Paroxysmal Nocturnal Hemoglobinuria: Current Concepts of Certain Pathophysiologic Features.
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چکیده
منابع مشابه
Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...
متن کاملLoss of expression of neutrophil proteinase-3: a factor contributing to thrombotic risk in paroxysmal nocturnal hemoglobinuria.
BACKGROUND A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example, the absence of certain glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria cells may influence expression ...
متن کاملParoxysmal nocturnal hemoglobinuria clone in 103 Brazilian patients: diagnosis and classification
BACKGROUND Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic anemia, which often manifests as peripheral blood cytopenias and thrombosis. OBJECTIVE The aim of this study is to describe a Brazilian population of paroxysmal nocturnal hemoglobinuria patients. METHODS One hundred and three paroxysmal nocturnal hemoglobinuria cases were retrospectively reviewed and the clinica...
متن کاملگزارش یک مورد بیمار مبتلا به Paroxysmal Nocturnal Hemoglobinuria طی حاملگی
سابقه و هدف: PNH فرم نادری از آنمی همولیتیک غیر ارثی است که با همولیز حاد و مزمن داخل عروقی و هموگلوبینوری مکرر مشخص می شود و اغلب منجر به سقط و عوارض عروقی از قبیل ترمبوزهای وریدی می گردد اما برخی موارد حاملگی موفق و بدون عارضه نزد بیماران PNH مشاهده می گردد. در این راستا اقدام به معرفی یک مورد بیمار حامله مبتلا به PNH مراجعه کننده به بیمارستان رسول اکرم تهران در زمستان سال گذشته می شود. گزارش...
متن کاملDetection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats.
BACKGROUND The presence of paroxysmal nocturnal hemoglobinuria clones in the setting of aplastic anemia or myelodysplastic syndrome has been shown to have prognostic and therapeutic implications. However, the status of paroxysmal nocturnal hemoglobinuria clones in various categories of myelodysplastic syndrome and in other bone marrow disorders is not well-studied. DESIGN AND METHODS By using...
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عنوان ژورنال:
- Blood
دوره 25 شماره
صفحات -
تاریخ انتشار 1965