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6,046 results
  • Pediatric Paroxysmal Nocturnal Hemoglobinuria Presenting as Acute Kidney Injury. [Journal Article]
    J Pediatr Hematol Oncol. 2020 Jun 02 [Online ahead of print]Henderson C, Lo M, Massey G
  • Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterized by variable and diverse symptoms including the classic triad of hemolytic anemia, thrombosis, and bone marrow failure. It is a disorder primarily seen in the adult population. The authors report a unique case of an 8-year-old girl diagnosed with PNH after initially presenting with a febrile illness and acute kidney injury. …
  • Terminal complement inhibition dampens the inflammation during COVID-19. [Letter]
    Br J Haematol. 2020 Jun 04 [Online ahead of print]Kulasekararaj AG, Lazana I, … Marsh JC
  • Emerging evidence suggests that activation of the complement system is critical in the pathogenesis of the novel coronavirus, SARS-CoV-2, the causative agent of COVID-19 related lung injury. Inhibition of the terminal complement pathway by targeting complement protein 5 (C5) may be an effective therapeutic intervention in CoV-mediated disease.1 Paroxysmal nocturnal haemoglobinuria (PNH) is a rare…
  • Blood Cell Disorders and the Nervous System. [Journal Article]
    Continuum (Minneap Minn). 2020 Jun; 26(3):659-674.Merkler AE
  • CONCLUSIONS: This article discusses sickle cell disease and the most recent advances in stroke preventive therapy as well as neurologic complications of paroxysmal nocturnal hemoglobinuria, immune thrombocytopenia, thrombotic thrombocytopenic purpura, polycythemia vera, and essential thrombocythemia.
  • Roles of immune responses in the pathogenesis of immunorelated pancytopenia. [Review]
    Scand J Immunol. 2020 May 31 [Online ahead of print]Xiao N, Hao S, … Shao Z
  • Some patients with pancytopenia do not conform to any diagnostic criteria of known hematological or nonhematological diseases; however, they respond well to corticosteroid, high-dose intravenous immunoglobulin and rituximab treatment. This abnormality is termed immunorelated pancytopenia (IRP). Later studies indicated that IRP might be a kind of autoimmune disease in which T helper (Th) type 2 ce…
  • Acute fulminant hemolysis after transcatheter mitral valve replacement for mitral annular calcification. [Case Reports]
    Catheter Cardiovasc Interv. 2020 May 06 [Online ahead of print]El-Sabawi B, Guerrero ME, … Rihal CS
  • Transcatheter mitral valve replacement (TMVR) is emerging as an alternative treatment strategy to surgery for patients with severe mitral annular calcification (MAC) who are not candidates for traditional mitral valve surgery. Paravalvular leak (PVL) is common following TMVR for severe MAC and can lead to heart failure symptoms and/or intravascular hemolysis, the latter of which usually is clinic…
  • Concomitant Existence of Paroxysmal Nocturnal Hemoglobinuria in a Patient with Hb E (HBB: c.79G>A) Trait. [Journal Article]
    Hemoglobin. 2020 Apr 30 [Online ahead of print]Halder R, Sundaram UKD, … Pati HP
  • Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder that manifests with bone marrow failure, thrombosis and hemolysis. We present a 28-year-old male who presented with weakness, jaundice and transfusion dependence. On initial investigation, he was found to have anemia with jaundice with hemoglobin (Hb) capillary zone electrophoresis suggestive of Hb E (HBB: c.79G>A) trait. Th…
  • Tangential flow filtration of haptoglobin. [Journal Article]
    Biotechnol Prog. 2020 Apr 29 [Online ahead of print]Pires IS, Palmer AF
  • Haptoglobin (Hp) is a plasma glycoprotein that scavenges cell-free hemoglobin (Hb). Hp has various potential therapeutic applications, but it has been mainly studied for treatment of acute hemolytic conditions that can arise from situations such as massive blood transfusion, infusion of stored red blood cells, severe burns, trauma, sepsis, radiation injury, and others. Therefore, Hp may also be b…
  • Complementopathies and precision medicine. [Review]
    J Clin Invest. 2020 May 01; 130(5):2152-2163.Gavriilaki E, Brodsky RA
  • The renaissance of complement diagnostics and therapeutics has introduced precision medicine into a widened field of complement-mediated diseases. In particular, complement-mediated diseases (or complementopathies) with ongoing or published clinical trials of complement inhibitors include paroxysmal nocturnal hemoglobinuria, cold agglutinin disease, hemolytic uremic syndrome, nephropathies, HELLP…
  • Coombs-positive Paroxysmal Nocturnal Haemoglobinuria. [Case Reports]
    Oxf Med Case Reports. 2020 Mar; 2020(3):omz125.Kalam S, Beale R, … Srirangalingam U
  • Autoimmune haemolytic anaemia (AIHA) and paroxysmal nocturnal haemoglobinuria (PNH) are two distinct causes of haemolytic anaemia. They have different mechanisms that underpin their pathogenesis and, therefore, require different treatment strategies. The direct antiglobulin test (DAT) or Coombs test is positive in cases of immune-mediated haemolytic anaemia and, thus, is positive in AIHA but nega…
  • Podocyte-targeted Heme Oxygenase (HO)-1 overexpression exacerbates age-related pathology in the rat kidney. [Journal Article]
    Sci Rep. 2020 Mar 31; 10(1):5719.Poulaki E, Detsika MG, … Gakiopoulou H
  • Although Heme Oxygenase-1 (HO-1) induction in various forms of kidney injury is protective, its role in age-related renal pathology is unknown. In the ageing kidney there is nephron loss and lesions of focal glomerulosclerosis, interstitial fibrosis, tubular atrophy and arteriolosclerosis. Underlying mechanisms include podocyte (visceral glomerular epithelial cell/GEC) injury. To assess whether H…
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