Neslihan KARAKURT, Fatma DEMİRBAŞ, Gönül ÇALTEPE, Canan ALBAYRAK, Ayhan Gazi KALAYCI

Çocukluk Çağında Ekstrahepatik Portal Ven Obstruksiyonuna Hematolojik Bakış Açısı

Amaç: Ekstrahepatik portal ven obstrüksiyonu nadir ancak önemli bir portal hipertansiyon PHT nedenidir. Hastalar sitopeni veya kanama ile hematoloji kliniğine başvurabilirler. Bu çalışmanın amacı bu hastalardaki tecrübemizi aktarmak ve portal ven trombozunda PVT trombofili araştırmasının önemini vurgulamaktır.Yöntem: Haziran 2006 ve Ekim 2018 tarihleri arasında hastanemize başvuran ve ekstra hepatik portal ven anomalisi tanısı alan çocuklar çalışmaya dahil edildi. Vasküler anomaliler trombus ve/ veya portal kavernom PK olarak ifade edildi. Bulgular retrospektif olarak incelendi. Bulgular: Yaş ortalaması 4,6 ± 3,4 olan 12 hasta K/E: 9/3 çalışmaya dahil edildi. Başvuruda en sık şikayet karın ağrısı ve gastrointestinal kanamaydı. Hastaların onunda %83 sitopeni vardı; sekizi %67 anemik, beşi %42 lökopenik ve dokuzu %75 trombositopenikti. Görüntüleme yöntemleri ile on hastada trombus, on hastada portal kavernom PK tespit edildi. Beş hastanın umbilikal kateterizasyon hikayesi olup birinde ayrıca had a history of umbilical catheterization and one was also positive for homozygous mutation of Factor V Leiden. Four patients were positive for heterozygous mutation of PAI- 1. Conclusion: PVT during childhood is rare, it may present with thrombocytopenia, splenomegaly and esophageal varices bleeding. Although PVT it is not a common cause of thrombocytopenia in children, clinicians are encouraged to get information regarding history of umbilical catheterization in neonatal period and obtain portal doppler ultrasound in cases with splenomegaly. Further studies about PVT Plasminogen activator inhibitor 1 mutations are needed. Faktör V Leiden homozigot mutasyonu saptandı. Dört hastada ise PAI-1 heterozigot mutasyonu saptandı.Sonuç: Çocukluk çağında PVT nadir olup trombositopeni, splenomegali ve özofagial varis kanaması ile prezente olabilir. Çocuklarda PVT trombositopeninin sık bir nedeni olmamasına rağmen klinisyenlerin yenidoğan döneminde umbilikal ven kateterizasyonuyla ilgili bilgi alması ve splenomegalisi olan hastalarda portal doppler USG değerlendirmesi önerilir. PAI-1 mutasyonları ile ilgili daha çok çalışmaya ihtiyaç vardır

Anahtar Kelimeler:

portal ven trombozu, trombositopeni, gastrointestinal kanama, splenomegali, plazminojen aktivatör inhibitör-1

Hematological Aspects of Extrahepatic Portal Vein Obstruction in Childhood

Objective: Extrahepatic portal vein obstruction is rare but considerable cause of portal hypertension PHT . Patients may present with cytopenia s or bleeding, to hematology clinics. The aim of this study is to present our experience of patients with this rare disease and emphasize the value of thrombophilia assessment in portal venous thrombosis PVT .Methods: Children admitted to our hospital between June 2006 and October 2018 with diagnosis of extra-hepatic portal venous anomalies are included. Vascular anomalies were defined as trombus and/or portal cavernoma PC . The medical reports were assessed retrospectively.Results: Twelve patients Female/ Male: 9/ 3 aged 4.6± 3.4 years old are included. The most common complaints on admission were abdominal pain and upper gastrointestinal bleeding. Ten patients 83% had cytopenia s at diagnosis; eight 67% had anemia, five had 42% leukopenia and nine had 75% thrombocytopenia. Imaging studies revealed thrombus in ten 83% and PC in ten 83% patients. Five patients had a history of umbilical catheterization and one was also positive for homozygous mutation of Factor V Leiden. Four patients were positive for heterozygous mutation of PAI- 1. Conclusion: PVT during childhood is rare, it may present with thrombocytopenia, splenomegaly and esophageal varices bleeding. Although PVT it is not a common cause of thrombocytopenia in children, clinicians are encouraged to get information regarding history of umbilical catheterization in neonatal period and obtain portal doppler ultrasound in cases with splenomegaly. Further studies about PVT Plasminogen activator inhibitor 1 mutations are needed.

Keywords:

Portal vein thrombosis, thrombocytopenia, gastrointestinal system bleeding, splenomegaly, plasminogen activator inhibitor-1,

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