Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature.
A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. Hepatitis B viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.
de Terlizzi
M
M
Department of 2nd Pediatrics, University of Bari, Italy.
Toma
M G
MG
Santostasi
T
T
Colella
R
R
Ceci
A
A
De Benedictis
G
G
D001796:Blood Protein Disorders / Q000150:complications*; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D007167:Immunotherapy; D007223:Infant; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology