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CD3-negative lymphoproliferative disease of granular lymphocytes in a girl with an unusual pattern of anti-Epstein-Barr virus antibodies.

AbstractUNLABELLED:
We describe an 11-year-old girl who initially had mild hepatosplenomegaly and then presented with abnormal expansion of CD3-negative granular lymphocytes in peripheral blood and Epstein-Barr virus (EBV) genome in the DNA obtained from the peripheral blood mononuclear cells (PBMNC). After approximately 3 years, she developed oedema, ascites, marked hepatosplenomegaly, and pancytopenia, and showed both a profile of anti-EBV antibodies of reactivated infection and a high titre of anti-cytomegalovirus antibody. Although she was treated with antibiotics, ganciclovir, and prednisolone, she died of hepatic failure.
CONCLUSION:
Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes.
AuthorsY Fukunaga, T Asano, J Takehana, K Ambo, K Matsuoka, M Yamamoto
JournalEuropean journal of pediatrics (Eur J Pediatr) Vol. 153 Issue 12 Pg. 894-7 (Dec 1994) ISSN: 0340-6199 [Print] Germany
PMID7859792 (Publication Type: Case Reports, Journal Article)
Chemical References
  • CD3 Complex
Topics
  • CD3 Complex (immunology)
  • Child
  • Fatal Outcome
  • Female
  • Granulocytes (immunology)
  • Hepatomegaly (etiology)
  • Herpesvirus 4, Human (immunology)
  • Humans
  • Lymphoproliferative Disorders (complications, immunology)
  • Splenomegaly (etiology)

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