Hemofagositik sendrom tanısı alan hastalarda mortalite belirteçlerinin araştırılması
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Dosyalar
Tarih
2020
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Ege Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
GİRİŞ: Hemofagositik lenfohistiyositoz (HLH), enfeksiyonlar, bağ doku hastalıkları ve maligniteler gibi çeşitli immun aktivasyon durumlarında immun regulasyon kaybı sonucunda ortaya çıkan, aşırı inflammasyon yanıtına bağlı çoklu organ tutulumu ile seyreden klinik bulgular bütünüdür. HLH, tanısının ya da uygun tedavisinin gecikmesi yüksek mortalite riskine sahip bir durumdur. Bununla birlikte, mortalite riski daha yüksek olan durumlarda daha agresif tedavi ve yakın takip uygulanması da dramatik iyileşme sağlamaktadır. AMAÇ VE YÖNTEM: Klinik pratikte sıklıkla kullanılan laboratuvar ve radyolojik tetkikler dahilinde mortalite belirteçlerini belirlemeyi amaçladık. Bu amaçla HLH-2004 tanı kriterlerini sağlayan 26'sı kadın ve 37'si erkek olmak üzere toplam 63 HLH tanılı olguyu değerlendirdik. Olguların demografik özellikleri belirlendi, yoğun bakım ünitesine yatırıldığında ve primer sonlanım (eksitus ya da sağkalım) zamanında periferik kan örnekleri (düzeylerindeki zamansal değişimi hesaplamak için), teleradyografileri ve kontrendikasyon bulunmadığı takdirde kemik iliği biyopsileri edinildi. Kategorik değişkenler Chi-Square testi ve sayısal değişkenler Mann-Whitney testi ile analiz edildi. Elde edilen veriler üzerinden mortalite öngörücülerinin belirlenmesi amacıyla lojistik regresyon analizi uygulandı. Ayrıca mortalite öngörücüsü olarak en doğru referans H skorunu (HScore) belirlemek amacıyla ROC (receiver operating characteristic) analizi yapıldı. BULGULAR: Çalışmamız sonucunda mortalite oranı %33,3 bulundu. İstatistiksel analizler sonucunda; serum C-reaktif protein (CRP), albumin, kreatinin, üre, ferritin, N-terminal pro-brain natiruretik peptit (NTpBNP), platelet sayısı, lenfosit sayısı, nötrofil/lenfosit oranı (NLR) ve ortalama platelet hacmi (MPV) düzeylerindeki zamansal değişim anlamlı mortalite ön görücüsü olarak gösterildi. Başvuru zamanındaki trombosit sayısı, sonlanım zamanındaki laktat dehidrogenaz (LDH) ve kardiyotorasik oran (KTO) değişiminin de mortalite öngörücüsü olduğu bulundu. Bunlara ek olarak, HLH populasyonunda H skorunun anlamlı mortalite belirteci olduğu sonucuna ulaşıldı. SONUÇ: HLH yüksek mortalite ile seyreden bir immun hiperaktivasyon durumu olup, hızlıca uygun tedaviye başlaması mortalitenin önemli bir belirleyicisidir. Bu olguların, klinik pratikte sıklıkla kullanılan, kolay ulaşılabilir, ucuz ve güvenilir laboratuvar ve radyolojik bulgular ile takibi mümkündür. Klinik bulguların tamamının her zaman bir arada bulunmayacağı; bu nedenle farklı bulgular bir arada olsa da klinik şüphenin en değerli yönlendirici olduğu unutulmamalıdır.
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH), is a multi-organ involved complex of clinical findings, which are results of hyper inflammatory response due to loss of immune regulation in case of various immune activation states such as infections, connective tissue diseases and malignancies. HLH has high mortality risk if diagnose or appropriate treatment is delayed. However, dramatic improvement is seen in situations with high mortality risk if we are aware of mortality predictors; and if aggressive treatment and close monitoring are applied. AIMS AND METHOD: We purposed that to determine mortality predictors by the way of laboratory and radiologic tests which are used frequently in clinical practice. With this purpose, we evaluate totally 63 case (26 woman and 37 man) who are diagnosed as HLH due to HLH-2004 diagnostic criteria and followed in the Ege University Hospital Internal Medicine Intesive Care Unit. Demographic characteristics are determined; peripheric blood samples (to calculate timely change in levels), teleradiographs are obtained at the time of hospitalization and at the primary end points (exitus or survived). Also bone marrow biopsies are obtained if there isn’t any contraindication. Categorical variables were analyzed by Chi-Square test and numerical variables were analyzed by Mann-Whitney Test. To determine the mortality predictors, logistic regression analysis were also performed. Moreover, ROC (receiver operating characteristic) analyze is performed to detect the best cut-off value for H score as a mortality predictor. RESULTS: Mortality ratio is found as 33,3% at the end of the study. Through the statistical analyzes, timely change of plasma CRP, albumin, creatinine, urea, ferritin, N-terminal pro-brain natriuretic peptide (NTpBNP), platelet count, lymphocyte count, neutrophil/lymphocyte raito (NLR) and mean platelet volume (MPV) are showed as mortality predictors. Also platelet counts at the time of hospitalization and lactate dehydrogenase (LDH) and cardiothoracic index (CTI) at the time of primary end point are found as mortality predictors. In addition these findings, H score is also found as a mortality predictor in the HLH population. CONCLUSION: HLH, is a immune hyperactivation state, which carries high mortality rates. Beginning to therapy immediately is an important predictor for mortality. It is possible that following these cases with easily reached, cheap and reliable laboratory and radiologic tests. It should be always remembered that, all these clinical findings may not arise in all patients and clinical suspicion is the most valuable guide for diagnosing and management of HLH.
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH), is a multi-organ involved complex of clinical findings, which are results of hyper inflammatory response due to loss of immune regulation in case of various immune activation states such as infections, connective tissue diseases and malignancies. HLH has high mortality risk if diagnose or appropriate treatment is delayed. However, dramatic improvement is seen in situations with high mortality risk if we are aware of mortality predictors; and if aggressive treatment and close monitoring are applied. AIMS AND METHOD: We purposed that to determine mortality predictors by the way of laboratory and radiologic tests which are used frequently in clinical practice. With this purpose, we evaluate totally 63 case (26 woman and 37 man) who are diagnosed as HLH due to HLH-2004 diagnostic criteria and followed in the Ege University Hospital Internal Medicine Intesive Care Unit. Demographic characteristics are determined; peripheric blood samples (to calculate timely change in levels), teleradiographs are obtained at the time of hospitalization and at the primary end points (exitus or survived). Also bone marrow biopsies are obtained if there isn’t any contraindication. Categorical variables were analyzed by Chi-Square test and numerical variables were analyzed by Mann-Whitney Test. To determine the mortality predictors, logistic regression analysis were also performed. Moreover, ROC (receiver operating characteristic) analyze is performed to detect the best cut-off value for H score as a mortality predictor. RESULTS: Mortality ratio is found as 33,3% at the end of the study. Through the statistical analyzes, timely change of plasma CRP, albumin, creatinine, urea, ferritin, N-terminal pro-brain natriuretic peptide (NTpBNP), platelet count, lymphocyte count, neutrophil/lymphocyte raito (NLR) and mean platelet volume (MPV) are showed as mortality predictors. Also platelet counts at the time of hospitalization and lactate dehydrogenase (LDH) and cardiothoracic index (CTI) at the time of primary end point are found as mortality predictors. In addition these findings, H score is also found as a mortality predictor in the HLH population. CONCLUSION: HLH, is a immune hyperactivation state, which carries high mortality rates. Beginning to therapy immediately is an important predictor for mortality. It is possible that following these cases with easily reached, cheap and reliable laboratory and radiologic tests. It should be always remembered that, all these clinical findings may not arise in all patients and clinical suspicion is the most valuable guide for diagnosing and management of HLH.
Açıklama
Anahtar Kelimeler
Hemofagositoz, Lenfohistiyositoz, NTpBNP, H skoru, Hemophagocytosis, Lymphohistiocytosis, H score
