ALK+ large B-cell lymphoma

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]

Biology

The median age of diagnosis is approximately late thirties to early forties.[2]:378[3][5] The estimates of childhood disease vary (8%,[7] 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

Upregulation of ALK is mainly due to chromosomal translocation t(2;17), resulting in a fusion gene of CLTC with ALK,[4][9] 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][9] 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

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][7][9] particularly in higher stage disease.[7]

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See also

References
  1. 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. 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310.
  2. Jaffe, ES; Harris, NL; Vardiman, JW; Campo, E; Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. 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.
  4. 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. PMID 9057627.
  5. 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.
  6. 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.
  7. 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.
  8. Armitage, JO; Mauch, PM; Harris, NL; et al. (2010). "Chapter 20". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
  9. 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.
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