ALK+ large B-cell lymphoma is a type of lymphoma. [1] [2]: 378 It was first reported in 1997. [2]: 378 [3] [4] It is a rare, aggressive large B-cell process that shows ALK expression. [2]: 378 [3] [5] It is distinct from anaplastic large cell lymphoma, a T-cell lymphoma. [2]: 564 [3] [6]
Signs and symptoms
![[icon]](/info/en/?search=File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by
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Pathophysiology
Upregulation of ALK is mainly due to chromosomal translocation t(2;17), resulting in a fusion gene of CLTC with ALK, [4] [7] but can rarely be due to t(2;5), fusing NPM1 with ALK; [2]: 378 the later is the usual finding in anaplastic large cell lymphoma (ALCL). [4] [7] The t(2;17) translocation occurs in less than 1% of cases of ALK+ ALCL, but has been identified in inflammatory myofibroblastic tumors. [3]
There is no association with Epstein–Barr virus [2]: 378 [6] or HHV8, [6] or immunosuppression. [2]: 378 The cells are CD20 and CD30 negative, [8]: 306 showing weak focal expression in 3% and 6% respectively. [2]: 378 They are EMA and CD138 positive, [8]: 306 showing 100% expression respectively. [2]: 378
Diagnosis
The median age of diagnosis is approximately late thirties to early forties. [2]: 378 [3] [5] The estimates of childhood disease vary (8%, [9] 15%, [3] 30% [2]: 378 ) but it can be seen at any age. [5] [8]: 306
The disease usually arises in lymph nodes, particularly the neck, but extranodal involvement, including in the gastrointestinal tract, nasal cavity, ovary and brain, has been described. [3] [5] Morphologically, there are large immunoblast-like cells with large central nucleoli, often cellular clusters, with a predilection for the lymph node sinuses [2]: 378 [4] [8]: 306 in a cohesive pattern that can suggest carcinoma cells. [2]: 378 [8]: 306
Treatment
Multiagent chemotherapy is given, and can result in long-term success, particularly in childhood [8]: 306 but prognosis is generally poor, [2]: 378 [3] [5] [9] [7] particularly in higher stage disease. [9]
See also
References
- ^ Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. Vol. 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310. Archived from the original on October 12, 2013.
- ^ a b c d e f g h i j k l m n Jaffe, ES; Harris, NL; Vardiman, JW; Campo, E; Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
- ^ a b c d e f g h Morgan EA, Nascimento AF (2012). "Anaplastic lymphoma kinase-positive large B-cell lymphoma: an underrecognized aggressive lymphoma". Adv Hematol. 2012: 529572. doi: 10.1155/2012/529572. PMC 3299366. PMID 22474449.
- ^ a b c d Delsol G, Lamant L, Mariamé B, et al. (1997). "A new subtype of large B-cell lymphoma expressing the ALK kinase and lacking the 2; 5 translocation". Blood. 89 (5): 1483–90. doi: 10.1182/blood.V89.5.1483. PMID 9057627.
- ^ a b c d e Xing X, Lin D, Ran W, Liu H (2014). "ALK-positive diffuse large B-cell lymphoma of the duodenum: A case report and review of the literature". Exp Ther Med. 8 (2): 409–412. doi: 10.3892/etm.2014.1786. PMC 4079440. PMID 25009592.
- ^ a b c Beltran B, Castillo J, Salas R, et al. (2009). "ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature". J Hematol Oncol. 2: 11. doi: 10.1186/1756-8722-2-11. PMC 2651189. PMID 19250532.
- ^ a b c Murga-Zamalloa C, Lim MS (2014). "ALK-driven tumors and targeted therapy: focus on crizotinib". Pharmgenomics Pers Med. 7: 87–94. doi: 10.2147/PGPM.S37504. PMC 3977456. PMID 24715763.
- ^ a b c d e f Armitage, JO; Mauch, PM; Harris, NL; et al. (2010). "Chapter 20". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
- ^ a b c Laurent C, Do C, Gascoyne RD, et al. (2009). "Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma: a rare clinicopathologic entity with poor prognosis". J. Clin. Oncol. 27 (25): 4211–6. doi: 10.1200/JCO.2008.21.5020. PMID 19636007.
ALK+ large B-cell lymphoma is a type of lymphoma. [1] [2]: 378 It was first reported in 1997. [2]: 378 [3] [4] It is a rare, aggressive large B-cell process that shows ALK expression. [2]: 378 [3] [5] It is distinct from anaplastic large cell lymphoma, a T-cell lymphoma. [2]: 564 [3] [6]
Signs and symptoms
|
| This section is empty. You can help by
adding to it. (November 2021) |
Pathophysiology
Upregulation of ALK is mainly due to chromosomal translocation t(2;17), resulting in a fusion gene of CLTC with ALK, [4] [7] but can rarely be due to t(2;5), fusing NPM1 with ALK; [2]: 378 the later is the usual finding in anaplastic large cell lymphoma (ALCL). [4] [7] The t(2;17) translocation occurs in less than 1% of cases of ALK+ ALCL, but has been identified in inflammatory myofibroblastic tumors. [3]
There is no association with Epstein–Barr virus [2]: 378 [6] or HHV8, [6] or immunosuppression. [2]: 378 The cells are CD20 and CD30 negative, [8]: 306 showing weak focal expression in 3% and 6% respectively. [2]: 378 They are EMA and CD138 positive, [8]: 306 showing 100% expression respectively. [2]: 378
Diagnosis
The median age of diagnosis is approximately late thirties to early forties. [2]: 378 [3] [5] The estimates of childhood disease vary (8%, [9] 15%, [3] 30% [2]: 378 ) but it can be seen at any age. [5] [8]: 306
The disease usually arises in lymph nodes, particularly the neck, but extranodal involvement, including in the gastrointestinal tract, nasal cavity, ovary and brain, has been described. [3] [5] Morphologically, there are large immunoblast-like cells with large central nucleoli, often cellular clusters, with a predilection for the lymph node sinuses [2]: 378 [4] [8]: 306 in a cohesive pattern that can suggest carcinoma cells. [2]: 378 [8]: 306
Treatment
Multiagent chemotherapy is given, and can result in long-term success, particularly in childhood [8]: 306 but prognosis is generally poor, [2]: 378 [3] [5] [9] [7] particularly in higher stage disease. [9]
See also
References
- ^ Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. Vol. 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310. Archived from the original on October 12, 2013.
- ^ a b c d e f g h i j k l m n Jaffe, ES; Harris, NL; Vardiman, JW; Campo, E; Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
- ^ a b c d e f g h Morgan EA, Nascimento AF (2012). "Anaplastic lymphoma kinase-positive large B-cell lymphoma: an underrecognized aggressive lymphoma". Adv Hematol. 2012: 529572. doi: 10.1155/2012/529572. PMC 3299366. PMID 22474449.
- ^ a b c d Delsol G, Lamant L, Mariamé B, et al. (1997). "A new subtype of large B-cell lymphoma expressing the ALK kinase and lacking the 2; 5 translocation". Blood. 89 (5): 1483–90. doi: 10.1182/blood.V89.5.1483. PMID 9057627.
- ^ a b c d e Xing X, Lin D, Ran W, Liu H (2014). "ALK-positive diffuse large B-cell lymphoma of the duodenum: A case report and review of the literature". Exp Ther Med. 8 (2): 409–412. doi: 10.3892/etm.2014.1786. PMC 4079440. PMID 25009592.
- ^ a b c Beltran B, Castillo J, Salas R, et al. (2009). "ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature". J Hematol Oncol. 2: 11. doi: 10.1186/1756-8722-2-11. PMC 2651189. PMID 19250532.
- ^ a b c Murga-Zamalloa C, Lim MS (2014). "ALK-driven tumors and targeted therapy: focus on crizotinib". Pharmgenomics Pers Med. 7: 87–94. doi: 10.2147/PGPM.S37504. PMC 3977456. PMID 24715763.
- ^ a b c d e f Armitage, JO; Mauch, PM; Harris, NL; et al. (2010). "Chapter 20". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
- ^ a b c Laurent C, Do C, Gascoyne RD, et al. (2009). "Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma: a rare clinicopathologic entity with poor prognosis". J. Clin. Oncol. 27 (25): 4211–6. doi: 10.1200/JCO.2008.21.5020. PMID 19636007.