Details

Autor(en) / Beteiligte

• Onciu, Mihaela
• Behm, Frederick G.
• Downing, James R.
• Shurtleff, Sheila A.
• Raimondi, Susana C.
• Ma, Zhigui
• Morris, Stephan W.
• Kennedy, Wren
• Jones, Sandra C.
• Sandlund, John T.

Titel

ALK-positive plasmablastic B-cell lymphoma with expression of the NPM-ALK fusion transcript: report of 2 cases

Ist Teil von

• Blood, 2003-10, Vol.102 (7), p.2642-2644

Ort / Verlag

Washington, DC: Elsevier Inc

Erscheinungsjahr

2003

Quelle

MEDLINE

Beschreibungen/Notizen

• While most anaplastic lymphoma kinase (ALK)-positive non-Hodgkin lymphomas (NHLs) are of T-cell lineage, a small number of B-lineage tumors with plasmablastic morphology and expression of the full-length ALK protein have been described in the literature. All of these reported tumors lacked the NPM-ALK fusion transcript. There is controversy regarding the existence of ALK fusion-positive B-cell NHL, with many investigators contending that ALK fusions are expressed uniquely in T- or null-cell lymphomas. Here we describe 2 well-characterized cases of ALK-positive B-cell lymphoma expressing the NPM-ALK fusion. Both tumors occurred in pediatric patients and showed poor response to chemotherapy. Each had plasmablastic morphology, showed immunoglobulin A restriction, and was ALK positive and CD30- by immunohistochemistry. One tumor showed the t(2;5)(p23;q35) chromosomal translocation by conventional cytogenetics. Both were positive for NPM-ALK by reverse transcriptase-polymerase chain reaction. Thus, ALK-positive plasmablastic B-cell lymphomas are more heterogeneous at the molecular level than previously recognized. (Blood. 2003;102:2642-2644)