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Abstract

Research in Pediatrics & Neonatology

A Rare Cause of Cytopenias in Childhood: Paroxysmal Nocturnal Hemoglobinuria

  • Open or CloseBaris Yilmaz*

    Division of Pediatric Hematology and Oncology, Turkey

    *Corresponding author:Baris Yilmaz, Division of Pediatric Hematology and Oncology, Education Hospital, SB Marmara University Pendik Research, Istanbul, Turkey

Submission: November 15, 2018;Published: January 22, 2019

DOI: 10.31031/RPN.2019.02.000559

ISSN: 2576-9200
Volume3 Issue2

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolysis, thrombosis and impaired bone marrow functions. PNH is rarely seen in childhood and usually presents with hypoplastic/aplastic anemia clinic. If the major clinical finding is hemolysis, eculizumab (monoclonal anti-C5 immunoglobulin) can be used for treatment. The other treatment options for PNH are immunosuppressive therapy and stem cell transplantation (SCT). Fluorescent aerolysin (FLAER)-based assay is a high sensitivity test for PNH diagnosis in children. FLAER has become the gold standard for the diagnosis and follow-up of PNH get today.

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