هماتوپوئز خارج مغز استخوان در سینوس اسفنوئیدال در بیمار با تالاسمی اینترمدیت و بررسی منابع

نوع مقاله: گزارش مورد

نویسنده

اســــتادیار همــــاتولوژی انکولــــوژی، بیمارســتان امــام رضــا (ع)، دانــشگاه علــوم پزشکی مشهد، مشهد، ایران

چکیده

مقدمه
هماتوپوئز اکسترامدولری یک عارضه مهم تالاسمی متوسط است . این عارضه معمولا در ناحیه پـاراورتبرال
و بافت نرم سایر مناطق به صورت توده فشارنده و بـ ه نـدرت در داخـل جمجمـه دیـده مـی شـود و تـا کنـون
موردی از آن در سینوس اسفنوئید گزارش نشده است.
معرفی بیمار
در این مقاله بیمار 21 ساله با تالاسمی متوسط و توده اسفنوئید گزارش میگردد. بیمار تحـت اسـپلنکتومی و
کله سیستکتومی همزمان به علت سنگ صفراوی متعدد قرار گرفت. بعد از اسپلنکتومی اریتروبلاسـت هـا در
خون محیطی به طور پیشرونده افزایش یافته و یـک سـال بعـد بیمـار بـا علائـم سـی نوزیت حـاد ویزیـت شـد.
مطالعات رادیولوژیک و بیوپسی، توده هماتوپوئتیک را در سینوس اسفنوتید مشخص کرد. سپس بیمار تحت
درمان جراحی و طبی قرار گرفت . بیمار به علت عدم پاسخ به درمان جهت پیوند سلول بنیادی هماتوپوئتیک
معرفی گردید.
نتیجه گیری
هماتوپوئز اکتوپیک یک پدیده نادر در تالاسمیهـا اسـت ولـی از آنجـایی کـه مـی توانـد در بعـضی منـاطق
حساس مثل پاراورتبرال و داخل جمجمه ایجـاد و باعـث عـوارض شـدید گـردد، تـشخیص و درمـان سـریع
بیماری در افراد با ریسک بالا میتواند از بروز عوارض آن پیشگیری کند. 

کلیدواژه‌ها


عنوان مقاله [English]

A Case Report of Extramedullary Hematopoiesis in Sphenoidal Sinus in Patient with Intermediate Beta Thalassemia and Review of Literature

نویسنده [English]

  • Zahra Mozaheb
Assistant Professor of Hematology and Onculogy, Emam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran
چکیده [English]

Introduction
Extramedullary hematopoiesis is an important side effect of intermediate thalassemia. It is usually observed in paravertebral and other soft tissues as a compressive mass and rarely in intracranial, but it has not been roported in sphenoidal sinus in literature until now.
Case report
In this article a 21- year old patient with intermediate thalassemia and sphenoidal mass has been reported. The patient underwent splenectomy and cholecystectomy simultaneously, for the billiary stones. After splenectomy his erythroblast increasesd progressively in peripheral blood, and one year later he was treated, because of acute sinusitis. Imaging studies and biopsy revealed the hematopoietic mass in sphenoidal sinus. The patient did not respond to surgical and medical treatments; therefore, he was referred for the hematopoietic stem cell transplantation.
Conclusion
Ectopic hematopoiesis is a rare event in thalassemia, but it can appear in such important areas such as paravertebral and intracranial, which causes severe side effects. Therefore, immediate diagnosis and treatment in high risk patients can prevent these effects.

کلیدواژه‌ها [English]

  • Extramedullary hematopoiesis
  • Intermediate thalassemia
  • Sphenoidal sinus mass
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