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ALK-positive anaplastic large-cell lymphoma: strong T and B anti-tumour responses may cause hypocellular aspects of lymph nodes mimicking inflammatory lesions

Cerato, Ch
Schwaller, J.
Wacker, P.
Brousse, N.
Published in European Journal of Haematology. 2003, vol. 71, no. 4, p. 243-249
Abstract The anaplastic large cell lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma which occurs in children mostly. The ALK protein is highly immunogenic and elicits both humoral and cellular immune responses. A 15-yr-old child presented with fever and adenopathy and did not respond to antibiotics. Biopsy of the enlarged lymph node contained almost no lymphoid element except for a few CD8-positive T cells, plasma cells and isolated CD30-positive blasts. The patient's condition improved following lymphadenectomy but relapse occurred 3 months later with multiple nodes, high fever and an abdominal mass. This time an ALK-positive ALCL was diagnosed and the retrospective analysis of the initial biopsy revealed rare, isolated ALK+ cells. Molecular analysis showed T-cell clones and oligoclonal B cells in both biopsies and peripheral blood of the patient. The tumour cells harbour a t(2;5) translocation, revealing a null phenotype by immunohistochemistry and no evidence for T-cell clonality by Southern blotting. The patient's serum contained anti-ALK antibodies. Our findings suggest that the T-cell clones and anti-ALK antibodies in this patient constitute an anti-tumour response that caused the hypocellularity of the initial lymph node. Hypocellular and oedematous lymph nodes occurring in a child with evocative symptoms should be tested for the presence of ALK.
Keywords AdolescentAntigensCD30/biosynthesisB-Lymphocytes/ immunology/metabolismBlottingSouthernFemaleHumansImmunoblottingImmunohistochemistryInflammationLymph Nodes/cytology/pathologyLymphatic MetastasisLymphomaLarge B-CellDiffuse/ genetics/ pathologyNon-Hodgkin/geneticsModelsGeneticRNAMessenger/metabolismRetrospective StudiesReverse Transcriptase Polymerase Chain ReactionT-Lymphocytes/ immunologyTime FactorsTranslocation
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PMID: 12950232
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