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      Abdominal pain in combination with an unexplained hemolytic anemia are crucial signs to test for paroxysmal nocturnal hemoglobinuria: A case report

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          Key Clinical Message

          Paroxysmal nocturnal hemoglobinuria ( PNH), a rare benign hematological disorder, presents with a wide variety of clinical symptoms. A direct Coombs‐negative hemolytic anemia combined with an increased LDH = Lactate dehydrogenase level are signs to test for PNH. Follow‐up does not need any microscopic review's only flow cytometric PNH clone size.

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          Thrombosis in paroxysmal nocturnal hemoglobinuria.

          Anita Hill, Peter Hillmen, Kevin Kelly (2013)
          The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the alternative pathway of complement. This may explain some of the complexity of the thrombosis in PNH. In this review, the recent changes in our understanding of the pathophysiology of thrombosis in PNH, as well as the treatment of thrombosis, will be discussed. Mechanisms explored include platelet activation, toxicity of free hemoglobin, nitric oxide depletion, absence of other glycosylphosphatidylinositol-linked proteins such as urokinase-type plasminogen activator receptor and endothelial dysfunction. Complement inhibition with eculizumab has a dramatic effect in PNH and has a major impact in the prevention of thrombosis as well as its management in this disease.
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            Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry.

            Joseph J. Illingworth, S. Richards, … (2010)
            Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by a somatic mutation in the PIGA gene, leading to a deficiency of proteins linked to the cell membrane via glycophosphatidylinositol (GPI) anchors. While flow cytometry is the method of choice for identifying cells deficient in GPI-linked proteins and is, therefore, necessary for the diagnosis of PNH, to date there has not been an attempt to standardize the methodology used to identify these cells. In this document, we present a consensus effort that describes flow cytometric procedures for detecting PNH cells. We discuss clinical indications and offer recommendations on data interpretation and reporting but mostly focus on analytical procedures important for analysis. We distinguish between routine analysis (defined as identifying an abnormal population of 1% or more) and high-sensitivity analysis (in which as few as 0.01% PNH cells are detected). Antibody panels and gating strategies necessary for both procedures are presented in detail. We discuss methods for assessing PNH populations in both white blood cells and red blood cells and the relative advantages of measuring each. We present steps needed to validate the more elaborate high-sensitivity techniques, including the need for careful titration of reagents and determination of background rates in normal populations, and discuss technical pitfalls that might affect interpretation. This document should both enable laboratories interested in beginning PNH testing to establish a valid procedure and allow experienced laboratories to improve their techniques. (c) 2010 Clinical Cytometry Society.
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              Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria.

              Charles J. Parker (2016)
              Once suspected, the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is straightforward when flow cytometric analysis of the peripheral blood reveals a population of glycosyl phosphatidylinositol anchor protein-deficient cells. But PNH is clinically heterogeneous, with some patients having a disease process characterized by florid intravascular, complement-mediated hemolysis, whereas in others, bone marrow failure dominates the clinical picture with modest or even no evidence of hemolysis observed. The clinical heterogeneity is due to the close, though incompletely understood, relationship between PNH and immune-mediated bone marrow failure, and that PNH is an acquired, nonmalignant clonal disease of the hematopoietic stem cells. Bone marrow failure complicates management of PNH because compromised erythropoiesis contributes, to a greater or lesser degree, to the anemia; in addition, the extent to which the mutant stem cell clone expands in an individual patient determines the magnitude of the hemolytic component of the disease. An understanding of the unique pathobiology of PNH in relationship both to complement physiology and immune-mediated bone marrow failure provides the basis for a systematic approach to management.
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                Author and article information

                Contributors
                N.S. Elias:
                ORCID: http://orcid.org/0000-0001-7086-7564
                n.s.elias@asz.nl
                Journal
                Journal ID (nlm-ta): Clin Case Rep
                Journal ID (iso-abbrev): Clin Case Rep
                Journal ID (doi): 10.1002/(ISSN)2050-0904
                Journal ID (publisher-id): CCR3
                Title: Clinical Case Reports
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2050-0904
                Publication date (Electronic): 04 December 2018
                Publication date Collection: January 2019
                Volume: 7
                Issue: 1 ( doiID: 10.1002/ccr3.2019.7.issue-1 )
                Pages: 175-179
                Affiliations
                [ 1 ] Department of Clinical Chemistry Albert Schweitzer Hospital Dordrecht The Netherlands
                [ 2 ] Result Laboratory Dordrecht The Netherlands
                [ 3 ] Department of Internal Medicine Albert Schweitzer Hospital Dordrecht The Netherlands
                [ 4 ] Department of Hematology Beatrix Hospital Gorinchem The Netherlands
                Author notes
                [*] [* ] Correspondence

                Naura Elias, Department of Clinical Chemistry, Albert Schweitzer Hospital, Dordrecht, The Netherlands.

                Email: n.s.elias@ 123456asz.nl

                Author information
                http://orcid.org/0000-0001-7086-7564
                Article
                Publisher ID: CCR31771
                DOI: 10.1002/ccr3.1771
                PMC ID: 6333074
                SO-VID: fb246032-b181-48d3-8d77-8dfa1301e1f6
                Copyright © © 2018 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                Date received : 22 January 2018
                Date revision received : 25 April 2018
                Date accepted : 09 June 2018
                Page count
                Figures: 2, Tables: 2, Pages: 5, Words: 2624
                Categories
                Subject: Case Report
                Subject: Case Reports
                Custom metadata
                source-schema-version-number 2.0
                component-id ccr31771
                cover-date January 2019
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_NLMPMC version:version=5.5.4 mode:remove_FC converted:15.01.2019

                Keywords: abdominal pain,coombs‐negative hemolysis,flow cytometry,hemolytic anemia,paroxysmal nocturnal hemoglobinuria
                Data availability:
                Keywords: abdominal pain, coombs‐negative hemolysis, flow cytometry, hemolytic anemia, paroxysmal nocturnal hemoglobinuria

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