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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 42 (2000), S. 215-217 
    ISSN: 1432-1920
    Keywords: Key words Kasabach-Merritt syndrome ; Haemangioendothelioma, kaposiform ; Haemangioma, bone changes ; Disseminated intravascular coagulation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Since the description in 1940 of Kasabach-Merritt syndrome (KMS) in patients with capillary haemangiomas, several other vascular tumours have been recognised as possible causes of this coagulopathy. The literature suggests a specific histological pattern of vascular tumours responsible for KMS, excluding capillary haemangioma [1]. There is an extensive literature on, haemangiomas accompanied by thrombocytopenia, and imaging of thrombosis in the lesion, especially cavernous haemangioma of the liver. However, no report has described a haemangioma of the mandible in the acute stage of the coagulopathy, or serial examinations of such a lesion. We report the features of a mandible lesion with KMS and discusses the interpretations of the changes observed.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0584
    Keywords: Key words Lymphocytosis ; Epstein-Barr virus ; LMP-1 ; LMP-2A ; Kindred
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Epstein-Barr virus (EBV) genomes have been detected in peripheral blood lymphocytes (PBL) of patients with persistent polyclonal B-cell lymphocytosis (PPBL). This is consistent with the hypothesis that latent EBV infection is involved in the pathogenesis of this disorder. Two EBV-encoded proteins expressed in viral latency are the latent membrane proteins 1 and 2A (LMP1 and LMP2A). We have studied the LMP1 oncogene and the LMP2A gene in a female patient with PPBL and her five siblings. A cell line derived from peripheral blood lymphocytes (PBL) of the patient was also analyzed. A distinct 69-base pair deletion was identified within the carboxy terminal NF-κB activation domain of the LMP1 oncogene in PBL of the patient and in the cell line, whereas none of the siblings harbored this deletion. The tyrosine-signaling motif and the HLA A2.1 epitope of the LMP2A gene were wild type in the patient and all siblings. The presence of a 69-base pair deletion variant of the LMP1 oncogene within the lymphocytes of a PPBL patient but absence of this deletion variant in the unaffected siblings suggests a direct implication of altered LMP1 oncoprotein-dependent function in the pathogenesis of PPBL.
    Type of Medium: Electronic Resource
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