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Long lasting lymphadenopathy in childhood as an expression of a severe hyperimmune B lymphocyte disorder
Author(s) -
Nezelof C.,
Virelizier J. L.
Publication year - 1983
Publication title -
hematological oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 44
eISSN - 1099-1069
pISSN - 0278-0232
DOI - 10.1002/hon.2900010305
Subject(s) - lymphocyte , expression (computer science) , immunology , medicine , biology , computer science , programming language
On the basis of the 6 cases reported here and scattered published cases, the existence of a childhood chronic immunoblastic lymphadenopathy syndrome is proposed. It is characterized by the following features: A systemic immunoblastic proliferation with varying degrees of maturation resembling the B lymphocyte hyperimmune disorder observed in angioimmunoblastic lymphadenopathy but with no deposits of interstitial amorphous material or vascular proliferation. Disseminated superficial and deep lymphadenopathy. Chronic, pronounced splenomegaly. A constant thrombocytopenia. A polyclonal hypergammaglobulinemia, with markedly elevated antibody titers to various agents. An early onset and a course of several years (up to 20), interrupted in half the cases by the occurrence of a virus‐associated (EBV, Papova) neoplastic process or a fatal viral infection. Low natural killer (NK) cell activity in 2 cases.