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hemolytic anemias

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https://www.readbyqxmd.com/read/28922657/methods-employed-in-cytofluorometric-assessment-of-eryptosis-the-suicidal-erythrocyte-death
#1
Mohamed Jemaà, Myriam Fezai, Rosi Bissinger, Florian Lang
Suicidal erythrocyte death or eryptosis contributes to or even accounts for anemia in a wide variety of clinical conditions, such as iron deficiency, dehydration, hyperphosphatemia, vitamin D excess, chronic kidney disease (CKD), hemolytic-uremic syndrome, diabetes, hepatic failure, malignancy, arteriitis, sepsis, fever, malaria, sickle-cell disease, beta-thalassemia, Hb-C and G6PD-deficiency, Wilsons disease, as well as advanced age. Moreover, eryptosis is triggered by a myriad of xenobiotics and endogenous substances including cytotoxic drugs and uremic toxins...
September 1, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28918421/macrophage-activation-syndrome-at-the-onset-of-glucocorticoid-resistant-systemic-lupus-erythematosus-a-case-report
#2
Delia Tulbă, Marius Balea, Cristian Băicuș
INTRODUCTION: Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects. CASE REPORT: A 28-year-old Romani male was admitted to our hospital for polyarthralgia, polyserositis and fatigability...
September 16, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28906413/differences-in-clinical-features-observed-between-childhood-onset-versus-adult-onset-systemic-lupus-erythematosus-a-systematic-review-and-meta-analysis
#3
Pravesh Kumar Bundhun, Alka Kumari, Feng Huang
BACKGROUND: Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis. METHODS: Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28905389/the-first-reported-case-of-concurrent-trimethoprim-sulfamethoxazole-induced-immune-hemolytic-anemia-and-thrombocytopenia
#4
Yevgeniy A Linnik, Edison W Tsui, Isabella W Martin, Zbigniew M Szczepiorkowski, Gregory A Denomme, Jerome L Gottschall, John M Hill, Nancy M Dunbar
BACKGROUND: Drug-induced immune hemolytic anemia (DIIHA) and drug-induced immune thrombocytopenia (DIIT) are rare but dangerous complications of pharmacotherapy that may be underrecognized in hematopoietic stem cell transplant (HSCT) patients due to overlap of signs and symptoms with those of more common disease processes. CASE REPORT: A 61-year-old woman with NK-cell deficiency and GATA-2-associated myelodysplastic syndrome, status post-recent allogeneic HSCT (Day +58), presented with 3 days of acute-onset severe back pain, muscle cramps, and increasingly dark urine...
September 14, 2017: Transfusion
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#5
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
September 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28904486/hemolytic-uremic-syndrome-complicating-acute-pancreatitis
#6
Narinder Pal Singh, Neeru P Aggarwal, Hardik R Shah, Laxmi Kant Jha, Anish Kumar
Hemolytic-uremic syndrome (HUS) is characterized by acute kidney injury with hemolytic anemia and thrombocytopenia. It has diverse etiologies, clinical manifestations, and risk factors. Acute pancreatitis as a cause of HUS is rare in adults. We report a case of 32-year-old male who presented with ethanol-induced acute pancreatitis complicated with hemolytic-uremic syndrome managed with hemodialysis and plasmapheresis.
August 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28899405/neonatal-hemolytic-anemia-does-not-always-indicate-thalassemia-a-case-report
#7
Arwa A Al-Harazi, Bilguis M Al-Eryani, Butheinah A Al-Sharafi
BACKGROUND: Congenital erythropoietic porphyria is a rare autosomal recessive disorder that affects heme-porphyrin synthesis. This disorder is due to the genetic defect of uroporphyrinogen III cosynthase. This defect results in the accumulation of high amounts of uroporphyrin I in all tissues, leading to clinical manifestations ranging from mild to severe chronic damage of the skin, cartilage and bone. Hypertrichosis, erythrodontia and reddish-colored urine are often present, as well as hemolytic anemia accompanied by hepatosplenomegaly...
September 12, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28888641/a-molecular-survey-of-small-ruminant-hemotropic-mycoplasmosis-in-turkey-including-first-laboratory-confirmed-clinical-cases-caused-by-mycoplasma-ovis
#8
Munir Aktas, Sezayi Ozubek
Hemotropic mycoplasmas are emerging zoonotic pathogens that cause mild to severe hemolytic anemia. Blood samples and thin blood smears were collected from six sheep exhibiting clinical symptoms typical of hemolytic anemia. Coccoid and ring-shaped M. ovis were found on the surface of erythrocytes of infected sheep. Parasitemia ranged from 0.7% to 3.7% in individual smears. Clinical cases caused by Mycoplasma ovis were evaluated, and prevalence, based on DNA screening for the 16S rRNA gene of hemoplasmas in apparently healthy sheep (n=328) and goats (n=272) from the same area, was 11...
September 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28883277/siblings-with-congenital-thrombotic-thrombocytopenic-purpura
#9
Yasutomo Funakoshi, Masahiko Okada, Masanori Matsumoto, Koichi Kokame, Hiroyuki Moriuchi
Congenital thrombotic thrombocytopenic purpura (TTP) is a rare hereditary deficiency of ADAMTS13 (von Willebrand factor-cleaving protease) characterized by thrombocytopenia and microangiopathic hemolytic anemia. The spectrum of the clinical phenotype is wide, ranging from asymptomatic episodes of thrombocytopenia to life-threatening multiorgan failure. Reportedly, some patients develop isolated thrombocytopenia during childhood. We herein report sibling cases of congenital TTP. An 11-year-old boy with thrombocytopenia accompanied by influenza virus infection was referred to our hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28880867/oral-tetrahydrouridine-and-decitabine-for-non-cytotoxic-epigenetic-gene-regulation-in-sickle-cell-disease-a-randomized-phase-1-study
#10
RANDOMIZED CONTROLLED TRIAL
Robert Molokie, Donald Lavelle, Michel Gowhari, Michael Pacini, Lani Krauz, Johara Hassan, Vinzon Ibanez, Maria A Ruiz, Kwok Peng Ng, Philip Woost, Tomas Radivoyevitch, Daisy Pacelli, Sherry Fada, Matthew Rump, Matthew Hsieh, John F Tisdale, James Jacobberger, Mitch Phelps, James Douglas Engel, Santhosh Saraf, Lewis L Hsu, Victor Gordeuk, Joseph DeSimone, Yogen Saunthararajah
BACKGROUND: Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal hemoglobin (HbF) interferes with this polymerization, but HbF is epigenetically silenced from infancy onward by DNA methyltransferase 1 (DNMT1). METHODS AND FINDINGS: To pharmacologically re-induce HbF by DNMT1 inhibition, this first-in-human clinical trial (NCT01685515) combined 2 small molecules-decitabine to deplete DNMT1 and tetrahydrouridine (THU) to inhibit cytidine deaminase (CDA), the enzyme that otherwise rapidly deaminates/inactivates decitabine, severely limiting its half-life, tissue distribution, and oral bioavailability...
September 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28878911/severe-autoimmune-hemolytic-anemia-during-pegylated-interferon-plus-ribavirin-treatment-for-chronic-hepatitis-c-a-case-report
#11
Shasha Wang, Eryun Qin, Yixiao Zhi, Rui Hua
We report a rare case of severe autoimmune hemolytic anemia triggered by pegylated interferon during combination therapy for chronic HCV. This case demonstrated that interferon can de novo induce autoimmune hemolytic anemia during therapy for chronic hepatitis C in a previously healthy patient.
September 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28878866/association-of-autoimmune-hepatitis-type-1-in-a-child-with-evans-syndrome
#12
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab...
August 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28875001/immune-thrombocytopenic-purpura-and-hemolytic-anemia-secondary-to-hepatitis-a
#13
Ghasem Miri-Aliabad, Somayeh Rashidi
Hepatitis A is common in children and usually is a self-limiting disease. Although extrahepatic and hematological immune manifestations following acute hepatitis A virus (HAV) infection have rarely been reported, they are frequently observed in other viral hepatitis. In this paper, we report the case of a 3-year-old girl who developed immune thrombocytopenic purpura (ITP) and hemolytic anemia after HAV infection. She was presented with malaise, pallor, ecchymosis, petechiae and purpura on the trunk and extremities...
April 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/28874307/prosthetic-mitral-valve-paravalvular-leak-a-problem-that-requires-dexterity
#14
James W Hansen, Gautam Gadey, Thomas C Piemonte
Prosthetic valve paravalvular leak (PVL) is a known and relatively common complication of surgical valve replacement, which may lead to significant morbidity. Patients with significant mitral valve replacement (MVR) PVL typically present with symptoms of heart failure and elevated filling pressures or with hemolytic anemia. Percutaneous closure of these leaks has become the preferred therapy. Percutaneous closure of MVR PVL can be technically challenging, given the anatomy of the approach (trans-septal, trans-apical), the level of associated comorbidities and the geographic location of the paravalvular defect...
July 24, 2017: Cardiovascular Revascularization Medicine: Including Molecular Interventions
https://www.readbyqxmd.com/read/28872489/a-case-of-pure-red-cell-aplasia-during-nivolumab-therapy-for-cardiac-metastatic-melanoma
#15
Akihiko Yuki, Tatsuya Takenouchi, Sumiko Takatsuka, Takuro Ishiguro
Nivolumab is an antibody against programmed cell death 1 and functions as an immune checkpoint inhibitor for various malignancies, including unresectable melanomas. Nivolumab causes several immune-related adverse events, which typically include skin rash, pneumonitis, thyroid dysfunction, hepatitis, and colitis; in rare cases, anemia may be present. There are several reports of autoimmune hemolytic anemia that has developed in response to nivolumab; however, there are few reports of pure red cell aplasia (PRCA)...
September 1, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28871669/facilitated-subcutaneous-immunoglobulin-fscig-in-autoimmune-cytopenias-associated-with-common-variable-immunodeficiency
#16
Veronica Pedini, Isabella Savore, Giovanna Maria Danieli
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immune deficiency of adulthood. Besides recurrent infections, autoimmune disorders-mainly cytopenias-affect 30% of patients with CVID. OBJECTIVES: To describe the efficacy and safety of facilitated subcutaneous immunoglobulin (fSCIg), which is a combination of 10% [human] SCIg with recombinant human hyaluronidase for the treatment of CVID-linked cytopenias. METHODS: We describe four women (mean age 54 years) with CVID associated with idiopathic thrombocytopenic purpura (ITP) (n=3) and autoimmune hemolytic anemia (AIHA) (n=1)...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28870408/thyroid-storm-and-warm-autoimmune-hemolytic-anemia
#17
Joseph A Moore, Louise Gliga, Srikanth Nagalla
Graves' disease is often associated with other autoimmune disorders, including rare associations with autoimmune hemolytic anemia (AIHA). We describe a unique presentation of thyroid storm and warm AIHA diagnosed concurrently in a young female with hyperthyroidism. The patient presented with nausea, vomiting, diarrhea and altered mental status. Laboratory studies revealed hemoglobin 3.9g/dL, platelets 171×10(9)L(-1), haptoglobin <5mg/dL, reticulocytosis, and positive direct antiglobulin test (IgG, C3d, warm)...
August 18, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28864783/mesenchymal-stromal-cells%C3%A2-can-be-applied-in-red-blood-cells%C3%A2-storage-as-one-kind-of-cellular-additives
#18
Yaozhen Chen, Jing Zhang, Shunli Gu, Dandan Yin, Qunxing An, Ning An, Lihong Weng, Jing Yi, Jinmei Xu, Wen Yin, Xingbin Hu
During storage in blood banks, red blood cells (RBCs) undergo the mechanical and metabolic damage, which may lead to the diminished capacity to deliver oxygen. At high altitude region, the mentioned damage may get worse. Thus, more attentions should be paid to preserve RBCs when these components need transfer from plain to plateau region. Recently, we found that mesenchymal stromal cells (MSCs) could rescue anemia, and MSCs have been demonstrated in hematopoietic stem cells (HSCs) transplantation to reconstitute hematopoiesis in vivo  by us...
September 1, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28858843/using-plasma-exchange-to-successfully-manage-thyrotoxicosis-in-a-patient-with-possible-antithyroid-drug-related-thrombotic-thrombocytopenic-purpura
#19
G Tazegul, T S Ogut, H Bozoglan, O Dogan, N Yilmaz, T Ulas, O Salim, R Sari, H A Altunbas, M K Balci
OBJECTIVE: Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal insufficiency. The association or co-existence of thyrotoxicosis or antithyroid drugs with TTP has not been previously reported. Subject and Results. Herein, we present a 54-year-old female patient newly diagnosed with toxic multinodular goiter accompanying with TTP, possibly triggered by either thyrotoxicosis or antithyroid drugs...
July 1, 2017: Endocrine Regulations
https://www.readbyqxmd.com/read/28838521/aortic-valve-replacement-as-a-trigger-of-atypical-hemolytic-uremic-syndrome
#20
Konstantinos Markakis, Timm H Westhoff, Nikolaos Pagonas
Mechanical hemolysis is a frequent but usually harmless complication of aortic valve replacement. The most common reason is valvular leakage. This report presents atypical hemolytic uremic syndrome (aHUS) as an alternative cause of mechanical hemolysis after this procedure. aHUS is a complement-mediated disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. It necessitates immediate specific treatment including plasma exchange or complement inhibition to avoid an adverse outcome...
September 2017: Annals of Thoracic Surgery
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