| dbo:abstract
|
- الاورام اللمفاوية للخلايا الكبيرة المتحولة أو اللمفوما ضخمة الخلايا فاقدة التمايز الخلوي (ALCL) هو نوع من الأورام اللمفاوية غير هودجكين ولها سمات الاورام اللمفاوية في تصنيف منظمة الصحة العالمية (WHO). وتستمد اسمها من (ar)
- Das anaplastisch-großzellige Lymphom (ALCL) ist ein malignes T-Zell-Lymphom, das nach der WHO-Klassifikation zu den Non-Hodgkin-Lymphomen gezählt wird. Der Name leitet sich aus den Begriffen ‚Anaplasie‘ und ‚großzelliges Lymphom‘ ab. Es wurde erstmals 1985 als eigenständige Tumorentität beschrieben. (de)
- Anaplastic large cell lymphoma (ALCL) refers to a group of non-Hodgkin lymphomas in which aberrant T cells proliferate uncontrollably. Considered as a single entity, ALCL is the most common type of peripheral lymphoma and represents ~10% of all peripheral lymphomas in children. The incidence of ALCL is estimated to be 0.25 cases per 100,000 people in the United States of America. There are four distinct types of anaplastic large cell lymphomas that on microscopic examination share certain key histopathological features and tumor marker proteins. However, the four types have very different clinical presentations, gene abnormalities, prognoses, and/or treatments. ALCL is defined based on microscopic histopathological examination of involved tissues which shows the presence of at least some ALCL-defining pleomorphic cells. These "hallmark" cells have abnormal kidney-shaped or horseshoe-shaped nuclei, prominent Golgi, and express the CD30 tumor marker protein on their surface membranes. In 2016, the World Health Organization (WHO) separated ALCL into four types: ALK-positive ALCL (also termed ALK+ ALCL), ALK-negative ALCL (ALK− ALCL), primary cutaneous ALCL (pcALCL), and breast implant-associated ALCL (BIA-ALCL). WHO defined BIA-ALCL as an ALCL type provisionally, i.e. subject to redefinition if future studies should support such a change. ALK-positive and ALK-negative ALCL are aggressive systemic lymphomas. They are differentiated based on their expression of an abnormal ALK protein made by a somatic recombination in the ALK gene. ALK, i.e. anaplastic lymphoma kinase, is a protein product of the ALK gene located on chromosome 2. In ALK-positive ALCL, a portion of the ALK gene has merged with another site on the same or different chromosome to form a chimeric gene consisting of part of the new site and part of the ALK gene coding for ALK's activity. This chimeric gene overproduces a fusion protein with excessive ALK activity. ALK is a serine/threonine-specific protein kinase that activities PI3K/AKT/mTOR, Ras-activated ERKs, Janus kinase-activated STAT proteins, and other cell signaling pathways as well as the expression of various genes by epigenetic mechanisms. Activations of these signaling pathways and genes may stimulate cell growth, proliferation, survival, and/or other behaviors that promote malignancy. ALK-negative ALCL, while not involving ALK translocations, has, in a variable percentage of cases, various translocations, rearrangements, and mutations that may contribute to its development. pcALCL and BIA-ALCL are far less aggressive lymphomas that tend to be localized to one or a very few sites. pcALCL presents as a single or, less commonly, multifocal skin papules or tumors that typically are limited to the dermis without infiltrating to the subcutaneous tissues or spreading to other sites. Its neoplastic cells may contain some gene translocations including, in very rare cases, ones with the ALK gene that are similar to those in ALK-positive ALCL. BIA-ALCL is caused by and develops around a breast implant. It typically presents many years after the surgical implantation as a deformation, textural change, and/or pain emanating in the area around implanted breast. In most cases, the disease is localized to the involved breast. BPI-ALCL is associated with occasional mutations in one or two genes but has not been reported to be associated with products of gene translocations or rearrangements. (en)
- Le lymphome anaplasique à grandes cellules (LAGC) est un lymphome non Hodgkinien à cellules T périphériques (LTP), rare et agressif, appartenant au groupe des syndromes lymphoprolifératifs CD30 positifs et affectant les ganglions lymphatiques, médiastinaux ou abdominaux avec extension possible à des sites extranodaux. Il comprend deux sous-types, selon l'expression ou non d'une protéine appelée kinase du lymphome anaplasique (ALK) :
* le LAGC ALK-positif (CIM-10 C84.6) dans lequel le gène ALK du récepteur de la tyrosine kinase du lymphome anaplasique est surexprimé ;
* le LAGC ALK-négatif (CIM-10 C84.7). Le LAGC peut se former dans le tissu cicatriciel entourant un implant mammaire, surtout s'il est texturé. On parle alors de LAGC associé à un implant mammaire ou LAGC-AIM (cf. enquête « Implant files »). Ces lymphomes sont pour la plupart ALK-négatifs et CD30-positifs. (fr)
- La locuzione linfoma a grandi cellule anaplastico (o ALCL dall'inglese anaplastic large cell lymphoma) identifica un tipo di linfoma non Hodgkin strettamente associato a riarrangiamenti che interessano il cromosoma 2, con formazione di una proteina chimerica NPM-ALK avente attività tirosin-chinasica costitutiva. (it)
- 未分化大細胞型リンパ腫（みぶんかだいさいぼうがたリンパしゅ、Anaplastic large cell lymphoma: ALCL）は、T細胞性リンパ腫の一型である。 (ja)
|
| rdfs:comment
|
- الاورام اللمفاوية للخلايا الكبيرة المتحولة أو اللمفوما ضخمة الخلايا فاقدة التمايز الخلوي (ALCL) هو نوع من الأورام اللمفاوية غير هودجكين ولها سمات الاورام اللمفاوية في تصنيف منظمة الصحة العالمية (WHO). وتستمد اسمها من (ar)
- Das anaplastisch-großzellige Lymphom (ALCL) ist ein malignes T-Zell-Lymphom, das nach der WHO-Klassifikation zu den Non-Hodgkin-Lymphomen gezählt wird. Der Name leitet sich aus den Begriffen ‚Anaplasie‘ und ‚großzelliges Lymphom‘ ab. Es wurde erstmals 1985 als eigenständige Tumorentität beschrieben. (de)
- La locuzione linfoma a grandi cellule anaplastico (o ALCL dall'inglese anaplastic large cell lymphoma) identifica un tipo di linfoma non Hodgkin strettamente associato a riarrangiamenti che interessano il cromosoma 2, con formazione di una proteina chimerica NPM-ALK avente attività tirosin-chinasica costitutiva. (it)
- 未分化大細胞型リンパ腫（みぶんかだいさいぼうがたリンパしゅ、Anaplastic large cell lymphoma: ALCL）は、T細胞性リンパ腫の一型である。 (ja)
- Anaplastic large cell lymphoma (ALCL) refers to a group of non-Hodgkin lymphomas in which aberrant T cells proliferate uncontrollably. Considered as a single entity, ALCL is the most common type of peripheral lymphoma and represents ~10% of all peripheral lymphomas in children. The incidence of ALCL is estimated to be 0.25 cases per 100,000 people in the United States of America. There are four distinct types of anaplastic large cell lymphomas that on microscopic examination share certain key histopathological features and tumor marker proteins. However, the four types have very different clinical presentations, gene abnormalities, prognoses, and/or treatments. (en)
- Le lymphome anaplasique à grandes cellules (LAGC) est un lymphome non Hodgkinien à cellules T périphériques (LTP), rare et agressif, appartenant au groupe des syndromes lymphoprolifératifs CD30 positifs et affectant les ganglions lymphatiques, médiastinaux ou abdominaux avec extension possible à des sites extranodaux. Il comprend deux sous-types, selon l'expression ou non d'une protéine appelée kinase du lymphome anaplasique (ALK) : (fr)
|
