Retrospective Evaluation of Hemophagocytic Lymphohistiocytosis Cases Treated

Öz

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory syndrome resulting from uncontrolled proliferation in the monocyte / macrophage system. It is divided into two classes as familial (primary) and secondary. Since the rate of consanguineous marriage in our country is high with 29.2%, the frequency of HLH is also increasing. Secondary HLH can be seen at any age, but its true incidence is unknown. It can accompany malignancies, infections and inflammatory processes. Major clinical findings are persistent high fever, cytopenia, splenomegaly and / or hepatomegaly. The first finding is usually fever. The goal of treatment is to stop abnormal inflammation and treat the underlying cause. MATERIALS and METHODS: We retrospectively reviewed 15 patients diagnosed and treated in our clinic between 2010 and 2019. Statistical analysis was performed with kruskal-wallis test. RESULTS: The median age of diagnosis of 15 patients diagnosed and treated in our clinic was 18 months (1 month-17 years). The female to male ratio was 6/9. Fever was seen in all patients, hepatosplenomegaly was 80%. The mean ferritin value was 33.927 ± 51.461µ / l (964-201.074). In our patient group, consanguineous marriage rate was 53% (n = 8) and it was high and the factor was EBV in all patients who developed secondary to infection. 33% (n = 5) of the cases were primary and 67% (n = 10) were secondary. 40% (n = 4) of secondary cases had malignancy in etiology, 50% (n = 5) infection and 10% (n = 1) metabolic disease. CONCLUSIONS: HLH is important for early diagnosis in cases with persistent high fever, hepatosplenomegaly and high ferritin conditions in our country. In cases presenting with HLH, malignancy should not be forgotten in etiology besides primary causes.

Anahtar Kelimeler

• Hemophagocytic lymphohistiocytosis,cytopenia,hepatosplenomegaly

Hemofagositik Lenfohistiyositozisile Tedavi Edilen Olgularının Retrospektif Olarak Değerlendirilmesi

Öz

GİRİŞ ve AMAÇ: Hemofagositik lenfohistiyositoz (HLH) monosit/makrofaj sistemindeki kontrolsüz proliferasyon sonucu oluşan nadir görülen enflamatuar bir sendromdur. Ailesel (primer) ve sekonder olmak üzere iki sınıfa ayrılmaktadır. Ülkemizde akraba evliliği oranı %29.2 ile yüksek olduğu için HLH sıklığı da artmaktadır. Sekonder HLH her yaşta görülebilmekle beraber gerçek insidansı bilinmemektedir. Maligniteler, enfeksiyonlar ve inflamatuar süreçlere eşlik edebilir. Major klinik bulgular persistan yüksek ateş, sitopeni, splenomegali ve/veya hepatomegalidir. İlk bulgu genellikle ateştir. Tedavide amaç anormal inflamasyonu durdurmaktır ve altta yatan nedeni tedavi etmektir. YÖNTEM ve GEREÇLER: Kliniğimizde 2010 ile 2019 yılları arasında tanı konulup tedavi edilen 15 hastayı retrospektif olarak değerlendirdik. İstatistiksel analiz kruskal- wallis testi ile yapıldı. BULGULAR: Kliniğimizde tanı alıp tedavi edilen 15 hastanın ortanca tanı yaşı 18 aydı(1ay- 17 yaş). Olguların kız erkek oranı 6/9 idi. Ateş bütün hastalarda görüldü, hepatosplenomegali %80 oranında vardı. Ortalama ferritin değeri 33.927±51.461µ/l (964-201.074)idi. Hasta grubumuzda akraba evliliği oranı %53 (n=8) olup yüksekti ve enfeksiyona sekonder gelişen hastaların tümünde etken EBV idi. Olguların %33’ü (n=5) primer, %67’si(n=10) sekonderdi. Sekonder olguların %40’ında(n=4) etyolojide malignite, %50’sinde (n=5) enfeksiyon ve %10’unda (n=1) metabolik hastalık vardı. TARTIŞMA ve SONUÇ: Ülkemiz koşullarında persistan yüksek ateş,hepatosplenomegali ve ferritin yüksekliği ile gelen olgularda HLH düşünülmesi erken tanı açısından önemlidir. HLH tablosu ile gelen olgularda primer nedenler yanı sıra etyolojide malignite de unutulmamalıdır

Anahtar Kelimeler

• Hemofagositik lenfohistiyositoz,sitopeni,hepatosplenomegali

Kaynakça

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