Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lethal midline granuloma syndrome (LMG) is the clinical term generally used to describe a rare clinical entity of unknown cause characterized by a progressive and often fatal ulceration and destruction of the upper airway involving the nose, the paranasal sinuses and the soft tissues of the face. The following histopathologic entities have always been grouped under the term LMG: Wegener granulomatosis (WG), non-Hodgkin lymphoma, polymorphic reticulosis (PR) and idiopathic midline destructive disease (IMDD). Today in order to begin an adequate therapy a differential diagnosis is necessary. The progresses in clinical immunology and immunohistochemistry and cytophotometric findings are useful to define the LMG syndrome better. First of all the LMG must be discriminated from localized WG occurring in the midface. Serologic examinations of patients with WG in its active form show a high percentage of autoantibodies against cytoplasmic structures in leucocytes and monocytes. Moreover a review of the literature allows us to suppose that cases described as IMDD and PR are indeed a large evolutionary spectrum from almost benign to fatal malignant lymphoma. We report a case of Ki 1-lymphoma presenting as LMG syndrome.
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PMID:Lethal midline granuloma: a case of Ki-1 lymphoma. 819 Oct 49

Lethal midline granuloma (LMG) is associated with Epstein-Barr virus (EBV). The latter has at least two subtypes with different biological properties. The subtypes can be identified by their genomic configuration. Using EBV-RNA (EBER) in situ hybridization and EBV polymerase chain reaction (PCR), we have looked for the presence of EBV in six LMGs and six non-Hodgkin's lymphomas (NHLs) located in the nasopharyngeal region, and determined the subtype of EBV. Six of six LMGs were positive by PCR and EBER in situ hybridization, whereas NHLs were either negative or, in three of six cases, showed few EBER-positive cells considered to be nonneoplastic lymphocytes. The subtype 2 was found in LMG lesions of three of six patients; the remaining three of six patients with LMG had the generally occurring subtype 1. The results indicate that the association of EBV with NHL may depend more on tumor type than on its localization. The occurrence of the rare subtype 2 in LMG may relate to a covert immune defect.
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PMID:Association of the subtype 2 of the Epstein-Barr virus with T-cell non-Hodgkin's lymphoma of the midline granuloma type. 839 51