Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Recent Advances
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چکیده
منابع مشابه
The 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 کاملReview: Laboratory Diagnosis of Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon acquired stem cell disorder associated with periodic hemolytic events. This benign clonal disease is caused by abnormalities of the Xlinked phosphatidylinositol glycan class A (PIGA) gene and is associated with cytopenias and thrombosis. Although the trilineage of bone marrow elements is affected, involvement of the red blood cell (RBC) l...
متن کاملParoxysmal Nocturnal Hemoglobinuria (PNH) — Symptoms and Diagnosis
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired defect in the myeloid stem cell lineage and can be seen as a rare, chronic, morbid disorder. Formally known as Marchiafava-Micheli syndrome, it received its current name as a descriptive term for the disease. Individuals afflicted with the disease wake up to dark, “cola” colored urine due to RBC breakdown and release of hemoglobin in the ...
متن کاملParoxysmal Nocturnal Hemoglobinuria (PNH) — Symptoms and Diagnosis
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired defect in the myeloid stem cell lineage and can be seen as a rare, chronic, morbid disorder. Formally known as Marchiafava-Micheli syndrome, it received its current name as a descriptive term for the disease. Individuals afflicted with the disease wake up to dark, “cola” colored urine due to RBC breakdown and release of hemoglobin in the ...
متن کاملParoxysmal Nocturnal Hemoglobinuria (PNH) — Symptoms and Diagnosis
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired defect in the myeloid stem cell lineage and can be seen as a rare, chronic, morbid disorder. Formally known as Marchiafava-Micheli syndrome, it received its current name as a descriptive term for the disease. Individuals afflicted with the disease wake up to dark, “cola” colored urine due to RBC breakdown and release of hemoglobin in the ...
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ژورنال
عنوان ژورنال: Indian Journal of Hematology and Blood Transfusion
سال: 2017
ISSN: 0971-4502,0974-0449
DOI: 10.1007/s12288-017-0868-y