ALK -positive diffuse large B-cell lymphoma

2010-07-01   Gian Matteo Rigolin , Maria Ciccone , Antonio Cuneo 

1.Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

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 stem cell 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).

Bibliography

Pubmed IDLast YearTitleAuthors
145764832003A case of a diffuse large B-cell lymphoma of plasmablastic type associated with the t(2;5)(p23;q35) chromosome translocation.Adam P et al
129202292003ALK+, CD30-, CD20- large B-cell lymphoma containing anaplastic lymphoma kinase (ALK) fused to clathrin heavy chain gene (CLTC).Chikatsu N et al
196360072009Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma: a rare clinicopathologic entity with poor prognosis.Laurent C et al
182203222008ALK-positive diffuse large B-cell lymphoma: report of three cases.Lee HW et al
175093952007Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma with a complex karyotype and cryptic 3' ALK gene insertion to chromosome 4 q22-24.Stachurski D et al
202078482010ALK-positive large B-cell lymphomas with cryptic SEC31A-ALK and NPM1-ALK fusions.Van Roosbroeck K et al

Citation

Gian Matteo Rigolin ; Maria Ciccone ; Antonio Cuneo

ALK -positive diffuse large B-cell lymphoma

Atlas Genet Cytogenet Oncol Haematol. 2010-07-01

Online version: http://atlasgeneticsoncology.org/haematological/1552/alk-positive-diffuse-large-b-cell-lymphoma

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