ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India
ALK-positive large B-cell lymphoma (ALK+ LBCL) is a rare neoplasm with an aggressive course and poor therapeutic response to the standard R-CHOP regimen. Owing to its negativity for usual B- and T-cell markers and immunopositivity for epithelial markers, it can be easily misdiagnosed if it is not co...
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Wolters Kluwer Medknow Publications
2025-01-01
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| Series: | Indian Journal of Pathology and Microbiology |
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| Online Access: | https://journals.lww.com/10.4103/ijpm.ijpm_369_24 |
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| author | Ajita Verma Zachariah Chowdhury Anil Singh Abhinav Kant Anil Yadav Shashikant C.U. Patne |
| author_facet | Ajita Verma Zachariah Chowdhury Anil Singh Abhinav Kant Anil Yadav Shashikant C.U. Patne |
| author_sort | Ajita Verma |
| collection | DOAJ |
| description | ALK-positive large B-cell lymphoma (ALK+ LBCL) is a rare neoplasm with an aggressive course and poor therapeutic response to the standard R-CHOP regimen. Owing to its negativity for usual B- and T-cell markers and immunopositivity for epithelial markers, it can be easily misdiagnosed if it is not contemplated. To study the clinicopathological parameters of cases of ALK+ LBCL diagnosed at our institution. A retrospective observational study of ALK+ LBCL was conducted at a tertiary cancer center of North India with cases diagnosed over a period of 3 years. Six cases of ALK+ LBCL were identified. The clinical findings at presentation included mean age of 38.8 years, male-to-female ratio of 5:1, extranodal presentation (1/6 cases), concurrent extranodal and nodal involvement (3/6), nodal presentation (2/6), high serum LDH (5/5), and bone marrow involvement (1/5). Histomorphology of diffuse (100%), alveolar/nested (16.6%), and sinusoidal pattern (1 case upon relapse) and immunoblastic and plasmablastic morphology (100%) and immunopositivity in all cases for ALK-1 protein (100%), CD138 (100%), MUM1 (100%), LCA (100%) along with negativity for EBER-ISH/EBV-LMP1 immunohistochemistry clinched the diagnosis. Fluorescence in situ hybridization analysis for ALK gene rearrangement was detected in 4/4 cases. Four patients received chemotherapy demonstrating relapse in 2 cases: residual disease and no response in one case each, along with death in 2 cases. A high degree of diagnostic suspicion is required for accurate recognition of ALK+ LBCL. Awareness of its histology, immunohistochemistry, and cytogenetics is pivotal for precise identification of this rare entity. |
| format | Article |
| id | doaj-art-9c4d227479124ad4bfe0b16b7717bb35 |
| institution | DOAJ |
| issn | 0377-4929 0974-5130 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj-art-9c4d227479124ad4bfe0b16b7717bb352025-08-20T03:06:19ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49290974-51302025-01-0168114114610.4103/ijpm.ijpm_369_24ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North IndiaAjita VermaZachariah ChowdhuryAnil SinghAbhinav KantAnil YadavShashikant C.U. PatneALK-positive large B-cell lymphoma (ALK+ LBCL) is a rare neoplasm with an aggressive course and poor therapeutic response to the standard R-CHOP regimen. Owing to its negativity for usual B- and T-cell markers and immunopositivity for epithelial markers, it can be easily misdiagnosed if it is not contemplated. To study the clinicopathological parameters of cases of ALK+ LBCL diagnosed at our institution. A retrospective observational study of ALK+ LBCL was conducted at a tertiary cancer center of North India with cases diagnosed over a period of 3 years. Six cases of ALK+ LBCL were identified. The clinical findings at presentation included mean age of 38.8 years, male-to-female ratio of 5:1, extranodal presentation (1/6 cases), concurrent extranodal and nodal involvement (3/6), nodal presentation (2/6), high serum LDH (5/5), and bone marrow involvement (1/5). Histomorphology of diffuse (100%), alveolar/nested (16.6%), and sinusoidal pattern (1 case upon relapse) and immunoblastic and plasmablastic morphology (100%) and immunopositivity in all cases for ALK-1 protein (100%), CD138 (100%), MUM1 (100%), LCA (100%) along with negativity for EBER-ISH/EBV-LMP1 immunohistochemistry clinched the diagnosis. Fluorescence in situ hybridization analysis for ALK gene rearrangement was detected in 4/4 cases. Four patients received chemotherapy demonstrating relapse in 2 cases: residual disease and no response in one case each, along with death in 2 cases. A high degree of diagnostic suspicion is required for accurate recognition of ALK+ LBCL. Awareness of its histology, immunohistochemistry, and cytogenetics is pivotal for precise identification of this rare entity.https://journals.lww.com/10.4103/ijpm.ijpm_369_24alk d5f3alk-1cd-138crizotinibmum-1 |
| spellingShingle | Ajita Verma Zachariah Chowdhury Anil Singh Abhinav Kant Anil Yadav Shashikant C.U. Patne ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India Indian Journal of Pathology and Microbiology alk d5f3 alk-1 cd-138 crizotinib mum-1 |
| title | ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India |
| title_full | ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India |
| title_fullStr | ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India |
| title_full_unstemmed | ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India |
| title_short | ALK-positive large B-cell lymphoma: A study of six cases from an oncopathology center in North India |
| title_sort | alk positive large b cell lymphoma a study of six cases from an oncopathology center in north india |
| topic | alk d5f3 alk-1 cd-138 crizotinib mum-1 |
| url | https://journals.lww.com/10.4103/ijpm.ijpm_369_24 |
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