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Targeted treatment of autoimmune cytopenias in primary immunodeficiencies.
Pacillo, Lucia; Giardino, Giuliana; Amodio, Donato; Giancotta, Carmela; Rivalta, Beatrice; Rotulo, Gioacchino Andrea; Manno, Emma Concetta; Cifaldi, Cristina; Palumbo, Giuseppe; Pignata, Claudio; Palma, Paolo; Rossi, Paolo; Finocchi, Andrea; Cancrini, Caterina.
Afiliación
  • Pacillo L; Academic Department of Pediatrics (DPUO), Immune and Infectious Diseases Division, Research Unit of Primary Immunodeficiencies, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Giardino G; Department of Systems Medicine, University of Tor Vergata, Rome, Italy.
  • Amodio D; Pediatric Section, Department of Translational Medical Sciences, Federico II University, Naples, Italy.
  • Giancotta C; Academic Department of Pediatrics (DPUO), Research Unit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Rivalta B; Academic Department of Pediatrics (DPUO), Research Unit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Rotulo GA; Academic Department of Pediatrics (DPUO), Immune and Infectious Diseases Division, Research Unit of Primary Immunodeficiencies, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Manno EC; Department of Systems Medicine, University of Tor Vergata, Rome, Italy.
  • Cifaldi C; Academic Department of Pediatrics (DPUO), Research Unit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Palumbo G; Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.
  • Pignata C; Academic Department of Pediatrics (DPUO), Research Unit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Palma P; Academic Department of Pediatrics (DPUO), Immune and Infectious Diseases Division, Research Unit of Primary Immunodeficiencies, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Rossi P; Department of Onco Hematology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
  • Finocchi A; Pediatric Section, Department of Translational Medical Sciences, Federico II University, Naples, Italy.
  • Cancrini C; Department of Systems Medicine, University of Tor Vergata, Rome, Italy.
Front Immunol ; 13: 911385, 2022.
Article en En | MEDLINE | ID: mdl-36052091
Primary Immunodeficiencies (PID) are a group of rare congenital disorders of the immune system. Autoimmune cytopenia (AIC) represents the most common autoimmune manifestation in PID patients. Treatment of AIC in PID patients can be really challenging, since they are often chronic, relapsing and refractory to first line therapies, thus requiring a broad variety of alternative therapeutic options. Moreover, immunosuppression should be fine balanced considering the increased susceptibility to infections in these patients. Specific therapeutic guidelines for AIC in PID patients are lacking. Treatment choice should be guided by the underlying disease. The study of the pathogenic mechanisms involved in the genesis of AIC in PID and our growing ability to define the molecular underpinnings of immune dysregulation has paved the way for the development of novel targeted treatments. Ideally, targeted therapy is directed against an overexpressed or overactive gene product or substitutes a defective protein, restoring the impaired pathway. Actually, the molecular diagnosis or a specific drug is not always available. However, defining the category of PID or the immunological phenotype can help to choose a semi-targeted therapy directed towards the suspected pathogenic mechanism. In this review we overview all the therapeutic interventions available for AIC in PID patients, according to different immunologic targets. In particular, we focus on T and/or B cells targeting therapies. To support decision making in the future, prospective studies to define treatment response and predicting/stratifying biomarkers for patients with AIC and PID are needed.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndromes de Inmunodeficiencia Tipo de estudio: Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Front Immunol Año: 2022 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Suiza
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndromes de Inmunodeficiencia Tipo de estudio: Guideline / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Front Immunol Año: 2022 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Suiza