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      Detection of paroxysmal nocturnal haemoglobinuria clones in cases of deep vein thrombosis in a tertiary care centre, western Rajasthan

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          Abstract

          Background & objectives:

          Paroxysmal nocturnal haemoglobinuria is a rare acquired disease characterized by bone marrow failure, intravascular haemolysis and thrombophilia. Thrombosis is the deadliest complication of paroxysmal nocturnal haemoglobinuria (PNH). The present study was conducted to study the prevalence of PNH in cases of deep vein thrombosis (DVT) which was previously undocumented from western Rajasthan.

          Methods:

          In the present cross-sectional study, 61 adult patients with DVT were tested using flow cytometry to detect PNH clones. Blood samples were processed using fluorescein-labelled proaerolysin, CD14, CD24, CD33 and CD45 panels for granulocytes and monocytes and CD59 and CD235a panel for red blood cells.

          Results:

          Three cases (4.92%) having large clones on monocytes as well as granulocytes, which fulfilled the diagnostic criteria of PNH were detected. Further, three cases (4.92%) showed small clones on both granulocytes and monocytes. Nine (15%) cases showed small clones only on granulocytes, and 11 (18%) cases showed small clones only on monocytes.

          Interpretation & conclusions:

          The results of the present study suggest that a higher proportion of patients had PNH in western Rajasthan compared to previously reported studies from elsewhere. It is suggested that PNH testing should be added to the procoagulant work-up panel in institutions of this region where it is not routinely done. This provides an otherwise missed opportunity to diagnose this disorder. Eculizumab may be employed, which is effective in reducing thrombophilic events in cases of PNH.

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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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            Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria.

            Ashley N Young, Monica Bessler, Hubert Schrezenmeier … (2007)
            Hemolysis and hemoglobinemia contribute to serious clinical sequelae in hemolytic disorders. In paroxysmal nocturnal hemoglobinuria (PNH) patients, hemolysis can contribute to thromboembolism (TE), the most feared complication in PNH, and the leading cause of disease-related deaths. We evaluated whether long-term treatment with the complement inhibitor eculizumab reduces the rate of TE in patients with PNH. Clinical trial participants included all patients in the 3 eculizumab PNH clinical studies, which recruited patients between 2002 and 2005 (n = 195); patients from these studies continued treatment in the current multinational open-label extension study. Thromboembolism rate with eculizumab treatment was compared with the pretreatment rate in the same patients. The TE event rate with eculizumab treatment was 1.07 events/100 patient-years compared with 7.37 events/100 patient-years (P < .001) prior to eculizumab treatment (relative reduction, 85%; absolute reduction, 6.3 TE events/100 patient-years). With equalization of the duration of exposure before and during treatment for each patient, TE events were reduced from 39 events before eculizumab to 3 events during eculizumab (P < .001). The TE event rate in antithrombotic-treated patients (n = 103) was reduced from 10.61 to 0.62 events/100 patient-years with eculizumab treatment (P < .001). These results show that eculizumab treatment reduces the risk of clinical thromboembolism in patients with PNH. This study is registered at http://clinicaltrials.gov (study ID no. NCT00122317).
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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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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Med Res
                Journal ID (iso-abbrev): Indian J Med Res
                Journal ID (publisher-id): IJMR
                Journal ID (publisher-id): Indian J Med Res
                Title: The Indian Journal of Medical Research
                Publisher: Wolters Kluwer - Medknow (India )
                ISSN (Print): 0971-5916
                ISSN (Electronic): 0975-9174
                Publication date (Print): September 2022
                Volume: 156
                Issue: 3
                Pages: 543-548
                Affiliations
                [1 ] Department of General Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
                [2 ] Department of Pathology & Laboratory Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
                Author notes
                For correspondence: Dr Abhishek Purohit, Department of Pathology & Laboratory Medicine, All India Institute of Medical Sciences, Basni Industrial Area Phase-II, Jodhpur 342 005, Rajasthan, India e-mail: purohitabhi80@ 123456gmail.com
                Article
                Publisher ID: IJMR-156-543
                DOI: 10.4103/ijmr.ijmr_2584_21
                PMC ID: 10101359
                PubMed ID: 36751751
                SO-VID: c84299f0-65bb-4b6e-93ae-9b1f68aed4f2
                Copyright © Copyright: © 2022 Indian Journal of Medical Research
                License:

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Date received : 23 August 2021
                Categories
                Subject: Practice: Student IJMR

                ScienceOpen disciplines: Medicine
                Keywords: deep vein thrombosis,flow cytometry,fluorescein-labelled proaerolysin,paroxysmal nocturnal haemoglobinuria,thrombophilia,thrombosis
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: deep vein thrombosis, flow cytometry, fluorescein-labelled proaerolysin, paroxysmal nocturnal haemoglobinuria, thrombophilia, thrombosis

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