Volume 17, Issue 75 (9-2010)                   RJMS 2010, 17(75): 77-82 | Back to browse issues page


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Ansari S, Tashvighi, M, Baghersalimi A. Portal Vein Thrombosis after Splenectomy in a 24-year-old Man with Major Thalassemia: A Case Report. RJMS. 2010; 17 (75) :77-82
URL: http://rjms.iums.ac.ir/article-1-1502-en.html

Pediatrician
Abstract:   (6668 Views)

    Introduction: Beta thalassemia major is a severe hemolytic anemia due to inadequate synthesis of beta chain globin. Splenomegaly secondary to extramedullary hematopoiesis and iron overload is one of the most prominent findings and makes splenectomy inevitable in majority of these patients. For many reasons these patients are at risk of hypercoagulation. Portal vein thrombosis is a rare consequence of splenectomy. We report a 24 year old  thalassemic patient that developed portal vein thrombosis following splenectomy.

Case Report:  A 24 year old man was diagnosed with thalassemia major at 2 years of age. Regular transfusion regimen was initiated at the time of the diagnosis. At this age, patient underwent open splenectomy secondary to hypersplenism with a smooth postoperative recovery. Twelve days later, he developed severe abdominal pain with fever. Portal vein thrombosis was detected by Color Doppler ultrasonography. Therapy with heparin which was replaced by low molecular weight heparin after 4 days was begun, and was followed by the addition of warfarin on day 5. The patient was discharged on the 12th day of admission with good condition, and with instructions to continue warfarin and to follow up with a hematologist. Four months later the patient remained well.

Conclusion:This case suggests that thalassemia major patients undergoing splenectomy have an increased risk of Portal Vein Thrombosis (PVT). We recommend that PVT should be considered in a patient with abdominal pain and fever after splenectomy. Color Doppler ultrasonography imaging is the best non-invasive study for the diagnosis at onset of presentation. Treatment with anticoagulants is safe and highly recommended

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Type of Study: case report | Subject: Pediatric Disease

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