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Primary Effusion Lymphoma (PEL)-Like Lymphoma in a Child With Congenital Immunodeficiency

Grace K S Lam1, Mohamed Abdelhaleem2, Gino R Somers2

  • 1Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.

Pediatric Blood & Cancer
|May 18, 2016
PubMed

Insights

This report details the first pediatric case of a rare Epstein-Barr virus positive/human herpes virus 8 negative Primary Effusion Lymphoma (PEL)-like lymphoma in a 9-year-old girl with combined immunodeficiency. Standard treatments proved ineffective, highlighting the need for new strategies for this rare lymphoma.

Area of Science:

  • Pediatric Oncology
  • Immunology
  • Virology

Background:

  • Primary Effusion Lymphoma (PEL) is a rare, aggressive non-Hodgkin lymphoma typically affecting immunocompromised adults.
  • PEL is strongly associated with human herpes virus 8 (HHV-8) infection.
  • Combined immunodeficiency (CID) predisposes individuals to various opportunistic infections and malignancies.

Observation:

  • A 9-year-old female with CID presented with a PEL-like lymphoma.
  • The lymphoma was characterized as Epstein-Barr virus (EBV) positive and HHV-8 negative.
  • Standard treatments including chemotherapy for non-Hodgkin lymphoma, zidovudine, and interferon-α were administered.

Findings:

  • The patient's disease progressed despite aggressive multimodal therapy.
  • This represents the first reported pediatric case of an EBV-positive/HHV-8-negative PEL-like lymphoma.
  • The case highlights a potential variant of PEL or a PEL-mimicking condition in pediatric populations.

Implications:

  • Increased diagnostic vigilance for PEL-like lymphomas in immunocompromised children is warranted.
  • Current treatment protocols may be insufficient for this rare pediatric lymphoma variant.
  • Further research into the pathogenesis and effective therapeutic strategies for EBV-positive/HHV-8-negative PEL-like lymphomas is crucial.

Keywords:
EBVchildrenlymphomaprimary effusion

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