Çocukluk Çağında Ekstrahepatik Portal Ven Obstruksiyonuna Hematolojik Bakış Açısı

Year 2019, Volume: 76 Issue: 4, 415 - 422, 01.12.2019

Neslihan Karakurt Fatma Demirbaş Gönül Çaltepe Canan Albayrak Ayhan Gazi Kalaycı

Abstract

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

Keywords

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

Hematological Aspects of Extrahepatic Portal Vein Obstruction in Childhood

Abstract

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

References

• 1. European Association for the Study of the Liver. Electronic address: [e-mail]. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016;64(1):179-202.
• 2. Valla DC, Cazals-Hatem D. Vascular liver diseases on the clinical side:definitions and diagnosis, new concepts. Virchows Arch. 2018;473(1):3-13.
• 3. Giouleme O, Theocharidou E. Management of portal hypertension in children with portal vein thrombosis.. J Pediatr Gastroenterol Nutr. 2013;57(4):419-25.
• 4. Kumar R, Kerlin BA. Thrombosis of the Abdominal Veins in Childhood. Front Pediatr. 2017 Sep 5;5:188.
• 5. Gürakan F, Eren M, Koçak N, Yüce A, Ozen H, Temizel IN, Demir H. Extrahepatic portal vein thrombosis in children: etiology and long-term follow-up. J Clin Gastroenterol. 2004;38(4):368-72.
• 6. El-Karaksy HM, El-Koofy N, Mohsen N, Helmy H, Nabil N, El-Shabrawi M. Extrahepatic portal vein obstruction in Egyptian children. J Pediatr Gastroenterol Nutr. 2015;60(1):105-9.
• 7. Jain M, Jain J, Passi GR, Jain K, Jain S. Profile of extrahepatic portal venous obstruction among children in Central India. Clin Exp Hepatol. 2017;3(4):209-211.
• 8. Sokal EM, Van Hoorebeeck N, Van Obbergh L, Otte JB, Buts JP. Upper gastro-intestinal tract bleeding in cirrhotic children candidates for liver transplantation. Eur J Pediatr. 1992;151(5):326-8.
• 9. Sharara AI, Rockey DC. Gastroesophageal variceal hemorrhage. N Engl J Med. 2001;345(9):669-81. Review.
• 10. Radovich PA. Portal vein thrombosis and liver disease. J Vasc Nurs. 2000;18(1):1-5. Review.
• 11. Morag I, Epelman M, Daneman A, Moineddin R, Parvez B, Shechter T, Hellmann J. Portal vein thrombosis in the neonate: risk factors, course, and outcome. J Pediatr. 2006;148(6):735-9.
• 12. Rajpurkar M, Sharathkumar A, Williams S, Lau K, Ling SC, Chan AK Recommendations for the assessment of non-extremity venous thromboembolism outcomes: communication from the SSC of the ISTH. Pediatric/ Neonatal Hemostasis and Thrombosis Scientific and Standardization Subcommittee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2015;13(3):477-80.
• 13. Middeldorp S. Inherited thrombophilia: a doubleedged sword. Hematology Am Soc Hematol Educ Program 2016; 2016: 1-9.
• 14. Celkan T, Dikme G. Thrombosis in children: Which test to whom, when and how much necessary? Turk Pediatri Ars. 2018;53(1):1-9.
• 15. Sobhan MR, Mahdinezhad-Yazdi M, Moghimi M, Aghili K, Jafari M, Zare-Shehneh M,Neamatzadeh H. Plasminogen Activator Inhibitor-1 4G/5G Polymorphism Contributes to Osteonecrosis of the Femoral Head Susceptibility: Evidence from a Systematic Review and Meta-analysis. Arch Bone Jt Surg. 2018;6(6):468-477.
• 16. Adly AA, Elbarbary NS, Ismail EA, Hassan SR. Plasminogen activator inhibitor-1(PAI-1) in children and adolescents with type 1 diabetes mellitus: relation to diabetic micro-vascular complications and carotid intima media thickness. J Diabetes Complications. 2014;28(3):340-7.
• 17. Gohil R, Peck G, Sharma P. The genetics of venous thromboembolism. A meta-analysis involving approximately 120,000 cases and 180,000 controls. Thromb Haemost. 2009;102(2):360-370.
• 18. Mahajerin A, Obasaju P, Eckert G, Vik TA, Mehta R, Heiny M. Thrombophilia testing in children: a 7 year experience. Pediatr Blood Cancer.2014;61(3):523-7.
• 19. Simone B, De Stefano V, Leoncini E. Risk of venous thromboembolism associated with single and combined effects of factor V Leiden, prothrombin 20210A and methylenetethraydrofolate reductase C677T: a metaanalysis involving over 11,000 cases and 21,000 controls. Eur J Epidemiol 2013; 28: 621- 47.
• 20. Winslow ER, Brunt ML, Drebin JA, et al. Portal vein thrombosis after splenectomy. Am J Surg. 2002;184:631–636.