ABSTRACT
Three healthy boys, 3.5, 5 and 1.5 years of age, were admitted to hospital with a severe bacterial skin infection, cerebellar ataxia, and pneumonia, respectively, one week after the onset of varicella. They recovered completely after treatment. Studies in Europe report complications from varicella in 2.5% of healthy children. Most of these are neurological complications and secondary bacterial infections of skin and soft tissue. Last year, a European consensus was published that recommended that all healthy children be vaccinated against chickenpox. In The Netherlands, routine varicella zoster virus (VZV) vaccination has not (yet) been implemented. We propose a new discussion on the possible inclusion of VZV vaccination in the national vaccination programme.
Subject(s)
Cerebellar Ataxia/etiology , Chickenpox Vaccine , Chickenpox/complications , Herpesvirus 3, Human/immunology , Pleuropneumonia/etiology , Skin Diseases, Bacterial/etiology , Cerebellar Ataxia/epidemiology , Chickenpox/prevention & control , Child, Preschool , Health Policy , Humans , Immunization Programs , Infant , Male , Netherlands/epidemiology , Pleuropneumonia/epidemiology , Skin Diseases, Bacterial/epidemiologyABSTRACT
X-linked lymphoproliferative disease (XLP) is a rare immunodeficiency caused by mutations in the signaling lymphocyte activating molecule-associated protein/SH2D1A gene and characterized by a dysregulated immune response to Epstein-Barr virus and other pathogens. The clinical presentation is heterogeneous and includes fulminant infectious mononucleosis, lymphoma, hypogammaglobulinemia and aplastic anemia. XLP is associated with a high morbidity and overall outcome is poor. At present, allogeneic stem cell transplantation (alloSCT) is the only curative treatment. XLP patients may be recognized in various stages of disease and even when symptoms are not yet evident. We here present two related XLP patients in different stages of disease that were both treated successfully with alloSCT using a matched unrelated donor. In addition, we have reviewed all reported cases of alloSCTs in XLP patients. Based on these results and in order to improve the final outcome, we conclude that alloSCT should be recommended in both symptomatic and asymptomatic XLP patients.
