title: PNH is a debilitating, fatal but treatable disease: same disease, different clinical presentations.
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.
authors.affiliation: Department of Hematology, Ege University Bornova, Izmir, Turkey ; PNH Education and Study Group (PESG) Turkey.
authors.forename: Fahri
authors.initials: F
authors.lastname: Sahin
authors.affiliation: Department of Hematology, Ege University Bornova, Izmir, Turkey ; PNH Education and Study Group (PESG) Turkey.
authors.forename: Asu F
authors.initials: AF
authors.lastname: Yilmaz
authors.affiliation: Department of Hematology, Ege University Bornova, Izmir, Turkey ; PNH Education and Study Group (PESG) Turkey.
authors.forename: Melda Comert
authors.initials: MC
authors.lastname: Ozkan
authors.affiliation: PNH Education and Study Group (PESG) Turkey ; Department of Immunology, Ege University Bornova, Izmir, Turkey.
authors.forename: Nihal Mete
authors.initials: NM
authors.lastname: Gokmen
authors.affiliation: Department of Hematology, Ege University Bornova, Izmir, Turkey ; PNH Education and Study Group (PESG) Turkey.
authors.forename: Guray
authors.initials: G
authors.lastname: Saydam
keywords: Paroxysmal nocturnal hemoglobinuria (PNH); abdominal pain; eculizumab; thrombosis