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Review
. 2016 Aug 5;6(2):19-27.
eCollection 2016.

Pesg PNH diagnosis, follow-up and treatment guidelines

Fahri Sahin  1 , Olga Meltem Akay  2 , Mesut Ayer  3 , Mehmet Sinan Dal  4 , Sehmus Ertop  5 , Osman Ilhan  6 , Volkan Karakus  7 , Mehmet Ali Ozcan  8 , Vildan Ozkocaman  9 , Hayri Ozsan  8 , Ozan Salim  10 , Mahmut Tobu  1 , Anil Tombak  11 , Tulin Firatli Tuglular  12 , Mehmet Yilmaz  13 , Ali Unal  14 , Mustafa Nuri Yenerel  12 , Guray Saydam  15
Affiliations
Review

Pesg PNH diagnosis, follow-up and treatment guidelines

Fahri Sahin et al. Am J Blood Res. .

Abstract

PNH Education and Study Group (PESG) have been established in December 2013 as a non-profit, independent, medical organization www.pesg.org. Paroxysmal Nocturnal Hemoglobinuria (PNH) is a multi-systemic disease that should be treated with a multidisciplinary approach. Patients may apply to the clinics other than the hematology due to variability and diversity of clinical findings which lower the rate of diagnosis due to low awareness about PNH. PNH might be overlooked and diagnosis might be delayed. Regarding these, PESG was established with the collaboration of Immunology, Cardiology, Thorax Diseases (Pulmonology), Neurology, Gastroenterology, General Surgery and Urology specialists in addition to hematologists dealing with PNH. The PESG study group aims to increase the awareness about PNH, including training activities about PNH, strengthening the relations between clinics and planning of clinical studies as a goal. It is the first professional organization focusing on PNH, in Turkey.In this guideline, we want to facilitate the diagnosis attributes of physicians from all specializations that deal with PNH and its systemic complications. One can perceive this as a tailor made guideline of international guidelines but not a compilation.

Keywords: PNH; diagnosis; guideline; treatment.

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Figures

Figure 1
Figure 1
Characteristics of Thrombosis for which PNH Clone is Recommended (Adapted from www.pesg.org).
Figure 2
Figure 2
PNH Treatment Algorithm. VTE: Venous Thromboembolism. *In case of bone marrow deficiency in non-hemolytic PNH, treatment of the underlying disease has priority. **High Disease Activity. Supportive treatment: folic acid, iron replacement and transfusion. 1Short-term steroid therapy.
Figure 3
Figure 3
Eculizumab Treatment Scheme in PNH.
See this image and copyright information in PMC

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