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ALK -positive diffuse large B-cell lymphoma

Written2010-07Antonio Cuneo, Gian Matteo Rigolin, Maria Ciccone
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

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ICD-Morpho 9737/3 ALK positive large B-cell lymphoma
Atlas_Id 1552

Clinics and Pathology

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).


Cytogenetics Molecular The Ig gene is clonally rearranged.
The founder lesion involves the ALK gene on chromosome 2 (Lee et al., 2008).
The t(2;17)(p23;q23) determining the clathrin/ALK fusion is the most frequent aberration (Chikatsu et al., 2003), accounting for approximately 70% of the cases.
The classical t(2;5)(p23;q35) was reported in 10% of the cases (Adam et al., 2003).
Other aberrations, mostly reported in individual cases include:
- cryptic insertion of the ALK gene into chromosome 4q22 (Stachurski et al., 2007),
- cryptic SEC31A-ALK fusion generated by an insertion of the 5' end of SEC31A (4q21) upstream of the 3' end of ALK. An in-frame fusion transcript in which exon 24 of SEC31A (4q21) was fused to exon 20 of ALK is generated by this rearrangement (Van Roosbroeck 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
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
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.
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
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
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
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


This paper should be referenced as such :
Cuneo, A ; Rigolin, GM ; Ciccone, M
ALK -positive diffuse large B-cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2011;15(4):352-353.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes EEF1D

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap (UZH-SIB Zurich)Topo ( C42) Morph ( 9737/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
REVIEW articlesautomatic search in PubMed
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