leukocyte adhesion deficiency type 1 presenting as leukemoid reaction
نویسندگان
چکیده
the hallmarks of leukocyte adhesion deficiency (lad) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. these molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. over last 20 years, three distinctive defects in the leukocyte adhesion cascade, involving several precise ordered steps such as rolling, integrin activation and firm adhesion of the leukocytes have been described. while lad i and ii are clearly autosomal recessive disorders, the mode of inheritance of lad iii is still not clear. lad i is due to structural defects in the integrin molecule, preventing firm adhesion to occur. we present a case of a male neonate referred with continuation of leukemoid reaction and multiple non healing ulcers, gingivitis and periodontitis in spite of good antibiotic therapy.
منابع مشابه
Leukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
متن کاملLeukocyte adhesion deficiency type 1 presenting as leukemoid reaction.
Leukocyte Adhesion Deficiency (LAD) is characterized by the inability of leukocytes, in particular neutrophilic granulocytes, to emigrate from the blood stream towards sites of inflammation. Infectious foci are nonpurulent and may eventually become necrotic because of abnormal wound healing. LAD-I is characterized by the absence of the beta-2 integrins CD II and CD 18 on leukocytes. We present ...
متن کاملLeukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
متن کاملDelayed wound healing in leukocyte adhesion deficiency type 1.
*KURAに登録されているコンテンツの利用については,著作権法に規定されている私的使用や引用などの範囲内で行ってください。 *著作権法に規定されている私的使用や引用などの範囲を超える利用を行う場合には,著作権者の許諾を得てください。ただし,著作権者 から著作権等管理事業者(学術著作権協会,日本著作出版権管理システムなど)に権利委託されているコンテンツの利用手続については ,各著作権等管理事業者に確認してください。 Title Delayed Wound Healing in Leukocyte Adhesion Deficiency Type 1 Author(s) Wada, Taizo; Tone, Yumi; Shibata, Fumie; Toma, Tomoko; Yachie, Akihiro Citation Journal of Pediatrics, 158(2):...
متن کاملOsteomyelitis in leukocyte adhesion deficiency type 1 syndrome.
Leukocyte adhesion deficiency type 1 (LAD-1) is a rare, inherited immunodeficiency that affects one per million people yearly and usually presents with recurrent, indolent bacterial infections of the skin, mouth, and respiratory tract and impaired pus formation and wound healing. A 13-year-old girl diagnosed LAD-I at the age of 7 years was brought to the Immunology, Asthma and Allergy Research ...
متن کاملprenatal diagnosis of leukocyte adhesion deficiency type-1 (five cases from iran with two new mutations).
leukocyte adhesion deficiency type-1(lad-1) is one of the autosomal recessive immunodeficiency diseases that results from mutation in integrin beta 2 (itgb2) gene. the aim of this study was to investigate molecular prenatal diagnosis of lad-1. four pregnant women with five fetuses (one twin fetus) with clinical and laboratory diagnosis of lad-1 in their previous children were studied. the chori...
متن کاملمنابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
iranian journal of pathologyناشر: iranian society of pathology
ISSN 1735-5303
دوره 8
شماره 3 2013
میزبانی شده توسط پلتفرم ابری doprax.com
copyright © 2015-2023