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Prepublished online as a Blood First Edition Paper on June 19, 2003; DOI 10.1182/blood-2003-04-1095.
Submitted April 9, 2003
Accepted May 15, 2003
ALK-positive plasmablastic B-cell lymphoma with expression of the NPM-ALK fusion transcript: Report of two 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
Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA
Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA; University of Tennessee Health Sciences Center, Memphis, TN, USA
Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA
Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA; University of Tennessee Health Sciences Center, Memphis, TN, USA
* Corresponding author; email: mihaela.onciu{at}stjude.org.
While most ALK-positive Non-Hodgkin lymphomas (NHL) 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, many investigators contending that ALK fusions are expressed uniquely in T- or null-cell lymphomas. Here we describe two 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 IgA restriction, and was ALK-positive and CD30-negative by immunohistochemistry. One tumor showed the t(2;5)(p23;q35) chromosomal translocation by conventional cytogenetics. Both were positive for NPM-ALK by RT-PCR. Thus, ALK-positive plasmablastic B-cell lymphomas are more heterogeneous at the molecular level than previously recognized.
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