Epstein-Barr virus-associated smooth muscle tumours in a patient with an immuno-osseous dysplasia
نویسندگان
چکیده
منابع مشابه
Non-Hodgkin Lymphoma in a Child with Schimke Immuno-Osseous Dysplasia
Schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid-resistant nephrotic syndrome, immunodeficiency, and spondyloepiphy-seal dysplasia. Mutations in SWI/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) gene are responsible for the disease. The present report describes, for the f...
متن کاملNovel compound mutations of SMARCAL1 associated with severe Schimke immuno-osseous dysplasia in a Chinese patient.
BACKGROUND Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive pleiotropic disease caused by mutations in the SMARCAL1 gene. To date there have been no data from the Chinese population. Here, we report the first SIOD case in the Chinese population. No case with gross carpal bone age retardation has been reported previously. METHODS The index patient was diagnosed by clinical...
متن کاملAn Epstein-Barr virus-associated smooth muscle tumor successfully treated with surgical resection: a case report and literature review.
Epstein-Barr virus–associated smooth muscle tumors (EBV-SMTs) occur predominantly in immunocompromised patients, either in the setting of solid organ transplantation or with HIV/AIDS.1 The role of EBV in the development of smooth muscle tumors was first described in the 1990s, after studies consistently showed high levels of EBV replication, EBV in-situ hybridization positivity (EBV encoded RNA...
متن کاملAn Epstein-Barr virus-associated superantigen
More than 90% of adults are latently infected with Epstein-Barr virus (EBV), the causative agent of infectious mononucleosis, a self-limiting lymphoproliferative disease characterized by extensive T cell activation. Reactivation of this herpesvirus during immunosuppression is often associated with oncogenesis. These considerations led us to analyze the early events that occur after exposure of ...
متن کاملLongevity in Schimke immuno-osseous dysplasia.
Schimke immuno-osseous dysplasia (SIOD) is characterised by autosomal recessive inheritance, spondyloepiphyseal dysplasia causing growth retardation, defective cellular immunity, progressive nephropathy leading to renal failure, hyperpigmented macules, and dysmorphic facial features. Half of SIOD patients also have hypothyroidism, half episodic cerebral ischaemia, and a tenth bone marrow failur...
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ژورنال
عنوان ژورنال: Clinical Radiology Extra
سال: 2004
ISSN: 1477-6804
DOI: 10.1016/j.cradex.2004.01.004