ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature.

Department of Pathology, University of New Mexico, Albuquerque, NM 87131, USA.
Modern Pathology (Impact Factor: 6.36). 04/2007; 20(3):310-9. DOI: 10.1038/modpathol.3800742
Source: PubMed

ABSTRACT We report detailed clinical and pathologic features of four cases of anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL), a rare entity with only 29 currently reported cases. This study is the third largest of all reported series. Biopsies from four adult patients aged 41, 49, 53, and 71 years (three lymph nodes and one nasopharyngeal mass) exhibited immunoblastic/plasmablastic morphology. By immunohistochemistry and/or flow cytometry, they expressed cytoplasmic ALK-1, CD138, VS38 (3/3), monoclonal cytoplasmic light chain, CD45, EMA, CD4, and CD57 (2/3), and were negative for CD3, CD30, CD56, and TIA-1. Two showed variable CD79a expression, and one had rare CD20(+) cells. Two of three cases exhibited rare CD43(+) reactivity. One case showed scattered cytokeratin(+) cells, which could possibly lead to a misdiagnosis of carcinoma. After CHOP and radiotherapy, two stage I patients were free of disease at 58 and 36 months, whereas a stage IV patient was dead of disease at 22 months.

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    ABSTRACT: Anaplastic lymphoma kinase-positive diffuse large B cell lymphoma represents a distinct subtype of diffuse large B cell lymphoma with a characteristic morphology, a distinct immunophenotypic profile, and recurrent cytogenetic/molecular genetic abnormalities. We report a case of anaplastic lymphoma diagnosed in the bone marrow trephine of a 24-year-old male. Histologically, the tumor cells exhibited plasmablastic morphology with expression of CD45, CD138, EMA, MUM1, and kappa immunoglobulin light chain, but negative for CD20, CD30, CD79a, PAX-5, CD10, and lambda light chain. In addition, the neoplastic cells showed positive immunoreactivity for anaplastic lymphoma kinase (ALK) with exclusive cytoplasmic granular staining pattern. Furthermore, interphase fluorescence in situ hybridization analysis, revealed ALK gene rearrangement in the lymphoid cells. The diagnosis of this rare entity in unusual extranodal sites such as the bone marrow is challenging, and it is based on detailed morphological and immunohistochemical analysis. Fluorescence in situ hybridization provides also a helpful tool in order to establish the diagnosis.
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    ABSTRACT: Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma (DLBCL) is a recently described, uncommon form of DLBCL, which has been seen primarily in young men and which presents with advanced disease. The fact that ALK-positive DLBCL is an uncommon diagnosis is likely due to the combined effects of this being an uncommon disease coupled with the challenges in the pathologic identification of this neoplasm. Prompt and accurate identification of this tumor is becoming increasingly important, however, as we enter the era of therapeutic ALK inhibitors, which are currently undergoing study in several clinical trials. Here, we report a case of ALK-positive DLBCL in a 39-year-old male patient who presented with spontaneous tumor lysis syndrome. We review the clinical, morphologic, immunohistochemical, and molecular aspects of this case and of ALK-positive DLBCL in general, with the purpose of bringing to light the existence of this disease and its potential future therapy.
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    ABSTRACT: Anaplastic large cell lymphoma (ALCL) is a common subtype of the heterogeneous group of peripheral T-cell lymphomas, which is characterized by large pleomorphic cells with strong expression of CD30. Translocations involving ALK, the anaplastic lymphoma kinase gene, are associated with a favorable clinical outcome. Such ALK-positive ALCLs are usually responsive to a multidrug chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). However, there is no general consensus on the optimal therapy for relapsed or refractory ALCL. We report the case of a 24-year-old male suffering from ALK-positive ALCL with an uncommon manifestation of only extranodal disease in the gastric cardia region that showed primary refractoriness to standard CHOP chemotherapy. A combination therapy consisting of the anti-CD30 drug conjugate, brentuximab vedotin, and classical lymphoma salvage regimen DHAP (cisplatin, high-dose cytarabine and dexamethasone) was administered. Following two treatment cycles in 21-day intervals, the lymphoma showed considerable regression based on imaging diagnostics and no evidence of vital lymphoma in a subsequent biopsy. We did not observe any increase in toxicity; in particular, polyneuropathy and febrile neutropenia were not observed. In summary, we report that the antibody-drug conjugate brentuximab vedotin and a classical regimen used for aggressive lymphoma, DHAP, could be combined as salvage therapy in a case of refractory ALK-positive ALCL. Phase I/II studies will be required for safety and efficacy analysis.
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