Non-Hodgkin lymphoma [DS:H02418] Primary central nervous system lymphoma [DS:H02424]
Description
Burkitt lymphoma (BL) is a highly aggressive mature B-cell non-Hodgkin's lymphoma consisting of endemic, sporadic, and immunodeficiency-associated variants. Endemic BL (eBL) affects children and young adults in Africa and some other geographical areas and carries Epstein-Barr virus (EBV) in more than 95% of cases. In contrast, sporadic BL (sBL) among adolescents in Europe and North America are mostly EBV-negative. A third type of BL is associated with HIV-infection in adults. All of these subtypes possess chromosomal rearrangements of the c-myc oncogene, the genetic hallmark of BL that contributes to lymphomagenesis through alterations in cell cycle regulation, cellular differentiation, apoptosis, cellular adhesion, and metabolism. Many BL carry point mutation in the p53 tumor suppressor gene or other defects in the p14ARF-MDM2-p53 pathway, and inactivation of the p16INK4a gene by promoter methylation or homozygous deletion. This indicates that disruption of both the pRb and p53 tumor suppressor pathways is critical for BL development.
Category
Cancer
Brite
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Neoplasms of haematopoietic or lymphoid tissues
Mature B-cell neoplasms
2A85 Other specified mature B-cell neoplasms or lymphoma
H00008 Burkitt lymphoma
Pathway-based classification of diseases [BR:br08402]
Signal transduction
nt06518 JAK-STAT signaling
H00008 Burkitt lymphoma
nt06516 TNF signaling
H00008 Burkitt lymphoma
Cellular process
nt06524 Apoptosis
H00008 Burkitt lymphoma
Immune system
nt06517 TLR signaling
H00008 Burkitt lymphoma
nt06519 RLR signaling
H00008 Burkitt lymphoma
nt06537 TCR/BCR signaling
H00008 Burkitt lymphoma
Tumor markers [br08442.html]
H00008
Hodgkin's lymphoma (HL) is one of the most frequent lymphomas in the Western world and often affects young adults. HL is subdivided into classical and nodular lymphocyte-predominant forms. About 95% of cases are classical HL, and 5% are nodular lymphocyte-predominant HL (NLPHL). A characteristic feature of HL is the rareness of the tumor cells, which are called Hodgkin's and Reed/Sternberg (HRS) cells in classical HL and lymphocytic and histiocytic (L&H) cells in NLPHL. These cells represent only about 1% of the cellular infiltrate, while the vast majority of infiltrating cells are T lymphocytes, histiocytes, eosinophilic granulocytes and plasma cells. HRS cells show constitutive activity of both the classical and alternative NF-{kappa}B signalling pathways, which is probably a major pathogenetic mechanism in Hodgkin's lymphoma. The NF-{kappa}B activity in HRS cells is probably mediated by diverse mechanisms: receptor signalling through CD40, RANK, BCMA, and TACI, genomic REL amplification, destructive mutations in IKBA and IKBE. In HL pathogenesis associated with Epstein-Barr virus infection, the activation of NF-{kappa}B is induced by viral latent membrane protein 1 (LMP1).
Category
Cancer
Brite
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Neoplasms of haematopoietic or lymphoid tissues
2B30 Hodgkin lymphoma
H00007 Hodgkin lymphoma
Pathway-based classification of diseases [BR:br08402]
Signal transduction
nt06518 JAK-STAT signaling
H00007 Hodgkin lymphoma
nt06516 TNF signaling
H00007 Hodgkin lymphoma
Cellular process
nt06524 Apoptosis
H00007 Hodgkin lymphoma
Immune system
nt06517 TLR signaling
H00007 Hodgkin lymphoma
nt06519 RLR signaling
H00007 Hodgkin lymphoma
nt06537 TCR/BCR signaling
H00007 Hodgkin lymphoma
Nasopharyngeal carcinoma (NPC) is a rare disease in most parts of the world, with an age-standardised annual incidence of less than 1 per 100000. However, in Southern China, parts of Southeast Asia and the Mediterranean basin, NPC is an endemic disease with an incidence of 10-30 per 100000. Genome-wide studies have unravelled multiple chromosomal abnormalities with involvement of specific oncogenes and tumor suppressor genes. Alterations of genes such as Ras association domain family 1A (RASSF1A), p16/INK4A, p14/ARF suggest that multiple cellular pathways were dysregulated in the NPC cells. Studies on the precancerous lesions revealed early genetic changes and a critical role of Epstein- Barr virus (EBV) latent infection in the development of this cancer.
Category
Cancer
Brite
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Malignant neoplasms, except primary neoplasms of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms of lip, oral cavity or pharynx
2B6B Malignant neoplasms of nasopharynx
H00054 Nasopharyngeal cancer
Pathway-based classification of diseases [BR:br08402]
Signal transduction
nt06518 JAK-STAT signaling
H00054 Nasopharyngeal cancer
nt06516 TNF signaling
H00054 Nasopharyngeal cancer
Cellular process
nt06524 Apoptosis
H00054 Nasopharyngeal cancer
Immune system
nt06517 TLR signaling
H00054 Nasopharyngeal cancer
nt06519 RLR signaling
H00054 Nasopharyngeal cancer
nt06537 TCR/BCR signaling
H00054 Nasopharyngeal cancer
Dai W, Zheng H, Cheung AK, Tang CS, Ko JM, Wong BW, Leong MM, Sham PC, Cheung F, Kwong DL, Ngan RK, Ng WT, Yau CC, Pan J, Peng X, Tung S, Zhang Z, Ji M, Chiang AK, Lee AW, Lee VH, Lam KO, Au KH, Cheng HC, Yiu HH, Lung ML
Title
Whole-exome sequencing identifies MST1R as a genetic susceptibility gene in nasopharyngeal carcinoma.
