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Prepublished online as a Blood First Edition Paper on June 19, 2003; DOI 10.1182/blood-2003-04-1095.
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Blood, 1 October 2003, Vol. 102, No. 7, pp. 2642-2644
NEOPLASIA
Brief report
ALK-positive plasmablastic B-cell lymphoma with expression of the NPM-ALK fusion transcript: report of 2 cases
Mihaela Onciu,
Frederick G. Behm,
James R. Downing,
Sheila A. Shurtleff,
Susana C. Raimondi,
Zhigui Ma,
Stephan W. Morris,
Wren Kennedy,
Sandra C. Jones, and
John T. Sandlund
From the Departments of Pathology and Hematology/Oncology at St Jude Children's Research Hospital, Memphis, TN; and the University of Tennessee Health Sciences Center, Memphis, TN.
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)
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