Scholarlink

<p>ALK-positive large B cell lymphoma (ALK+ LBCL) is a rare, aggressive lymphoma composed of large ALK-positive monomorphic B-cells with a relative paucity of published literature. We retrospectively reviewed the clinico-morphological and immunohistochemical (IHC) profile of a&#xa0;series of ALK+LBCL diagnosed over the last decade (2015-2025) at our centre. Moreover, data on EBERISH and FISH studies were also presented. ALK+ LBCL accounted for 6 of 1501 lymphomas (0.4%) reported over the last decade. All occurred in males with a mean age of 39 years (24-52). Five were nodal and one was extra-nodal in origin with dominant renal involvement at the time of diagnosis; only one had B symptoms, which later relapsed with bone marrow and liver metastasis. Two cases each belonged to Ann Arbor stages II, III, and IV. As per IPI, one was in high risk, and two were in high intermediate risk groups. On IHC, all cases showed a diffuse cytoplasmic granular ALK1 positivity; 5/6 were positive for MUM1 and CD138; whereas CD3, CD20, CD30, and CD45 were negative. FISH analysis performed in three cases using a break-apart probe showed ALK1 gene rearrangement, and EBERISH was negative in one case where it was performed. Three&#xa0;patients died within one year of diagnosis and treatment with CHOEP regimen.ALK+ LBCL is a rare and underdiagnosed entity that needs to be differentiated from its close mimics, such as ALCL (CD30+), plasmablastic lymphoma (ALK-, EBERISH+), and DLBCL (ALK-, CD20+), for risk stratification and appropriate management. We report six cases of ALK+ LBCL and discuss the differential diagnoses, pattern of ALK staining, pathogenesis, and potential future therapy of this aggressive lymphoma.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Anaplastic Lymphoma Kinase—Positive Large B Cell Lymphoma: An Underdiagnosed Entity

  • 期刊论文

  • 发表日期：

    2026年1月27日

查看全文
  • Pritinanda Mishra,
  • J. N. Aparnna,
  • Chinmayee Panigrahi,
  • Tapas Kanti Parida,
  • Somanath Padhi,
  • Ashutosh Panigrahi,
  • Mukund N. Sable

摘要

ALK-positive large B cell lymphoma (ALK+ LBCL) is a rare, aggressive lymphoma composed of large ALK-positive monomorphic B-cells with a relative paucity of published literature. We retrospectively reviewed the clinico-morphological and immunohistochemical (IHC) profile of a series of ALK+LBCL diagnosed over the last decade (2015-2025) at our centre. Moreover, data on EBERISH and FISH studies were also presented. ALK+ LBCL accounted for 6 of 1501 lymphomas (0.4%) reported over the last decade. All occurred in males with a mean age of 39 years (24-52). Five were nodal and one was extra-nodal in origin with dominant renal involvement at the time of diagnosis; only one had B symptoms, which later relapsed with bone marrow and liver metastasis. Two cases each belonged to Ann Arbor stages II, III, and IV. As per IPI, one was in high risk, and two were in high intermediate risk groups. On IHC, all cases showed a diffuse cytoplasmic granular ALK1 positivity; 5/6 were positive for MUM1 and CD138; whereas CD3, CD20, CD30, and CD45 were negative. FISH analysis performed in three cases using a break-apart probe showed ALK1 gene rearrangement, and EBERISH was negative in one case where it was performed. Three patients died within one year of diagnosis and treatment with CHOEP regimen.ALK+ LBCL is a rare and underdiagnosed entity that needs to be differentiated from its close mimics, such as ALCL (CD30+), plasmablastic lymphoma (ALK-, EBERISH+), and DLBCL (ALK-, CD20+), for risk stratification and appropriate management. We report six cases of ALK+ LBCL and discuss the differential diagnoses, pattern of ALK staining, pathogenesis, and potential future therapy of this aggressive lymphoma.