Çocuklarda enfeksiyon ilişkili hemofagositik sendrom: 15 vakanın geriye dönük analizi

Amaç:Hemofagositik sendrom (HFS) aşırı immün aktivasyona bağlı hızlı ilerleyen ve hayatı tehdit eden bir sendromdur. HFS ile ilişkili klinik tablo ve laboratuvar anormalliklerinin erken tanınması ve tedaviye hemen başlanması hayat kurtarıcı olabilmektedir. Bu çalışmada enfeksiyon ilişkili hemofagositik sendrom (EİHFS) tanısıyla izlediğimiz çocuk hastalarda; altta yatan enfeksiyöz etkenlerin, klinik, laboratuvar bulguların ve tedavi sonuçlarının incelenmesi amaçlanmıştır. Gereç ve yöntem: Aralık 2012 ile Ocak 2016 arasında EİHFS tanısı konan çocuk hastaların kayıtları geriye dönük olarak incelendi. Bulgular: Çalışmaya yaşları ortanca 6 yaş (2-16 yaş) olan toplam 15 hasta (8 erkek, 7 kız) dahil edildi. HFS’yi tetikleyen en sık enfeksiyöz etken olarak Brusella (8 hasta, %53) saptandı. Diğer altta yatan enfeksiyöz etkenler; 3 hastada Leishmania, 1 hastada Salmonella Typhi, 1 hastada Mycobacterium tuberculosis, 1 hastada parvovirüs B19 ve 1 hastada influenza A (H3N2) idi. Ateş, bi/pansitopeni, hiperferritinemi ve serum transaminaz düzeylerinde yükseklik tüm hastalarda (%100), splenomegali 12 hastada (%80) mevcuttu. Tüm hastaların kemik iliği aspirasyon incelemesinde hemofagositoz görüldü. Tüm hastalara altta yatan enfeksiyonuna yönelik antimikrobiyal tedavi; uzamış ateş ve klinik durumunda giderek kötüleşme olan 8 hastaya intravenöz immünglobülin (İVİG), 2 hastaya İVİG+steroid tedavileri verildi. Olguların %93’ünde (14 hasta) iyileşme kaydedildi. İnfluenza A (H3N2) pnömonisi olan sadece 1 hastada (%6) ölüm görüldü. Sonuç: Hekimler, enfeksiyon nedeniyle izlenen hastalarda uzamış ateş, splenomegali ve sitopeninin varlığında HFS gelişimi konusunda farkındalığa sahip olmalıdır.

Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases

Purpose: Haemophagocytic syndrome (HPS) is a rapidly progressive, life-threatening syndrome of excessive immune activation. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. The aim of this study was to investigate the infectious etiologies, clinical and laboratory characteristics, and treatment outcomes of pediatric patients with infection associated hemophagocytic syndrome (IAHPS). Materials and methods: This is a retrospective record review of pediatric patients diagnosed to have IAHFS between December 2012 and January 2016. Results: A total of 15 pediatric patients with IAHPS (8 boys / 7 girls; median age: 6 years, range 2-16 years) were enrolled in this study. Brucella (8 patients, 53%) was the most frequent infectious trigger of HFS. Other infectious triggers were Leishmania (3 patients), Salmonella Typhi (1 patient), Mycobacterium tuberculosis (1 patient), parvovirus B19 (1 patient), and influenza A (H3N2) (1 patient). Fever, bi/pansitopenia, hyperferritinemia, and elevated transaminases were present in all patients (100%), and splenomegaly in 12 patients (80%). All of the patients underwent bone marrow aspirations that confirmed hemophagocytosis. All patients received antimicrobial treatment for the underlying infections; 8 patients received intravenous immunoglobulin (IVIG) and 2 patients received IVIG+steroids due to persistent fever and progressively worsening clinical conditions. Recovery was noted in 14 (93%) patients. Mortality was only seen in 1 patient (6%) with influenza A (H3N2) pneumonia.

Kaynakça

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