Anaplastic large cell lymphoma

Anaplastic large cell lymphoma
Updated: 08/20/2024
© Jun Wang, MD, PhD

General features
  • Moderately aggressive
  • Likely in young population, men
  • Both nodal and extranodal involvement
  • Most common extranodal sites include skin, bone
  • Morphologically similar to Hodgkin lymphoma
Key pathogenesis
  • Translocation involving ALK (anaplastic lymphoma receptor tyrosine kinase)
  • Abnormal activation of MAPK pathway
Key morphological features
  • Markedly pleomorphic bizarre nuclei:  Horseshoe ("hallmark") cells, wreath-like or multiple nuclei, multiple nucleoli, perinuclear eosinophilic region, occasional nuclear pseudoinclusions ("doughnut" cells)
Markers
  • Positive: CD30, ALK, T-cell markers such as CD5, CD45 (A marker negative in classic type Hodgkin lymphoma)
  • Negative: B cell markers, such as CD20
Genetic abnormalities
  • Translocation involving ALK gene
  • T cell receptor (TCR) gene rearrangement

ALK- anaplastic large cell lymphoma

  • T cell neoplasm with bizarre nuclei but ALK negative
  • More common in 40 - 65 years, slightly more common in male
  • Probably originates from activated T cells
  • Commonly loss of CD3 and other T cell markers
  • Gains of the 1q41-qter and 6p21 common
  • Other molecular abnormalities include: rearrangement involving IRF4 or TP63, and activating mutations of STAT3
  • Morphologically identical with ALK positive ALCL 
  • Positive for CD30, CD45
  • Negative for ALK, EBV
  • Chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) or CHOP plus etoposide (CHOEP)


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