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Liver disease, hemolytic anemia

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https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#1
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
March 8, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28225953/drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-and-its-relation-with-autoimmunity-in-a-reference-center-in-mexico
#2
Juan Manuel Ruiz Matta, Silvia Méndez Flores, Judith Domínguez Cherit
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe adverse drug reaction, with a reported mortality of 10%. Long-term outcomes involve organic failure and autoimmune diseases in some populations. OBJECTIVE: To evaluate the clinical prognosis of patients with drug reaction with eosinophilia and systemic symptoms. METHODS: We conducted a retrospective review at a referral hospital in Mexico City in a period of 22 years (1992-2013), looking up for records with diagnosis of DRESS according to RegiSCAR criteria...
January 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28143953/hemolytic-anemia-repressed-hepcidin-level-without-hepatocyte-iron-overload-lesson-from-g%C3%A3-nther-disease-model
#3
Sarah Millot, Constance Delaby, Boualem Moulouel, Thibaud Lefebvre, Nathalie Pilard, Nicolas Ducrot, Cécile Ged, Philippe Lettéron, Lucia de Franceschi, Jean Charles Deybach, Carole Beaumont, Laurent Gouya, Hubert De Verneuil, Saïd Lyoumi, Hervé Puy, Zoubida Karim
Hemolysis occurring in hematologic diseases is often associated with an iron loading anemia. This iron overload is the result of a massive outflow of hemoglobin into the bloodstream, but the mechanism of hemoglobin handling has not been fully elucidated. Here, in a congenital erythropoietic porphyria mouse model, we evaluate the impact of hemolysis and regenerative anemia on hepcidin synthesis and iron metabolism. Hemolysis was confirmed by a complete drop in haptoglobin, hemopexin and increased plasma lactate dehydrogenase, an increased red blood cell distribution width and osmotic fragility, a reduced half-life of red blood cells, and increased expression of heme oxygenase 1...
February 2017: Haematologica
https://www.readbyqxmd.com/read/27869428/-hemolytic-anemia-as-the-first-clinical-presentation-of-wilson-disease-a-pediatric-case
#4
José A Henao C, Kathia Valverde Muñoz, María L Ávila A
Wilson disease is an autosomal recessive disorder of the copper's hepatic metabolism; it results in toxicity due to accumulation of the mineral. The hemolytic anemia is present in 17% at some point of the disease, although it is a rare initial clinical presentation. CASE REPORT: 11 years old boy who presented with negative Coombs hemolytic anemia and elevation of liver enzymes. The possibility of Wilson disease was considered, which was confirmed with the finding of a Kayser-Fleischer ring in the eye exam...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#5
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates...
February 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27824770/microangiopathic-hemolytic-anemia-due-to-malignancy-in-pregnancy
#6
Sarah K Happe, Amanda C Zofkie, David B Nelson
BACKGROUND: Microangiopathic disorders during pregnancy remain a diagnostic challenge because these conditions often imitate more common obstetric diseases. CASE: A 31-year-old multigravid woman delivered at term with a postpartum course complicated by laboratory indices consistent with hemolysis, elevated liver enzymes, and low platelet count syndrome. After clinical recovery, she was readmitted 2 weeks after delivery with presumptive pyelonephritis. Persistent hemolysis and worsening thrombocytopenia suggested a diagnosis of thrombotic microangiopathy, which did not respond to plasma exchange...
December 2016: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27428900/on-demand-erythrocyte-disposal-and-iron-recycling-requires-transient-macrophages-in-the-liver
#7
Igor Theurl, Ingo Hilgendorf, Manfred Nairz, Piotr Tymoszuk, David Haschka, Malte Asshoff, Shun He, Louisa M S Gerhardt, Tobias A W Holderried, Markus Seifert, Sieghart Sopper, Ashley M Fenn, Atsushi Anzai, Sara Rattik, Cameron McAlpine, Milan Theurl, Peter Wieghofer, Yoshiko Iwamoto, Georg F Weber, Nina K Harder, Benjamin G Chousterman, Tara L Arvedson, Mary McKee, Fudi Wang, Oliver M D Lutz, Emanuele Rezoagli, Jodie L Babitt, Lorenzo Berra, Marco Prinz, Matthias Nahrendorf, Guenter Weiss, Ralph Weissleder, Herbert Y Lin, Filip K Swirski
Iron is an essential component of the erythrocyte protein hemoglobin and is crucial to oxygen transport in vertebrates. In the steady state, erythrocyte production is in equilibrium with erythrocyte removal. In various pathophysiological conditions, however, erythrocyte life span is compromised severely, which threatens the organism with anemia and iron toxicity. Here we identify an on-demand mechanism that clears erythrocytes and recycles iron. We show that monocytes that express high levels of lymphocyte antigen 6 complex, locus C1 (LY6C1, also known as Ly-6C) ingest stressed and senescent erythrocytes, accumulate in the liver via coordinated chemotactic cues, and differentiate into ferroportin 1 (FPN1, encoded by SLC40A1)-expressing macrophages that can deliver iron to hepatocytes...
August 2016: Nature Medicine
https://www.readbyqxmd.com/read/27375817/insulator-based-dielectrophoretic-diagnostic-tool-for-babesiosis
#8
Ezekiel O Adekanmbi, Massaro W Ueti, Brady Rinaldi, Carlos E Suarez, Soumya K Srivastava
Babesia species are obligate intraerythrocytic tick-borne protozoan parasites that are the etiologic agents of babesiosis, a potentially life-threatening, malaria-like illness in humans and animals. Babesia-infected people have been known to suffer from complications including liver problems, severe hemolytic anemia, and kidney failure. As reported by the Food and Drug Administration, 38% of mortality cases observed in transfusion recipients were associated with transfusion transmitted diseases of which babesiosis is the chief culprit...
May 2016: Biomicrofluidics
https://www.readbyqxmd.com/read/27352093/causes-of-macrocytic-anemia-among-628-patients-mean-corpuscular-volumes-of-114-and-130%C3%A2-fl-as-critical-markers-for-categorization
#9
Natsuko Takahashi, Junichi Kameoka, Naoto Takahashi, Yoshiko Tamai, Kazunori Murai, Riko Honma, Hideyoshi Noji, Hisayuki Yokoyama, Yasuo Tomiya, Yuichi Kato, Kenichi Ishizawa, Shigeki Ito, Yoji Ishida, Kenichi Sawada, Hideo Harigae
There have been no studies on the distribution of causes of macrocytic anemia with respect to mean corpuscular volume (MCV) cutoff values. We retrospectively investigated the causes of macrocytic anemia (MCV ≥100 fL) among 628 patients who visited the outpatient hematology clinic in Tohoku University Hospital. To ensure data validity, we also analyzed data from 307 patients in eight other hospitals in the Tohoku district. The leading causes of macrocytic anemia (number of patients, %) were myelodysplastic syndromes (121, 19...
September 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27316915/french-intensive-care-society-international-congress-r%C3%A3-animation-2016
#10
(no author information available yet)
No abstract text is available yet for this article.
June 2016: Annals of Intensive Care
https://www.readbyqxmd.com/read/27296447/small-molecule-phagocytosis-inhibitors-for-immune-cytopenias
#11
REVIEW
Anton Neschadim, Lakshmi P Kotra, Donald R Branch
Immune cytopenias are conditions characterized by low blood cell counts, such as platelets in immune thrombocytopenia (ITP) and red blood cells in autoimmune hemolytic anemia (AIHA). Chronic ITP affects approximately 4 in 100,000 adults annually while AIHA is much less common. Extravascular phagocytosis and massive destruction of autoantibody-opsonized blood cells by macrophages in the spleen and liver are the hallmark of these conditions. Current treatment modalities for ITP and AIHA include the first-line use of corticosteroids; whereas, IVIg shows efficacy in ITP but not AIHA...
August 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27249898/wilson-s-disease-in-thai-children-between-2000-and-2012-at-king-chulalongkorn-memorial-hospital
#12
Palittiya Sintusek, Voranush Chongsrisawat, Yong Poovorawan
OBJECTIVE: Wilson's disease (WD) is a rare autosomal recessive disorder characterized by copper accumulation. Clinical presentations are extraordinarily diverse, and currently no single diagnostic test can confirm WD with high accuracy. A complete understanding of the presentations and improved diagnostic methods are important for disease management. The authors' aimed to examine disease characteristics, management, and treatment outcome of WD in children, especially when genetic analysis and liver copper measurements were limited...
February 2016: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
https://www.readbyqxmd.com/read/27169452/-fulminant-visceral-disseminated-varicella-zoster-virus-infection-without-skin-involvement-in-a-patient-with-autoimmune-hemolytic-anemia-on-prednisolone-therapy
#13
REVIEW
Megumi Akiyama, Kota Yoshifuji, Tetsuya Fukuda, Shuji Tohda, Tohru Miki, Osamu Miura, Masahide Yamamoto
An 80-year-old man with autoimmune hemolytic anemia (AIHA) received immunosuppressive therapy with prednisolone (1 mg/kg). One month later, his hemoglobin level had normalized, and the prednisolone dose was tapered. The next day, he complained of acute and progressive back pain. He was admitted to our hospital for further examination approximately 24 h after the pain had started. Computed tomography revealed only localized pneumonia. However, he showed signs of severe disseminated intravascular coagulation (DIC), liver dysfunction, and respiratory failure...
April 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27098678/effect-and-safety-of-daclatasvir-asunaprevir-combination-therapy-for-chronic-hepatitis-c-virus-genotype-1b-infected-patients-on-hemodialysis
#14
Ryoichi Miyazaki, Kyoko Miyagi
The prevalence of anti-hepatitis C virus (HCV)antibodies is higher in hemodialysis patients than in the general population, and several studies have reported poor vital prognosis in HCV-infected dialysis patients because infection with HCV is not only the cause of cirrhosis and hepatocellular carcinoma, but also a risk factor for cardiovascular disease. Although sustained virologic response (SVR) is achievable with interferon and ribavirin in dialysis patients, SVR rates are lower, and the use of ribavirin is challenging because of the risk of hemolytic anemia...
October 2016: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/27047209/immunological-hematological-biochemical-and-histopathological-studies-on-cows-naturally-infected-with-lumpy-skin-disease
#15
Ahmed N F Neamat-Allah
AIM: Lumpy skin disease (LSD) is an infectious viral disease of cattle caused by LSD virus (LSDV) of the family Poxviridae characterized by skin nodules covering all parts of the body. There are many aspects of LSD remaining unknown, thus immunological, hematological, and biochemical parameters were estimated. MATERIALS AND METHODS: During an outbreak of LSD in Sharkia governorate from Egypt, 211 cows aging (2-4 years) were examined clinically for the presence of LSD lesions during the period from July to November 2014...
September 2015: Veterinary World
https://www.readbyqxmd.com/read/26989287/intracardiac-thrombosis-in-sickle-cell-disease
#16
Marzieh Nikparvar, Mohammad Reza Evazi, Tasnim Eftekhari, Farzaneh Moosavi
In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive crisis. The patient had manifestations of microangiopathic hemolytic anemia, including laboratory evidence of hemolytic anemia, thrombocytopenia, respiratory distress, fever, jaundice, and abnormal liver function and coagulation tests, accompanied by clot formation on the Eustachian valve of the inferior vena cava in the right atrium and also a long and worm-like thrombus in the right ventricle...
March 2016: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/26862317/dress-syndrome-associated-with-type-2-diabetes-in-a-child
#17
Semiha Bahceci Erdem, Hikmet Tekin Nacaroglu, Ozlem Bag, Canan Sule Unsal Karkiner, Huseyin Anil Korkmaz, Demet Can
Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon, life-threatening drug reaction. The basic findings are skin rash, multiorgan involvement, and eosinophilia. Most of the aromatic anticonvulsants, such as phenytoin, phenobarbital and carbamazepine can induce DRESS. Herein we report a 14-year-old patient with DRESS syndrome related to carbamazepine use. The patient presented with signs of involvement of the skin, lungs, liver, and microscopic hematuria. Carbamazepine treatment was discontinued; antihistamines and steroids were started...
2015: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/26841192/giant-cell-hepatitis-with-autoimmune-hemolytic-anemia-in-a-korean-infant
#18
Myung Hyun Cho, Hee Sun Park, Hye Seung Han, Sun Hwan Bae
Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a very rare disease characterized by early onset and severe clinical manifestations, including immune hemolytic anemia and hepatitis with cholestasis. The prognosis is poor despite aggressive immunosuppressive therapy. We report here the first case of GCH with AHA in East Asia. A 2-month-old boy was admitted with jaundice. Blood test indicated abnormal liver function and low hemoglobin. Direct Coombs test and several autoantibodies associated with liver disease were positive, and liver biopsy was consistent with GCH...
July 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/26807378/acute-hepatic-phenotype-of-wilson-disease-clinical-features-of-acute-episodes-and-chronic-lesions-remaining-in-survivors
#19
Hisao Hayashi, Yasuaki Tatsumi, Shinsuke Yahata, Hiroki Hayashi, Kenji Momose, Ryohei Isaji, Youji Sasaki, Kazuhiko Hayashi, Shinya Wakusawa, Hidemi Goto
BACKGROUND AND AIMS: Wilson disease (WD) is an inherited disorder of copper metabolism, and an international group for the study of WD (IGSW) has proposed three phenotypes for its initial presentation: acute hepatic, chronic hepatic, and neurologic phenotypes. Characterization of the acute hepatic phenotype may improve our understanding of the disease. METHODS: Clinical features of 10 WD patients with the acute hepatic phenotype and characteristics of chronic lesions remaining in survivors were assessed by the European Association for the Study of the Liver (EASL) guidelines...
December 28, 2015: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/26707317/choice-of-partial-splenic-embolization-technique-in-liver-transplant-recipients-correlates-with-risk-of-infectious-complications
#20
M A Elfeki, R Paz-Fumagalli, A M Tiemeier, S Pungpapong, D M Sella, G T Frey, K R Musto, J H Nguyen, D M Harnois
BACKGROUND: Complications of cirrhosis may persist after liver transplantation. When indicated, partial splenic embolization (PSE) is an alternative to splenectomy but can cause severe infection. The identification of modifiable risk factors when performing PSE in immunocompromised liver transplant recipients may help reduce the risk of severe infection. METHODS: Data were collected retrospectively for all PSE performed after liver transplantation at a single institution and included demographics, etiology of liver disease, indication for PSE, vaccination status, laboratory findings, procedural details, extent and pattern of splenic infarction, hospital length-of-stay, readmissions, procedural complications, and mortality...
December 2015: Transplantation Proceedings
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