Hematopoiesis and the defect in paroxysmal nocturnal hemoglobinuria.
نویسنده
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 کاملAnalysis of T cells in paroxysmal nocturnal hemoglobinuria provides direct evidence that thymic T-cell production declines with age.
Peripheral blood T cells in patients with paroxysmal nocturnal hemoglobinuria (PNH) comprise a mixture of residual normal and glycosylphosphatidylinositol (GPI)-deficient PNH cells. Using multicolor flow cytometry, we demonstrated significant differences between the proportions of naive and memory cells within these populations. PNH T cells comprise mainly naive cells (CD45RA(+)CD45R0(-)), wher...
متن کاملThe Management of Paroxysmal Nocturnal Hemoglobinuria— Recent Advances in Diagnosis and Treatment, and New Hope for Patients Pathophysiology of Paroxysmal Nocturnal Hemoglobinuria
Pathophysiology of Paroxysmal Nocturnal Hemoglobinuria Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant clonal disorder of hematopoiesis. The genetic basis is acquired somatic mutations of the X-chromosomal gene PIG-A in one or few hematopoietic stem/progenitor cells. The protein encoded by PIG-A is essential for the synthesis of the glycosylphosphatidylinositol (GPI) anchor. The GP...
متن کاملPreferential hematopoiesis by paroxysmal nocturnal hemoglobinuria clone engrafted in SCID mice.
In paroxysmal nocturnal hemoglobinuria (PNH), little is known about the molecular events leading to the clinical manifestations except for the hemolysis. To unfold the complex pathophysiology, it is necessary to elucidate the nature of the PNH clone. PNH exhibits an acquired stem cell disorder, a clonal expansion of affected cells, concomitant depression of normal hematopoiesis in bone marrow (...
متن کاملSevere telomere shortening in patients with paroxysmal nocturnal hemoglobinuria affects both GPI- and GPI+ hematopoiesis.
A most distinctive feature of paroxysmal nocturnal hemoglobinuria (PNH) is that in each patient glycosylphosphatidylinositol-negative (GPI-) and GPI+ hematopoietic stem cells (HSCs) coexist, and both contribute to hematopoiesis. Telomere size correlates inversely with the cell division history of HSCs. In 10 patients with hemolytic PNH the telomeres in sorted GPI- granulocytes were shorter than...
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ورودعنوان ژورنال:
- The Journal of clinical investigation
دوره 100 5 شماره
صفحات -
تاریخ انتشار 1997