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Related Experiment Videos

Hydroa-like lymphoma with CD56 expression.

Katherine Doeden1, Hernan Molina-Kirsch, Edgar Perez

  • 1Department of Pathology, Stanford University Medical Center, Stanford, CA 94305, USA. ksdoeden@hotmail.com

Journal of Cutaneous Pathology
|November 3, 2007
PubMed
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Hydroa-like lymphoma, a rare aggressive childhood cancer, can present with unusual CD56+ natural killer cell markers. These cases suggest a potentially better prognosis than typical aggressive natural killer cell lymphomas.

Area of Science:

  • Pediatric Oncology
  • Dermatology
  • Hematology

Background:

  • Hydroa-like lymphoma is an extremely rare, aggressive T-cell lymphoma primarily affecting children in Latin America and Asia.
  • It is characterized by ulcerative blistering lesions on sun-exposed and non-sun-exposed skin.
  • Unlike classical hydroa vacciniforme, it is associated with systemic T-cell lymphoma expressing CD4 or CD8.

Observation:

  • Two pediatric cases of hydroa-like lymphoma presented with atypical CD56 expression, resembling natural killer (NK) cell lymphomas.
  • Patients, both 9-year-old boys, had a history of ulcerative blistering lesions since age 3.
  • Skin biopsies revealed atypical lymphocytes with a CD3+, CD56+, CD30+ phenotype, lacking CD4 and CD8.

Findings:

  • The study identified two unusual cases of hydroa-like lymphoma with a natural killer cell phenotype (CD56+).

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  • These cases differed from previously described hydroa-like lymphomas, which are typically T-cell derived (CD4+ or CD8+).
  • Both patients remained alive with disease one year post-diagnosis.

    • Implications:

    • Hydroa-like lymphoma with an NK-cell phenotype may share similar outcomes to T-cell derived hydroa-like lymphoma.
    • The prognosis for this NK-cell variant might be more favorable than for classical aggressive NK cell lymphomas.
    • This finding expands the understanding of hydroa-like lymphoma subtypes and their clinical behavior.