Literature DB >> 26171282

PNH is a debilitating, fatal but treatable disease: same disease, different clinical presentations.

Fahri Sahin1, Asu F Yilmaz1, Melda Comert Ozkan1, Nihal Mete Gokmen2, Guray Saydam1.   

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease characterized by chronic persistent hemolysis, multi-organ damage and eventually multiple organ failure. PNH develops as a result of increased sensitivity to complement due to an acquired deficiency of certain glycosylphosphatidylinositol (GPI)-linked proteins. The clinical presentation of PNH varies greatly from one patient to another. We present three cases of PNH with different clinical presentations to illustrate the debilitating nature of the disease, possible fatal outcomes, and the need to timely diagnosis and targeted therapy. These cases also underline the need for increased awareness of PNH among relevant healthcare specialties. PNH should be considered as a differential diagnosis in patients with unexplained abdominal pain, dyspnea, renal failure, thrombosis and non-immune hemolytic anemia.

Entities:  

Keywords:  Paroxysmal nocturnal hemoglobinuria (PNH); abdominal pain; eculizumab; thrombosis

Year:  2015        PMID: 26171282      PMCID: PMC4497495     

Source DB:  PubMed          Journal:  Am J Blood Res        ISSN: 2160-1992


  13 in total

1.  Clinical features and prognostic factors of Asian patients with paroxysmal nocturnal hemoglobinuria: results from a single center in China.

Authors:  Meili Ge; Xingxin Li; Jun Shi; Yingqi Shao; Yizhou Zheng
Journal:  Ann Hematol       Date:  2012-01-28       Impact factor: 3.673

2.  Inhibition of complement activity by humanized anti-C5 antibody and single-chain Fv.

Authors:  T C Thomas; S A Rollins; R P Rother; M A Giannoni; S L Hartman; E A Elliott; S H Nye; L A Matis; S P Squinto; M J Evans
Journal:  Mol Immunol       Date:  1996-12       Impact factor: 4.407

Review 3.  Paroxysmal nocturnal hemoglobinuria as a molecular disease.

Authors:  W F Rosse
Journal:  Medicine (Baltimore)       Date:  1997-03       Impact factor: 1.889

4.  Eculizumab for paroxysmal nocturnal haemoglobinuria.

Authors:  Charles Parker
Journal:  Lancet       Date:  2009-01-12       Impact factor: 79.321

Review 5.  Thrombosis in paroxysmal nocturnal hemoglobinuria.

Authors:  Anita Hill; Richard J Kelly; Peter Hillmen
Journal:  Blood       Date:  2013-04-22       Impact factor: 22.113

6.  Paroxysmal nocturnal hemoglobinuria: pathophysiology, natural history and treatment options in the era of biological agents.

Authors:  Antonio M Risitano; Bruno Rotoli
Journal:  Biologics       Date:  2008-06

7.  Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories.

Authors:  Régis Peffault de Latour; Jean Yves Mary; Célia Salanoubat; Louis Terriou; Gabriel Etienne; Mohamad Mohty; Sophie Roth; Sophie de Guibert; Sebastien Maury; Jean Yves Cahn; Gerard Socié
Journal:  Blood       Date:  2008-06-05       Impact factor: 22.113

8.  Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry.

Authors:  Jong Wook Lee; Jun Ho Jang; Jin Seok Kim; Sung-Soo Yoon; Je-Hwan Lee; Yeo-Kyeoung Kim; Deog-Yeon Jo; Jooseop Chung; Sang Kyun Sohn
Journal:  Int J Hematol       Date:  2013-05-01       Impact factor: 2.490

9.  Natural history of paroxysmal nocturnal hemoglobinuria.

Authors:  P Hillmen; S M Lewis; M Bessler; L Luzzatto; J V Dacie
Journal:  N Engl J Med       Date:  1995-11-09       Impact factor: 91.245

10.  Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria.

Authors:  Robert A Brodsky; Neal S Young; Elisabetta Antonioli; Antonio M Risitano; Hubert Schrezenmeier; Jörg Schubert; Anna Gaya; Luke Coyle; Carlos de Castro; Chieh-Lin Fu; Jaroslaw P Maciejewski; Monica Bessler; Henk-André Kroon; Russell P Rother; Peter Hillmen
Journal:  Blood       Date:  2007-11-30       Impact factor: 22.113

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  2 in total

1.  Paroxysmal Nocturnal Hemoglobinuria in the Differential Diagnosis of Thrombocytopenia.

Authors:  Fusun Gediz; Bahriye Kadriye Payzin; Ozlem Zekiye Cakmak; Yusuf Uzum; Damla Ernur; Fahri Sahin
Journal:  Hematol Rep       Date:  2017-03-22

2.  Modeling the activation of the alternative complement pathway and its effects on hemolysis in health and disease.

Authors:  Antonello Caruso; Jannik Vollmer; Matthias Machacek; Elod Kortvely
Journal:  PLoS Comput Biol       Date:  2020-10-02       Impact factor: 4.475

  2 in total

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