Back to Search
Start Over
The Pathological Spectrum of Systemic Anaplastic Large Cell Lymphoma (ALCL)
- Source :
- Cancers, Vol 10, Iss 4, p 107 (2018), Cancers
- Publication Year :
- 2018
- Publisher :
- MDPI AG, 2018.
-
Abstract
- Anaplastic large cell lymphoma (ALCL) represents a group of malignant T-cell lymphoproliferations that share morphological and immunophenotypical features, namely strong CD30 expression and variable loss of T-cell markers, but differ in clinical presentation and prognosis. The recognition of anaplastic lymphoma kinase (ALK) fusion proteins as a result of chromosomal translocations or inversions was the starting point for the distinction of different subgroups of ALCL. According to their distinct clinical settings and molecular findings, the 2016 revised World Health Organization (WHO) classification recognizes four different entities: systemic ALK-positive ALCL (ALK+ ALCL), systemic ALK-negative ALCL (ALK− ALCL), primary cutaneous ALCL (pC-ALCL), and breast implant-associated ALCL (BI-ALCL), the latter included as a provisional entity. ALK is rearranged in approximately 80% of systemic ALCL cases with one of its partner genes, most commonly NPM1, and is associated with favorable prognosis, whereas systemic ALK− ALCL shows heterogeneous clinical, phenotypical, and genetic features, underlining the different oncogenesis between these two entities. Recognition of the pathological spectrum of ALCL is crucial to understand its pathogenesis and its boundaries with other entities. In this review, we will focus on the morphological, immunophenotypical, and molecular features of systemic ALK+ and ALK− ALCL. In addition, BI-ALCL will be discussed.
- Subjects :
- 0301 basic medicine
Cancer Research
NPM1
CD30
ALK (anaplastic lymphoma kinase)
lymphoma
Review
medicine.disease_cause
breast implant-associated ALCL
lcsh:RC254-282
Pathogenesis
03 medical and health sciences
0302 clinical medicine
hemic and lymphatic diseases
morphology
Anaplastic lymphoma kinase
Medicine
Anaplastic large-cell lymphoma
Pathological
business.industry
medicine.disease
lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens
anaplastic large cell lymphoma (ALCL)
3. Good health
Lymphoma
030104 developmental biology
Oncology
030220 oncology & carcinogenesis
Cancer research
business
Carcinogenesis
Subjects
Details
- Language :
- English
- ISSN :
- 20726694
- Volume :
- 10
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Cancers
- Accession number :
- edsair.doi.dedup.....44e4f17b19e45faffda6435f456af0ca