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A Prospective Study on Children with Initial Diagnosis of Transient Hypogammaglobulinemia of Infancy: Results from the Italian Primary Immunodeficiency Network

2008; SAGE Publishing; Volume: 21; Issue: 2 Linguagem: Inglês

10.1177/039463200802100211

2058-7384

Viviana Moschese, Simona Graziani, Maria Antonietta Avanzini, Rita Carsetti, Massimo Marconi, Marianna La Rocca, L Chini, Claudio Pignata, Annarosa Soresina, Rita Consolini, Grazia Bossi, Antonino Trizzino, Silvana Martino, Fabio Cardinale, Patrizia Bertolini, Gian Luigi Marseglia, Marco Zecca, Silvia Di Cesare, Isabella Quinti, Roberto Rondelli, Maria Cristina Pietrogrande, Paolo Rossi, Alessandro Plebani,

Blood disorders and treatments

Transient hypogammaglobulinemia of infancy (THI) is a heterogeneous disorder characterized by reduced serum IgG levels in early infancy. A putative diagnosis is initially made after exclusion of other causes of hypogammaglobulinemia while a definitive diagnosis of THI can only be made a posteriori in patients with normalization of IgG levels. The aim of this study is to characterize clinical and immunological features of children with an initial diagnosis of THI in correlation to natural outcome, and to assess predictive laboratory parameters of clinical evolution for this disorder. We prospectively analysed clinical and immunological characteristics of 77 THI children at initial diagnosis and of 57 patients at follow-up. Memory B cell subsets and in vitro immunoglobulin production were evaluated. Seventy patients (91 percent) showed clinical symptoms. Patients suffered from infections (91 percent), allergies (47 percent) and autoimmune disease (4 percent). During follow-up 41/57 children (72 percent) normalized IgG values, mostly within 24 months of age (p less than 0.001), allowing the diagnosis of THI. The 16 children who did not normalize their IgG levels showed a higher frequency of severe infections and autoimmune disease (p less than 0.01). Moreover, they expressed a reduced frequency of IgM and switched memory B cells (p less than 0.01) and an inability to produce IgG in vitro (p less than 0.02). We conclude that most patients with an initial diagnosis of THI spontaneously recover within 24 months of age and have a benign clinical course, while a subgroup of children with undefined hypogammaglobulinemia share a clinical and immunological profile with other primary immunodeficiencies. Early recognition of children with hypogammaglobulinemia during infancy who are likely to suffer from permanent immunodeficiencies later in life would allow prompt and appropriate laboratory and clinical interventions.