76,295 results match your criteria Hemolytic Anemia


The Role of Plasmapheresis in Treating Lethal Cupric Sulfate Poisoning.

Authors:
Yu Du Yi Mou

Am J Med Sci 2018 Nov 29. Epub 2018 Nov 29.

Geroscience and Chronic Disease Department, The 8th Municipal People's Hospital, Chengdu, China. Electronic address:

The mortality rate of cupric sulfate is relatively high in contrast to that of other heavy metals. Cases of orally ingested cupric sulfate poisoning are very rare, with a reported half lethal dose of 10 g. Cupric sulfate poisoning leads to gastrointestinal corrosion, intravascular hemolysis, hemolytic anemia, methemoglobinemia and acute renal and hepatic impairment. Read More

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http://dx.doi.org/10.1016/j.amjms.2018.11.014DOI Listing
November 2018

Complement Activation Contributes to the Pathophysiology of Shiga Toxin-Associated Hemolytic Uremic Syndrome.

Microorganisms 2019 Jan 10;7(1). Epub 2019 Jan 10.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, 24126 Bergamo, Italy.

Shiga toxin (Stx)-producing (STEC) infections have become a threat to public health globally because of the severe illnesses that they can trigger, such as hemorrhagic colitis and the post-diarrheal hemolytic uremic syndrome (HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Glomerular endothelial cells are primary targets of Stx which, after binding to its specific receptor globotriaosylceramide, upregulates proinflammatory proteins involved both in the recruitment and adhesion of leukocytes and thrombus formation at the site of endothelial injury. In this review, we discuss the role of complement activation in promoting glomerular microvascular dysfunction, providing evidence from experimental models and patients with STEC-HUS. Read More

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http://dx.doi.org/10.3390/microorganisms7010015DOI Listing
January 2019

Oral manifestations of sickle cell disease.

Br Dent J 2019 Jan;226(1):27-31

Henri Mondor Hospital, Dental Department, Paris-Descartes University, Ile-de France, France.

Sickle cell disease is one of the most common autosomal recessive genetic diseases. It gives rise to abnormally shaped red blood cells with altered function, the primary clinical features being haemolytic anaemia and vascular occlusion. Acute complications are frequent and variable and include chest syndrome, stroke, infection mainly due to asplenia, bone pain and priapism. Read More

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http://www.nature.com/doifinder/10.1038/sj.bdj.2019.4
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http://dx.doi.org/10.1038/sj.bdj.2019.4DOI Listing
January 2019
1 Read

Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus.

Acta Med Indones 2018 Oct;50(4):343-345

Department of Internal Medicine, Faculty of Medicine, Padjadjaran University, Bandung, Indonesia.

A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Read More

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October 2018

[Analysis of 5 children with congenital thrombotic thrombocytopenic purpura].

Zhonghua Er Ke Za Zhi 2019 Jan;57(1):50-54

Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

To explore the clinical features and points of diagnosis and treatment for congenital thrombotic thrombocytopenic purpura (TTP) in children. The clinical manifestations, laboratory tests, genetic analysis and treatments of 5 children with congenital TTP hospitalized in Beijing Children's Hospital, Capital Medical University from February 2015 to July 2017 were analyzed retrospectively. Among the 5 children with congenital TTP diagnosed by genetic monitoring and enzymology, there were 1 male and 4 females, 3 cases had suspicious positive family history, the age of onset was several hours after birth (range several hours after birth to 28 months). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.01.012DOI Listing
January 2019
1 Read

Autoimmune Cytopenias Occurring after Treatment with Chemoimmunotherapy for Non-Hodgkin Lymphomas.

Acta Haematol 2019 Jan 10;141(2):79-83. Epub 2019 Jan 10.

Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. Read More

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http://dx.doi.org/10.1159/000495600DOI Listing
January 2019

Ceftriaxone-induced immune hemolytic anemia in a case with large vestibular aqueduct syndrome after cochlear implant.

Chin Med J (Engl) 2019 Jan;132(1):100-102

Department of Pharmacy, Peking University Third Hospital, Beijing 100191, China.

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http://dx.doi.org/10.1097/CM9.0000000000000017DOI Listing
January 2019
1 Read

Severe congenital hemolytic anemia caused by a novel compound heterozygous PKLR gene mutation in a Chinese boy.

Chin Med J (Engl) 2019 Jan;132(1):92-95

Department of Hematology, Hematologic Research Laboratory, West China Hospital of Sichuan University. Chengdu, Sichuan 610041, China.

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http://dx.doi.org/10.1097/CM9.0000000000000027DOI Listing
January 2019

Immune Cytopenias After Ex-Vivo CD34+ Selected Allogeneic Hematopoietic Cell Transplantation.

Biol Blood Marrow Transplant 2019 Jan 6. Epub 2019 Jan 6.

Adult Bone Marrow Transplant Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York; Department of Medicine, Weill Cornell Medical College, New York, New York.

Immune mediated cytopenias (IC), such as immune thrombocytopenia (ITP) and immune hemolytic anemia (IHA), are among the adverse events after allogeneic hematopoietic cell transplantation (allo-HCT). Previous reports suggest that in-vivo T-cell depletion may increase the incidence of IC after allo-HCT. We evaluated whether a strategy that reduces functional donor T-cells via ex-vivo CD34-selection associates with the development of IC in a cohort of 408 patients who underwent allo-HCT for hematologic malignancy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10838791193001
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http://dx.doi.org/10.1016/j.bbmt.2018.12.842DOI Listing
January 2019
2 Reads

Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.

N Engl J Med 2019 Jan 9. Epub 2019 Jan 9.

From the Department of Haematology, University College London Hospitals, Cardiometabolic Program, National Institute for Health Research UCLH-UCL Biomedical Research Center, London (M.S.); the Division of Hematology, Department of Internal Medicine, Ohio State University, Columbus (S.R.C.); Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, and the Department of Pathophysiology and Transplantation, University of Milan, Milan (F.P.); the Department of Hematology, Saint-Antoine University Hospital, Paris (P.C.); the Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna (P.K.); the Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland (J.A.K.H.); the Division of Hematology, Duke University School of Medicine, Durham, NC (A.M.); the Hematology Department, Universidad Católica de Valencia Hospital Dr. Peset, Valencia, Spain (J.R.); the Departments of Medicine and Laboratory Medicine, St. Michael's Hospital and University of Toronto, Toronto (K.P.); and Clinical Development, Ablynx, Zwijnaarde, Belgium (F.C., D.B., H.D.W., R.K.Z.).

Background: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

Methods: In this double-blind, controlled trial, we randomly assigned 145 patients with TTP to receive caplacizumab (10-mg intravenous loading bolus, followed by 10 mg daily subcutaneously) or placebo during plasma exchange and for 30 days thereafter. Read More

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http://dx.doi.org/10.1056/NEJMoa1806311DOI Listing
January 2019

Hydroxyurea - An Essential Medicine for Sickle Cell Disease in Africa.

N Engl J Med 2019 01;380(2):187-189

From the Department of Hematology and Sickle Cell Program, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.

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http://dx.doi.org/10.1056/NEJMe1814706DOI Listing
January 2019

Novel Sickle Cell Disease Therapies: Targeting Pathways Downstream of Sickling.

Semin Hematol 2018 Apr 20;55(2):68-75. Epub 2018 Apr 20.

Department of Pediatrics, Division of Pediatric Hematology/Oncology, Albert Einstein College of Medicine, Children's Hospital at Montefiore, Bronx, NY. Electronic address:

Sickle cell disease is an inherited hemoglobinopathy characterized by hemolytic anemia, frequent painful episodes, poor quality of life, end organ damage and a shortened lifespan. Although the seminal event is the polymerization of the abnormal hemoglobin, the downstream pathophysiology of vaso-occlusion results from heterotypic interactions between the altered, adhesive sickle cell RBCs, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00371963173020
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http://dx.doi.org/10.1053/j.seminhematol.2018.04.007DOI Listing
April 2018
6 Reads

Generation of a core set of items to develop classification criteria for Scleroderma renal crisis using consensus methodology.

Arthritis Rheumatol 2019 Jan 7. Epub 2019 Jan 7.

Jewish General Hospital, Montreal, Canada.

Background: This project was undertaken to generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology.

Methods: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In Round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. Read More

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http://doi.wiley.com/10.1002/art.40809
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http://dx.doi.org/10.1002/art.40809DOI Listing
January 2019
2 Reads

Prevalence and associations of anti-phosphatidylserine/prothrombin antibodies with clinical phenotypes in patients with primary antiphospholipid syndrome: aPS/PT antibodies in primary antiphospholipid syndrome.

Thromb Res 2018 Dec 29;174:141-147. Epub 2018 Dec 29.

Department of Medicine, Division of Rheumatology, The Ottawa Hospital, University of Ottawa, 1967 Riverside Drive, K1H 7W9 Ottawa, ON, Canada. Electronic address:

Objective: The clinical significance of anti-phosphatidylserine/prothrombin (aPS/PT) in antiphospholipid syndrome (APS) is still controversial. We assessed the prevalence of aPS/PT antibodies, their association with other anti-phospholipid antibodies (aPL) and with different APS clinical phenotypes.

Methods: We included 95 primary APS patients according to the Sydney classification criteria, and patients with thrombocytopenia and/or hemolytic anemia who also fulfilled the serological APS criteria. Read More

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http://dx.doi.org/10.1016/j.thromres.2018.12.023DOI Listing
December 2018
1 Read

An integration-free iPSC line (MUSIi008-A) derived from a patient with severe hemolytic anemia carrying compound heterozygote mutations in KLF1 gene for disease modeling.

Stem Cell Res 2018 Dec 14;34:101344. Epub 2018 Dec 14.

Siriraj Center of Excellence for Stem Cell Research, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. Electronic address:

We generated an induced pluripotent stem cell (iPSC) line from peripheral blood mononuclear cells (PBMNCs) isolated from a 1-year old female carrying compound heterozygote for KLF1 mutations (G176RfsX179 and A298P mutations). Non-integrating Sendai viral (SeV) vectors containing KOS, hc-MYC and hKLF4 were used for reprogramming. The established MUSIi008-A cell line contained the same mutations found in the patient, expressed pluripotent markers, differentiated into cells of three embryonic germ layers both in vitro and in vivo, and exhibited normal karyotype. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18735061183027
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http://dx.doi.org/10.1016/j.scr.2018.09.021DOI Listing
December 2018
2 Reads
3.693 Impact Factor

Clinicopathological findings in 41 dogs (2008-2018) naturally infected with Ehrlichia ewingii.

J Vet Intern Med 2019 Jan 2. Epub 2019 Jan 2.

Vector Borne Disease Diagnostic Laboratory, Comparative Medicine Institute, College of Veterinary Medicine, North Carolina State University, Raleigh, North Carolina.

Background: Ehrlichia ewingii is the most seroprevalent Ehrlichia-infecting dogs in the southern and mid-western United States. Fever, lameness, and polyarthritis are commonly reported findings in dogs naturally infected with E. ewingii. Read More

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http://dx.doi.org/10.1111/jvim.15354DOI Listing
January 2019

Rasburicase induced severe hemolysis and methemoglobinemia in a Caucasian patient complicated by acute renal failure and ARDS.

Respir Med Case Rep 2019 23;26:142-145. Epub 2018 Dec 23.

Pulmonary and Critical Care Medicine, Marshall University School of Medicine, Huntington, WV, USA.

Rasburicase is a recombinant urate-oxidase enzyme and is a very important medication for tumor lysis syndrome. Methemoglobinemia and hemolysis are known side effects of rasburicase that result from oxidative stress caused by hydrogen peroxide, a byproduct generated during the breakdown of uric acid to allantoin. Patients with G6PD deficiency have a decreased tolerance to oxidative stress and are therefore at a greater risk of hemolysis and methemoglobinemia with rasburicase. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.12.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307101PMC
December 2018
1 Read

Case Report: Management of Corneal Clouding from Lecithin: Cholesterol Acyltransferase Deficiency.

Optom Vis Sci 2018 Dec 29. Epub 2018 Dec 29.

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York

Significance: Given that there are few reported cases of lecithin:cholesterol acyltransferase (LCAT) deficiency, recognition of the condition with proper management is notable. Long-term follow-up and contact lens fitting after penetrating keratoplasty provide best possible outcomes.

Purpose: The purpose of this study was to report a case of LCAT deficiency successfully treated with penetrating keratoplasty and longer-term follow-up with contact lens fitting. Read More

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http://dx.doi.org/10.1097/OPX.0000000000001329DOI Listing
December 2018
2 Reads

Acute Liver Failure due to Wilson Disease: Eight Years of the National Liver Transplant Program in Uruguay.

Ann Hepatol 2018 Dec;18(1):187-192

Hepatic Biliary and Pancreatic National Center - Teaching and Assistance Unit (UDA) and Bi-Intuitional Unit of Liver Transplantation, Military Hospital, Montevideo, Uruguay.

Introduction And Aim: Wilson's disease (WD) is an uncommon cause of acute liver failure (ALF). Our aim was to describe clinical features, diagnostic findings, treatments, and outcomes of patients with ALF due to WD.

Material And Methods: Retrospective medical record reviews of all patients with ALF due to WD in eight years in Uruguay. Read More

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http://dx.doi.org/10.5604/01.3001.0012.7911DOI Listing
December 2018

First Report of Known Rare Rhnull Phenotype Individuals in Iran.

Int J Hematol Oncol Stem Cell Res 2018 Jul;12(3):181-184

Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Rhnull phenotype is a rare blood group with a frequency of approximately 1 in 6 million individuals, transmitted via an autosomal recessive mode. It is characterized by the weak (Rhmod) or lack (Rhnull) of expression of all Rh antigens on the red cells. The clinical significance of its assessment is that such patients with Rhnull syndrome are associated with chronic hemolytic anemia of varying degrees. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305262PMC

Invasive molecular prenatal diagnosis of alpha and beta thalassemia among Hakka pregnant women.

Medicine (Baltimore) 2018 Dec;97(52):e13557

Clinical Core Laboratory.

This study is a retrospective analysis of the prenatal genetic diagnosis results of fetuses with high risk of major thalassemia to provide information for clinical genetic counseling and to better control the birth of major thalassemia child in Hakka population. Totally, 467 fetuses in at-risk pregnancies were collected from Meizhou people's hospital from January 2014 to December 2017. Genomic DNAs were extracted from peripheral blood of the couples and villus, amniotic fluid or cord blood of the fetuses. Read More

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http://dx.doi.org/10.1097/MD.0000000000013557DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314693PMC
December 2018
1 Read

Acute Retroviral Syndrome Presenting with Hemolytic Anemia Induced by G6PD Deficiency.

Trop Med Infect Dis 2018 Dec 27;4(1). Epub 2018 Dec 27.

Department of Medicine, Division of Infectious Diseases, University of Miami, Miller School of Medicine, Miami, Fl 33136, USA.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme defect described in humans. Hemolysis in affected patients is usually triggered by circumstances involving free radical damage. While acute HIV infection is known to be a state of overwhelming oxidative stress, virus-induced hemolytic events in G6PD-deficient patients has rarely been reported. Read More

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http://dx.doi.org/10.3390/tropicalmed4010006DOI Listing
December 2018
1 Read

G6PD deficiency is not an uncommon cause of pigment nephropathy.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1371-1375

Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Acute kidney injury (AKI) with evidence of hemolysis is associated with tropical infections. However, pigment-induced AKI can happen with relatively uncommon genetic causes of hemolytic anemia, i.e. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1371/248316
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http://dx.doi.org/10.4103/1319-2442.248316DOI Listing
December 2018
5 Reads

A Novel Mutation of Glucose Phosphate Isomerase (GPI) Causing Severe Neonatal Anemia Due to GPI Deficiency.

J Pediatr Hematol Oncol 2018 Dec 21. Epub 2018 Dec 21.

Department of Pediatrics, Alrijne Hospital, Leiderdorp.

Glucose-6-phosphate isomerase (GPI) deficiency is very rare, but one of the most common erythroenzymopathies, causing hereditary nonspherocytic hemolytic anemia. This case report describes the clinical features and the molecular etiology of a Dutch patient with GPI deficiency. She is the fifth patient with GPI deficiency identified to date in the Netherlands and was found to be compound heterozygous for the previously reported c. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001393DOI Listing
December 2018

Gastric duplication cyst in an adult with autoimmune hemolytic anemia: a case report and review of the literature.

J Med Case Rep 2018 Dec 26;12(1):380. Epub 2018 Dec 26.

Department of Surgical Oncology, Mohamed I University, 30050, Oujda, Morocco.

Background: Gastric duplication cysts are uncommon congenital anomalies found primarily in children and rarely seen in the adult population. Accurate diagnosis of cysts before resection is difficult even using the most advanced imaging techniques.

Case Presentation: In this report, we describe a 28-year-old Moroccan patient with a history of autoimmune hemolytic anemia who presented with an asymptomatic abdominal cystic mass detected during abdominal computed tomography performed before splenectomy. Read More

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http://dx.doi.org/10.1186/s13256-018-1895-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305996PMC
December 2018
1 Read

Genetic Mutations in the S-loop of Human Glutathione Synthetase: Links Between Substrate Binding, Active Site Structure and Allostery.

Comput Struct Biotechnol J 2019 29;17:31-38. Epub 2018 Nov 29.

Center for Advanced Scientific Computing and Modeling (CASCaM), Department of Chemistry, University of North Texas, Denton, TX 76203, United States.

The second step in the biosynthesis of the cellular antioxidant glutathione (GSH) is catalyzed by human glutathione synthetase (hGS), a negatively cooperative homodimer. Patients with mutations in hGS have been reported to exhibit a range of symptoms from hemolytic anemia and metabolic acidosis to neurological disorders and premature death. Several patient mutations occur in the S-loop of hGS, a series of residues near the negatively cooperative γ-GC substrate binding site. Read More

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http://dx.doi.org/10.1016/j.csbj.2018.11.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297838PMC
November 2018
1 Read

[Neonatal screening for glucose-6-phosphate dehydrogenase (G6PD) deficiency in Mauritania].

Pan Afr Med J 2018 25;30:224. Epub 2018 Jul 25.

Hôpital National de Nouakchott, Mauritanie.

Introduction: We conducted a cross-sectional descriptive study aiming to identify risk factors associated with G6PD deficiency, its frequency and geographic distribution in Nouakchott, in order to provide useful informations to monitor it. As this disease has never previously been studied in Mauritania, we tried to define the epidemiological profile and the burden of morbidity related to G6PD deficiency in a newborn population in two health structures in the city of Nouakchott.

Methods: This study was conducted in two hospitals in Nouakchott, at the Maternity and Infant Hospital and at the Health Center in Sebkha between August and October 2015. Read More

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http://dx.doi.org/10.11604/pamj.2018.30.224.13947DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295302PMC
January 2019

Vanishing spleen syndrome post right partial nephrectomy in a sicklemic patient.

Pan Afr Med J 2018 16;30:211. Epub 2018 Jul 16.

Division of Urology, Harlem Hospital Center, Columbia University, New York, NY, USA.

Splenic infarction after contralateral laparoscopic renal surgery has not, to our knowledge, been reported. The spleen is the most affected organ in sickle cell disease and the mechanism of auto infarction is thought to result from the crystallization of abnormal hemoglobin during periods of hypoxia or acidosis resulting in parenchymal ischemia and ultimately tissue necrosis. We report a case of 45 year old female with sickle cell disease who had an unremarkable spleen at the time of a laparoscopic right partial nephrectomy and was subsequently found to have marked diminution in her splenic volume. Read More

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http://www.panafrican-med-journal.com/content/article/30/211
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http://dx.doi.org/10.11604/pamj.2018.30.211.15454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6294983PMC
January 2019
3 Reads

Fatal Acute Hemolytic Transfusion Reaction due to Anti-Wr.

Transfus Med Hemother 2018 Nov 24;45(6):438-441. Epub 2018 Aug 24.

Laboratory of Clinical Chemistry, Hematology and Transfusion Medicine, Maasstad Hospital, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands.

Background: The Wr blood group antigen is a low-frequency antigen. Antibody screening sets used in pretransfusion laboratory investigations usually do not contain a Wr(a+) cell. If subsequent cross-matching is performed without indirect antiglobulin test (IAT), Wr antibodies reacting with donor red blood cells (RBCs) will be missed. Read More

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http://dx.doi.org/10.1159/000488863DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288625PMC
November 2018

Hemolytic jaundice induced by pharmacological dose ascorbic acid in glucose-6-phosphate dehydrogenase deficiency: A case report.

Medicine (Baltimore) 2018 Dec;97(51):e13588

Clinical Pharmacy, Shanghai East Hospital, Tongji University School of Medicine, Shanghai, China.

Rationale: Hemolysis induced by high dose ascorbic acid (AA) in patients with G6PD deficiency has been reported, but is rare. To our knowledge, this is the first reported case of a male with G6PD deficiency, coexpressed with cholecystolithiasis and cholecystitis, who developed extreme hemolysis and hyperbilirubinemia after receiving pharmacological doses ascorbic acid infusion.

Patient Concerns: A 27-year-old man history with glucose-6-phosphate dehydrogenase deficiency was admitted to our hospital because of cholecystolithiasis and cholecystitis. Read More

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http://dx.doi.org/10.1097/MD.0000000000013588DOI Listing
December 2018

Progressive Retinal Findings in Hemolytic Uremic Syndrome.

J Pediatr Ophthalmol Strabismus 2018 Dec 19;55:e49-e51. Epub 2018 Dec 19.

Ocular involvement in hemolytic uremic syndrome is rare and in most cases presents with retinal ischemia, hemorrhages, and neovascularization. The authors describe the progression of retinal involvement as a rare complication of typical hemolytic uremic syndrome in a 2-year-old boy. Progression of retinal findings were demonstrated with serial fundus photographs. Read More

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http://dx.doi.org/10.3928/01913913-20181017-03DOI Listing
December 2018
1 Read

[Sickle cell disease: A diagnosis to keep in mind].

Rev Chil Pediatr 2018 Aug;89(4):525-529

División de Pediatría, Pontificia Universidad Católica de Chile, Santiago, Chile.

Sickle cell disease (SCD) is an autosomal recessive genetic disorder. It is the most frequent structural hemoglobinopathy worldwide, and it is produced by an alteration in the globin chain genes. In Chile, there is no data on the prevalence of SCD since it is considered a very rare condition. Read More

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http://dx.doi.org/10.4067/S0370-41062018005000604DOI Listing

Expression of C1q in the serum of patients with non‑severe aplastic anemia, and its association with disease severity.

Mol Med Rep 2018 Dec 12. Epub 2018 Dec 12.

Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.

A type of aplastic anemia (AA), non-severe aplastic anemia (NSAA) is defined as AA that does not meet the diagnostic criteria of severe aplastic anemia (SAA). Complement component 1q (C1q) has an important role in the pathogenesis of various autoimmune diseases; however, the role of C1q in the immune pathogenesis of NSAA is not clear. The current study aimed to determine whether C1q has an important role in the pathogenesis of NSAA. Read More

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http://dx.doi.org/10.3892/mmr.2018.9754DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323203PMC
December 2018
2 Reads
1.484 Impact Factor

IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report.

Intern Med 2018 Dec 18. Epub 2018 Dec 18.

Department of Internal Medicine, Ako City Hospital, Japan.

A 79-year-old man with Sjogren's syndrome and systemic lupus erythematosus developed acute impaired consciousness and hemolytic anemia. The patient's red blood cells agglutinated spontaneously at 25-37°C. The treatment of red blood cells with 2-mercaptoethanol resulted in the loss of spontaneous agglutination. Read More

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https://www.jstage.jst.go.jp/article/internalmedicine/advpub
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http://dx.doi.org/10.2169/internalmedicine.1291-18DOI Listing
December 2018
3 Reads

Metastatic lung adenocarcinoma- associated thrombotic microangiopathy in a renal transplant recipient.

BMJ Case Rep 2018 Dec 13;11(1). Epub 2018 Dec 13.

Medical Department, Liverpool Hospital, Sydney, New South Wales, Australia.

Thrombotic microangiopathy (TMA) after renal transplantation can be a diagnostic challenge. TMA can occur with calcineurin inhibitors, allograft rejection, infection, mutations in complement regulatory proteins and autoimmunity. A 52-year-old male renal transplant recipient presented with extensive deep vein thrombosis. Read More

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http://dx.doi.org/10.1136/bcr-2018-226707DOI Listing
December 2018
1 Read

Severe refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab.

BMJ Case Rep 2018 Dec 13;11(1). Epub 2018 Dec 13.

Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK.

We report a case of severe idiopathic warm autoimmune haemolytic anaemia (wAIHA) which was initially poorly responsive to treatment with corticosteroids, intravenous immunoglobulin, mycophenolate mofetil and rituximab, and required transfusion of more than 30 units of red cells over 12 weeks. Off-label use of the terminal complement pathway inhibitor, eculizumab, led to rapid amelioration of the haemolysis, presumably by the inhibition of an intravascular component, and allowed time for slower acting immunosuppressive agent to take effect. This novel approach warrants further evaluation, given the poor prognosis of multirefractory wAIHA. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22642
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http://dx.doi.org/10.1136/bcr-2018-226429DOI Listing
December 2018
3 Reads

Rifampicin: not always an innocent drug.

BMJ Case Rep 2018 Dec 9;11(1). Epub 2018 Dec 9.

Department of Medicine and Research Laboratory of Internal Medicine, University Hospital of Larissa, Larissa, Greece.

Rifampicin has been widely used due to its broad antibacterial spectrum. Acute haemolysis is a rarely encountered complication of rifampicin. A 58-year-old woman was admitted to our department because of high-grade fever with rigors, accompanied by abdominal and lumbar pain and laboratory evidence of acute haemolysis. Read More

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http://dx.doi.org/10.1136/bcr-2018-227356DOI Listing
December 2018
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Glycosylated haemoglobin: a false sense of security.

BMJ Case Rep 2018 Dec 7;11(1). Epub 2018 Dec 7.

Department of Haematology, Doncaster and Bassetlaw Teaching Hospitals NHS Foundation Trust, Doncaster, UK.

We report the unusual case of a patient found to have a low glycosylated haemoglobin (HbA1c) despite having recently been diagnosed with diabetes mellitus type 2. The patient, who was not anaemic, with no symptoms or family history of haematological conditions, was subsequently found to have an elevated reticulocyte count, inferring increased red cell turnover as the culprit for the discordant HbA1c result. A diagnosis of hereditary spherocytosis was made based on characteristic peripheral blood film appearances and confirmed by eosin-5-maleimide binding test. Read More

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http://dx.doi.org/10.1136/bcr-2018-227668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6301642PMC
December 2018

Liver sequestration in sickle-cell disease and hepatitis.

Lancet 2018 12;392(10164):e16

Department of Transfusion Medicine and Haematology, Fondazione IRCCS Ca' Granda Ospedale, Maggiore Policlinico, Milan, Italy. Electronic address:

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http://dx.doi.org/10.1016/S0140-6736(18)32851-4DOI Listing
December 2018

[Systemic lupus erythematosus with acquired hemophilia A: a case report].

Authors:
F Yang Y S Zhou Y Jia

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1108-1111

Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. Read More

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December 2018
5 Reads

[Case series and clinical analysis of 14 cases of catastrophic antiphospholipid syndrome].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1033-1038

Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China.

Objective: Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a special subtype of antiphospholipid syndrome (APS) characterized by multiple intravascular thrombosis involving multiple organs systems or tissues simultaneously or continuously, high titer antiphospholipid antibodies and high mortality rate. This article's aims was to analyze the clinical manifestation, laboratory examination and treatment therapy of CAPS for the purpose of improving the understanding, diagnosis and treatment of the disease in clinical practice.

Methods: Retrospective analysis and descriptive statistics were applied to the clinical manifestations and laboratory findings of 14 CAPS cases from APS Shanghai Database (APS-SH) with catastrophic antiphospholipid. Read More

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December 2018

Morbidity pattern and interferon gamma level in sickle cell anemia patients with autosplenectomy.

Niger J Clin Pract 2018 Dec;21(12):1615-1621

Department of Immunology, Faculty of Basic Medical Science, College of Medicine, University of Ibadan, Ibadan, Nigeria.

Introduction: In sickle cell anemia patients (SCA), the spleen suffers multiple occlusion of its microvasculature causing ischemia and subsequently autosplenectomy. Among the functions of the spleen is the production of gamma interferon (IFN-γ) which has several immunological roles. This function could be impaired in these patients. Read More

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http://dx.doi.org/10.4103/njcp.njcp_217_18DOI Listing
December 2018
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Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H.

Pediatr Nephrol 2018 Dec 17. Epub 2018 Dec 17.

Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France.

Background: Hemolytic uremic syndrome (HUS) has been associated with a number of infectious agents. We report here the case of an infant with severe Bordetella pertussis infection who developed HUS.

Case Diagnosis/treatment: A 2-month-old preterm male was admitted for severe Bordetella pertussis infection. Read More

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http://dx.doi.org/10.1007/s00467-018-4174-1DOI Listing
December 2018
1 Read

Vitamin E deficiency in South Asian population and the therapeutic use of alpha-tocopherol (Vitamin E) for correction of anemia.

Pak J Med Sci 2018 Nov-Dec;34(6):1571-1575

Prof. Dr. Mohammad Perwaiz Iqbal, PhD. Department of Biological and Biomedical Sciences, Aga Khan University, Karachi, Pakistan.

Mild to moderate vitamin E deficiency because of inadequate consumption of vitamin E-rich foods and intestinal fat malabsorption is common in growing children, women of reproductive age and elderly South Asian population. Severe vitamin E deficiency may lead to peripheral and motor neurodegenerative diseases (e.g ataxia and motor skeletal myopathy), impaired immune response and free radical-induced hemolytic anemias. Read More

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http://pjms.com.pk/index.php/pjms/article/view/15880
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http://dx.doi.org/10.12669/pjms.346.15880DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290196PMC
December 2018
5 Reads

Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first-in-human trial.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria;

Cold agglutinin disease is a difficult-to-treat autoimmune hemolytic anemia in which immunoglobulin M antibodies bind to erythrocytes and fix complement, resulting in predominantly extravascular hemolysis. This trial tested the hypothesis that the anti-C1s antibody sutimlimab would ameliorate hemolytic anemia. Ten patients with cold agglutinin disease participated in the phase 1b component of a first-in-human trial. Read More

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http://dx.doi.org/10.1182/blood-2018-06-856930DOI Listing
December 2018
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Anemia secondary to the use of sodium valproate for preventing postoperative seizures in a 79-year-old man: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13626

Department of Geriatrics.

Rationale: Sodium valproate is a widely used antiepileptic drug and also used to prevent postoperative seizures in neurosurgery. Anemia caused by sodium valproate is occasionally reported and most are from pediatric patients.

Patient Concerns: We present the case of a 79-year-old man who developed anemia in the setting of a short-term sodium valproate therapy for the prevention of postoperative seizures. Read More

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http://dx.doi.org/10.1097/MD.0000000000013626DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320182PMC
December 2018
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Feasibility of transcatheter prosthetic paravalvular leakage closure: a single-center, exploratory safety evaluation study of transcatheter closure of prosthetic paravalvular regurgitation in Japan (STOP PARA study).

Gen Thorac Cardiovasc Surg 2018 Dec 14. Epub 2018 Dec 14.

Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Japan.

Objective: The standard treatment of prosthetic paravalvular leakage (PVL) accompanied by heart failure or hemolytic anemia is repeat open surgery. Although favorable outcomes for transcatheter prosthetic PVL closure in patients with a high surgical risk for repeat open surgery have been reported, procedural feasibility has not been examined in Japan.

Methods: From March 2015 to November 2015, transcatheter PVL closure in mitral position was performed in four patients (age range 41-78, three females) with high surgical risk due to history of repeated surgeries, chest radiation and reconstruction of the mitral annulus after debridement of abscess owing to infective endocarditis. Read More

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http://dx.doi.org/10.1007/s11748-018-1049-0DOI Listing
December 2018
6 Reads

Relapsing Evans syndrome and systemic lupus erythematosus with antiphospholipid syndrome treated with Bortezomib in combination with plasma exchange.

Clin Immunol 2018 Dec 10. Epub 2018 Dec 10.

Laboratory of the Mosaics of Autoimmunity, Saint-Petersburg University, 7/9 Universitetskaya Emb., Saint- Petersburg 199034, Russia.

Relapsing Evans syndrome (ES) and systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS) is very rare association. Coexistence of these syndromes is potentially fatal and require high-dose combined immunosuppressive therapy. We describe a case of successful use of Bortezomib and plasma exchange in a patient with ES and APS refractory to standard therapy. Read More

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http://dx.doi.org/10.1016/j.clim.2018.12.010DOI Listing
December 2018

Immune-mediated hemolytic anemia and thrombocytopenia in clonal B-cell disorders: a review.

Clin Adv Hematol Oncol 2018 Oct;16(10):670-676

Mayo Clinic, Rochester, Minnesota.

Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. Read More

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October 2018

Subclinical Cardiac Dysfunction Is Associated With Extracardiac Organ Damages.

Front Med (Lausanne) 2018 20;5:323. Epub 2018 Nov 20.

Cardiology Department, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris and UMR_S970, Université Paris Descartes, Inserm, Paris, France.

Several studies conducted in America or Europe have described major cardiac remodeling and diastolic dysfunction in patients with sickle cell disease (SCD). We aimed at assessing cardiac involvement in SCD in sub-Saharan Africa where SCD is the most prevalent. In Cameroon, Mali and Senegal, SCD patients and healthy controls of the CADRE study underwent transthoracic echocardiography if aged ≥10 years. Read More

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http://dx.doi.org/10.3389/fmed.2018.00323DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262341PMC
November 2018
1 Read