Abstract
We retrospectively analysed 17 cases of anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+, LBCL) according to the morphological, immunohistochemical, molecular and clinical features, using which we intend to elucidate the clinicopathological characteristics of this rare entity. In this study, all cases de facto share common features that defined them as a single entity, and various characteristics may expand the spectrum. Among 15 cases, 60% followed an aggressive clinical course with advanced stage and high IPI scores; the median survival of these patients was only 8 months. An analysis showed that both the IPI score and the Ann Arbor stage were significant prognostic factors. Most patients received a chemotherapy regimen including CHOP, CHOEP, EPOCH, and CVAD, and some also underwent localized radiotherapy. However, ALK+, LBCL cases display a dismal clinical outcome and can only be cured with conventional chemotherapy protocols at the stage of localized disease. Novel front-line intensive chemotherapy regimens should therefore be evaluated in this group of patients.
Highlights
Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+, LBCL) was originally recognized in 1997 by Delsol and colleagues [1] and was listed as a distinct entity in the updated WHO Classification of Haematopoietic and Lymphoid Tissues [2]. It appears to be very rare because it represents less than 1% of all diffuse large B cell lymphomas (DLBCLs), which is partially due to the under-recognition of this disease [2]
Thirteen cases presented as lymphadenopathy, of which 10 cases were located in the cervical region; 5 cases presented as retroperitoneal lymphadenopathy accompanied by abdominal pain and inguinal lymph node enlargement
The serum lactate dehydrogenase (LDH) level was elevated in 15 documented patients, and HIV serology was negative
Summary
Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+, LBCL) was originally recognized in 1997 by Delsol and colleagues [1] and was listed as a distinct entity in the updated WHO Classification of Haematopoietic and Lymphoid Tissues [2]. It appears to be very rare because it represents less than 1% of all diffuse large B cell lymphomas (DLBCLs), which is partially due to the under-recognition of this disease [2]. The tumour cells co-express ALK (the staining pattern changes according to the gene fusion type and often shows a restricted cytoplasmic staining pattern), a panel of plasma cell markers, CD45, epithelial membrane antigen (EMA) and often contain single light-chain cytoplasmic immunoglobulin A
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