Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal hematopoietic stem cell disorder, characterized by the deficiency of glycosylphosphatidylinositol (GPI) that anchors proteins in cell membranes. PNH is manifest variously with hemoglobinuria, thrombosis, or bone marrow failure. This retrospective study was aimed at assessing the incidence and characteristics of patients diagnosed with PNH in the King Fisal Specialist Hospital and research center.
Methods: Patients referred for PNH diagnosis at King Faisal Specialist Hospital and Research Centre, Riyadh, during the 2-year period (2012-2013) were included in the analysis. Peripheral blood samples were used for multi-parametric flow cytometry analysis based on fluorescent inactive aerolysin (FLAER), and the markers, CD235a and CD59 on red blood cells (RBCs), and CD14, CD45, CD64, CD24, and CD15 on white blood cells (WBCs) exclusively monocytes and granulocytes. Univariate analysis of the disease characteristics was performed.
Results: Of the 366 samples submitted for PNH screening, 14 were positive (4%) and 11 were evaluable. Of the 11 patients analyzed, 8 patients (73%) presented with aplastic anemia, 1 patient (9%) each with pancytopenia, Budd-Chiari syndrome, and immune thrombocytopenia purpura. All samples showed type II and III GPI-deficient clones with a median clone size of 15% (range, 0.7%-56%) in the RBCs, and 63% (range, 3.8%-100%) in WBCs (monocytes and granulocytes).
Conclusions: This study confirms the rarity of PNH and its predominant presentation as aplastic anemia or thrombosis in a Saudi Arabian population, similar to the worldwide incidence.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/cyto.b.21317 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Vδ2 T-cells response in people with Mpox infection: a three-month longitudinal assessment.
Emerg Microbes Infect
January 2025
HIV/AIDS Unit, National Institute for Infectious Diseases "Lazzaro Spallanzani" IRCCS, Rome, Italy.
The first evidence that Orthopoxvirus induced the expansion and the recall of effector innate Vδ2T-cells was described in a macaque model. Although, an engagement of αβ T-cells specific response in patients infected with human monkeypox (Mpox) was demonstrated, little is known about the role of γδ T-cells during Mpox infection. IFN-γ-producing γδ T-cells in the resistance to poxviruses may a key role in inducing a protective type 1 memory immunity.
View Article and Find Full Text PDFKW2478 and Cisplatin Synergistically Anti-colorectal Cancer by Targeting PI3K/AKT/mTOR Pathway.
Anticancer Agents Med Chem
January 2025
Laboratory Animal Center, Affiliated Hospital of Chengde Medical University, Chengde, Hebei, 067000, P.R. China.
Objective: The objective of this study is to examine the impact of KW-2478 combined with DDP on colorectal cancer cells both in vitro and in vivo and to elucidate the molecular mechanism of KW-2478 in colorectal cancer.
Methods: qRT-PCR and Western blot were employed to assess HSP90 mRNA and protein expression in normal intestinal epithelial and colorectal cancer cells. DLD-1 and HCT116 were selected for the experiment.
Fe3O4-viral-like Mesoporous Silica Nanoparticle(Fe3O4-vMSN)-Sustained Release of Lenvatinib for Targeted Treatment of Hepatocellular Carcinoma.
Curr Cancer Drug Targets
January 2025
Department of Clinical Laboratory, Gongli Hospital of Shanghai Pudong New Area, Shanghai, 200135, China.
Background: Lenvatinib is an oral tyrosine kinase inhibitor that selectively inhib-its receptors involved in tumor angiogenesis and tumor growth. It is an emerging first-line treatment agent for hepatocellular carcinoma (HCC). However, there is no intravenous ad-ministration of Lenvatinib.
View Article and Find Full Text PDFTranscriptome analysis reveals molecular targets of erythrocyte invasion phenotype diversity in natural isolates from Cameroon.
Front Parasitol
May 2024
Disease Control and Elimination (DCE), Medical Research Council The Gambia Unit at the London School of Hygiene and Tropical Medicine (LSHTM), Fajara, Gambia.
Further understanding of the molecular mediators of alternative RBC invasion phenotypes in endemic malaria parasites will support malaria blood-stage vaccine or drug development. This study investigated the prevalence of sialic acid (SA)-dependent and SA-independent RBC invasion pathways in endemic parasites from Cameroon and compared the schizont stage transcriptomes in these two groups to uncover the wider repertoire of transcriptional variation associated with the use of alternative RBC invasion pathway phenotypes. A two-color flow cytometry-based invasion-inhibition assay against RBCs treated with neuraminidase, trypsin, and chymotrypsin and deep RNA sequencing of schizont stages harvested in the first replication cycle in culture were employed in this investigation.
View Article and Find Full Text PDFInterleukin-17A facilitates tumor progression upregulating programmed death ligand-1 expression in hepatocellular carcinoma.
World J Gastrointest Oncol
January 2025
The First School of Clinical Medicine, Lanzhou University, Lanzhou 730000, Gansu Province, China.
Background: Hepatocellular carcinoma (HCC) is an inflammation-associated tumor with a dismal prognosis. Immunotherapy has become an important treatment strategy for HCC, as immunity is closely related to inflammation in the tumor microenvironment. Inflammation regulates the expression of programmed death ligand-1 (PD-L1) in the immunosuppressive tumor microenvironment and affects immunotherapy efficacy.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!