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2016, Number 620

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Rev Med Cos Cen 2016; 73 (620)

Hemoglobinuria paroxística nocturna

García RVA, Mora HGA
Full text How to cite this article

Language: Spanish
References: 22
Page: 659-664
PDF size: 186.82 Kb.


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ABSTRACT

Paroxysmal nocturnal hemoglobinuria is a rare acquired chronic hemolytic anemia, which affects both sexes with equal frequency. It occurs at any age and more frequently in Southeast Asian countries. It is the result of non malignant clonal expansion of hematopoietic progenitor cells. It is characterized by intravascular hemolytic anemia, recurrent thrombosis and a variable component of bone marrow failure. It is associated with other hematologic disorders such as aplastic anemia and myelodysplastic syndrome. Flow cytometry is the method of choice for diagnosis. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies.


REFERENCES

  1. Arruda M, Rodrigues C, Yamamoto M, Figueiredo M. Hemoglobinúria paroxística noturna: da fisiopatologiaa o tratamento. Rev Assoc Med Bras 2010; 56(2): 214- 21.

  2. Brodsky R. Narrative review: Paroxysmal nocturnal hemoglobinuria: the physiology of complement-related hemolytic anemia. Ann.Intern.Med. 2008; 148:587–595.

  3. Brodsky R . Paroxysmal nocturnal hemoglobinuria. Blood 2014; 124:2804.

  4. Brodsky R, Young N, Antonioli E, Risitano AM, Schrezenmeier H, Schubert J, et al. Multicenter phase 3 study of the complement inhibitor eculizumab for thetreatment of patients with paroxysmal nocturnal hemoglobinuria. Blood 2008; 111:1840–7.

  5. Brodsky R. How I treat paroxysmal nocturnal hemoglobinuria. Blood 2009;113:6522–7.

  6. DeZern A, Dorr D, Brodsky R. Predictors of hemoglobin response to eculizumab therapy in paroxysmal nocturnal hemoglobinuria. Eur J Haematol. 2013;90(1):16-24.

  7. Hernández P, Almeida J, Orfao A. Hemoglobinuria paroxística nocturna. MedClin (Barc) 2008;131:617–30.

  8. Hernández P, Almeida J, Acevedo M, Sánchez M, Alberca I, Vidriales B, et al. Detailed immunophenotypic characterization of different major and minor subsets of peripheral blood cells in patients with paroxysmal nocturnal hemoglobinuria. Transfusion 2008;48:1403–14.

  9. Hill A, Kelly R, Hillmen P. Thrombosis in paroxysmal nocturnal hemoglobinuria. Blood. 2013;121(25):4985-4996.

  10. Hill A, Sapsford R, Scally A, et al. Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function. Br J Haematol. 2012; 158(3):409-414.

  11. Hillmen P, Elebute M, Kelly R, et al. Long-termeffect of the complement inhibitor eculizumab on kidney function in patients with paroxysmal nocturnal hemoglobinuria. Am J Hematol. 2010; 85(8):553-559.

  12. Hillmen P, Muus P, Röth A, et al. Long-term safety and efficacy of sustain edeculizumab treatment in patients with paroxismal nocturnal haemoglobinuria. Br J Haematol. 2013;162(1): 62-73.

  13. Hillmen P, Young N, Schubert J, Brodsky A, Socié G, Muus P, et al. The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med 2006;355:1233–43.

  14. Hoekstra, J. Et al. Paroxysmal nocturnal hemoglobinuria in Budd- Chiari Syndrome: Findings from a cohort study. J Hepatol. 2009 Oct;51(4):696-706.

  15. Moyo V, Mukhina G, Garrett E, Brodsky R. Natural history of paroxismal nocturnal haemoglobinuria using modern diagnostic assays. Br J Haematol. 2004;126(1):133-138.

  16. Parker C, Omine M, Richards S, et al. International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106(12):3699-3709.

  17. Risitano A. Paroxysmal nocturnal hemoglobinuria and other complement-mediated hematological disorders. Immunobiology 217 (2012) 1080– 1087.

  18. Rother R, Bell L, Hillmen P, Gladwin M. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005; 293(13):1653-1662.

  19. Sánchez E, et al. Hemoglobinuria paroxística nocturna. Novedades patogénicas y terapéuticas. An. Med. Interna (Madrid) Vol. 18, N.º 8, pp. 435-439, 2001.

  20. Urbano A, et al. Diagnóstico y tratamiento de la hemoglobinuria paroxística nocturna MedClin (Barc). 2011;136(3):121–127.

  21. van Bijnen S, Østerud B, Barteling W, Verbeek K, Willemsen M, van Heerde W, Muus P. Alterations in markers of coagulation and fibrinolysis in patients with Paroxysmal Nocturnal Hemoglobinuria before and during treatment with eculizumab. Thromb Res. 2015 Aug;136(2):274-81.

  22. Vega S, et al. Estudio ultraestructural de las plaquetas de pacientes con hemoglobinuria paroxística nocturna. Rev Invest Clin 2006; 58 (5): 475-486.




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Rev Med Cos Cen. 2016;73