Issue 21, Page 2085-2086, November 2018.
November 22, 2018: New England Journal of Medicine
C H Yu, Y H Lu, J H Lu
No abstract text is available yet for this article.
February 1, 2019: QJM: Monthly Journal of the Association of Physicians
Claudia Bozzini, Silvia Galbiati, Elisa Tinazzi, Raffaella Aldigeri, Giovanna De Matteis, Domenico Girelli
No abstract text is available yet for this article.
February 2003: Haematologica
Deborah Schrag, Ethan Basch
No abstract text is available yet for this article.
November 8, 2018: JAMA: the Journal of the American Medical Association
Bart Barlogie, Joshua Richter
No abstract text is available yet for this article.
October 18, 2018: Blood
2018-11-03 12:35:37
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