| Disease | Anaplastic lymphoma kinase (ALK) -positive diffuse large B-cell lymphoma (DLBCL) is a variant of DLBCL, expressing the ALK kinase (Delsol et al., 2008). |
| Phenotype / cell stem origin | The postulated normal counterpart is post-germinal centre B cell with features of plasma cell differentiation.
The immunophenotype typically show ALK+ (cytoplasmic staining). Plasma cell markers such as CD138 test positive, whereas B-cell markers such as CD20 and CD79a are negative, CD30 is usually negative. Cytoplasmic staining for IgA and, less frequently, IgG is typically seen in the majority of cases. |
| Clinics | The disease runs an aggressive course (Laurent et al., 2010). Long term survival was reported in some children. |
| Pathology | The lymph node sections show large immunoblastic cells with nucleoli. |
| Treatment | Chemotherapy using CHOP or CHOP-like regimens is the standard of care. Due to CD20 negativity, the monoclonal antibody rituximab is ineffective. |
| Prognosis | Median overall survival was 20 months in a study (Laurent et al., 2010). |
| A case of a diffuse large B-cell lymphoma of plasmablastic type associated with the t(2;5)(p23;q35) chromosome translocation. |
| Adam P, Katzenberger T, Seeberger H, Gattenlohner S, Wolf J, Steinlein C, Schmid M, Muller-Hermelink HK, Ott G. |
| Am J Surg Pathol. 2003 Nov;27(11):1473-6. |
| PMID 14576483 |
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| ALK+, CD30-, CD20- large B-cell lymphoma containing anaplastic lymphoma kinase (ALK) fused to clathrin heavy chain gene (CLTC). |
| Chikatsu N, Kojima H, Suzukawa K, Shinagawa A, Nagasawa T, Ozawa H, Yamashita Y, Mori N. |
| Mod Pathol. 2003 Aug;16(8):828-32. |
| PMID 12920229 |
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| Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma with a complex karyotype and cryptic 3' ALK gene insertion to chromosome 4q22-24. |
| Stachurski D, Miron PM, Al-Homsi S, Hutchinson L, Harris NL, Woda B, Wang SA. |
| Hum Pathol. 2007 Jun;38(6):940-5. |
| PMID 17509395 |
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| ALK-positive large B-cell lymphoma. |
| Delsol G, Campo E, Gascoyne RD. |
| Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, ed. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. WHO press, 4th ed. Lyon: IARC. 2008; pp 254-255. |
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| ALK-positive diffuse large B-cell lymphoma: report of three cases. |
| Lee HW, Kim K, Kim W, Ko YH. |
| Hematol Oncol. 2008 Jun;26(2):108-13. |
| PMID 18220322 |
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| Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma: a rare clinicopathologic entity with poor prognosis. |
| Laurent C, Do C, Gascoyne RD, Lamant L, Ysebaert L, Laurent G, Delsol G, Brousset P. |
| J Clin Oncol. 2009 Sep 1;27(25):4211-6. Epub 2009 Jul 27. Erratum in: J Clin Oncol. 2010 Jan 1;28(1):182. |
| PMID 19636007 |
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| ALK-positive large B-cell lymphomas with cryptic SEC31A-ALK and NPM1-ALK fusions. |
| Van Roosbroeck K, Cools J, Dierickx D, Thomas J, Vandenberghe P, Stul M, Delabie J, De Wolf-Peeters C, Marynen P, Wlodarska I. |
| Haematologica. 2010 Mar;95(3):509-13. |
| PMID 20207848 |
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