28 July 2003
Med Sci Monit 2003; 9(7): RA161-172 :: ID: 13086
Paroxysmal nocturnal hemoglobinuria (PNH) is a unique clonal stem cell disorder characterized by intravascular hemolysis, thrombotic events and bone marrow failure. There has been accelerated progress in understanding the mechanisms underlying the clinical features of the disease over the last decade. The development of PNH requires not only a somatic mutation of the phospatidylinositol glycan complementation class A (PIG-A) gene, but also a survival advantage of the PNH clone (‘dual pathogenesis’ theory). There is increasing evidence that negative selection against the non-mutated cells rather than positive selection of the PIG-A gene mutant cells is responsible for the dominance of the PNH clone. In this review, we summarize the important advances in the understanding of PNH, but we also concentrate on the presence of PNH clones in other hematological disorders, including aplastic anemia (AA), myelodysplastic syndromes (MDS), acute leukemias, and myeloproliferative and lymphoproliferative syndromes. The fuller comprehension of the pathophysiology of PNH may have wider implications than for PNH itself, as indicated by the presence of PNH clones in these hematological malignancies, and by the therapeutic implications of this fact, as already described in patients with AA and MDS.
Keywords: Glycosylphosphatidylinositols - metabolism
01 May 2022 : EditorialEditorial: Cardiovascular Complications at One Year After SARS-CoV-2 Infection are Independent of Underlying Cardiovascular Risk Factors or Severity of COVID-19
Med Sci Monit 2022; 28:e937048
04 May 2022 : Clinical ResearchEffects of Wearing Face Masks on Exercise Capacity and Ventilatory Anaerobic Threshold in Healthy Subjects ...
Med Sci Monit In Press; DOI: 10.12659/MSM.936069
22 April 2022 : Clinical ResearchFactors Associated with Falls During Hospitalization for Coronavirus Disease 2019 (COVID-19)
Med Sci Monit In Press; DOI: 10.12659/MSM.936547
24 May 2022 : Clinical ResearchRemimazolam Tosilate Sedation with Adjuvant Sufentanil in Chinese Patients with Liver Cirrhosis Undergoing ...
Med Sci Monit In Press; DOI: 10.12659/MSM.936580
23 May 2022 : Clinical ResearchFully Automatic Knee Joint Segmentation and Quantitative Analysis for Osteoarthritis from Magnetic Resonanc...
Med Sci Monit In Press; DOI: 10.12659/MSM.936733
Most Viewed Current Articles
30 Dec 2021 : Clinical ResearchRetrospective Study of Outcomes and Hospitalization Rates of Patients in Italy with a Confirmed Diagnosis o...
Med Sci Monit 2021; 27:e935379
08 Mar 2022 : Review articleA Review of the Potential Roles of Antioxidant and Anti-Inflammatory Pharmacological Approaches for the Man...
Med Sci Monit 2022; 28:e936292
01 Nov 2020 : Review articleLong-Term Respiratory and Neurological Sequelae of COVID-19
Med Sci Monit 2020; 26:e928996