گزارش یک مورد هیستیوسیتوزسینوزال با لنفادنوپاتی ماسیو (بیماری روزائی- دورفمن)

نوع مقاله : گزارش مورد

نویسندگان

1 استادیار پاتولوژی

2 استادیار جراحی اطفال

3 استادیار اطفال

4 رزیدنت پاتولوژی

چکیده

هیستیوسیتوزسینوزال با لنفادنوپاتی ماسیو (بیماری روزائی- دورفمن)، یک بیماری نادر خوش خیم هیستیوسیتی با علت ناشناخته بوده، که به طور معمول با
بزرگی غدد لنفاوی سطحی و عمقی همراه است. بیماری معمولا سیر بالینی طولانی داشته و با دوره های متناوب از تشدید و فروکش علائم، توام است.
در این مقاله یک مورد از بیماری روزائی- دورفمن در پسر 5 ساله، با لنفادنوپاتی حجیم ناحیه اینگوینال راست، لنفادنوپاتی گردنی، تب گاهگاهی و کم خونی
گزارش گردیده است و نشانه های بالینی، ویژگی های هیستوپاتولوژیک و درمان آن، مورد بحث قرار گرفت.
 

کلیدواژه‌ها


عنوان مقاله [English]

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A Case Report

نویسندگان [English]

  • N Zabolinejad 1
  • M. Hiradfar 2
  • Z Badiee 3
  • N Ghaemi 3
  • A. Attaranzadeh 4
1
2
3
4
چکیده [English]

Introduction: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites.
It usually shows a prolonged clinical course with occasional exacerbation and remission phases.
In this article case of RDD in a 5-year-old male with large right inguinal and bilateral cervical lymphadenopathies were, occasional fever and anemia was described. The clinical presentation, histopathologic characteristics, and treatments of RDD were discussed.

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