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ALK-positive large B-cell lymphomas: A clinicopathologic study.

Jagan Chandramohan1, Gautami Ganapule1, Elanthenral Sigamani1

  • 1Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India.

Indian Journal of Pathology & Microbiology
|April 18, 2022
PubMed
Summary

Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphomas (ALK+-LBCLs) are rare, aggressive, and often misdiagnosed CD20-negative lymphomas. This study analyzed 11 cases, highlighting their unique morphology and poor treatment outcomes.

Keywords:
Aggressive B-cell non-Hodgkin lymphomaCD20-negative large B-cell lymphomaanaplastic lymphoma kinaselarge cell lymphoma with plasmablastic differentiation

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphomas (ALK+-LBCLs) are rare, aggressive CD20-negative lymphomas, comprising less than 1% of diffuse large B-cell lymphomas.
  • Their peculiar immunophenotypic characteristics contribute to frequent misdiagnosis.

Purpose of the Study:

  • To analyze the clinical, morphological, and immunophenotypic profile of ALK+-LBCLs.
  • To understand the diagnostic challenges and outcomes of these rare lymphomas.

Main Methods:

  • Retrospective analysis of 11 ALK+-LBCL cases diagnosed between September 2009 and August 2020.
  • Review of clinical data, hematoxylin and eosin (H&E) stained slides, and immunohistochemistry.

Main Results:

  • Seven of eleven patients presented with extranodal disease.
  • Tumors exhibited immunoblastic and plasmablastic morphology with ALK-1 positivity, CD20 negativity, and plasmablastic differentiation.
  • Only one of eight treated patients achieved short-term remission; two died of disease, and five had progressive disease.

Conclusions:

  • ALK+-LBCLs require consideration in the differential diagnosis of tumors with plasmablastic and immunoblastic morphology, particularly in extranodal sites.
  • Early and accurate diagnosis is crucial to avoid delays and improve patient management, despite poor treatment response observed.