Your browser doesn't support javascript.
loading
Infantile systemic juvenile xanthogranuloma case with massive liver infiltration
Rodríguez-Velasco, Alicia; Rodríguez-Zepeda, María del Carmen; Ortiz-Hidalgo, Carlos.
Afiliação
  • Rodríguez-Velasco, Alicia; UMAE. Hospital de Pediatría del Centro Médico Nacional IMSS. Department of Pathology. Ciudad de México. MX
  • Rodríguez-Zepeda, María del Carmen; UMAE. Hospital de Pediatría del Centro Médico Nacional IMSS. Department of Hematology. Ciudad de México. MX
  • Ortiz-Hidalgo, Carlos; Hospital ABC Medical Center. Department of Surgical Pathology. Ciudad de México. MX
Autops. Case Rep ; 9(2): e2018081, Abr.-Jun. 2019. ilus, tab
Article em En | LILACS | ID: biblio-994658
Biblioteca responsável: BR26.7
ABSTRACT
Infantile systemic juvenile xanthogranuloma (ISJXG) is an uncommon form of juvenile xanthogranuloma, a non-Langerhans cell proliferation of infancy and early childhood. In a small percentage of patients, the visceral involvement­most commonly to the central nervous system, liver, spleen, or lungs­may be associated with severe morbidity, and eventually fatal outcome. Here we describe the clinical and pathological findings of a 28-day-old girl with ISJXG who died with respiratory distress syndrome. She had few cutaneous lesions but massive liver and spleen infiltration; other affected organs were multiple lymph nodes, thoracic parasympathetic nodule, pleura, pancreas, and kidneys. Additional findings were mild pulmonary hypoplasia and bacteremia. Immunohistochemistry on fixed tissues is the standard for diagnosis. Immunophenotype cells express CD14, CD68, CD163, Factor XIIIa, Stabilin-1, and fascin; S100 was positive in less than 20% of the cases; CD1a and langerin were negative. No consistent cytogenetic or molecular genetic defect has been identified. This case demonstrates that the autopsy is a handy tool, because hepatic infiltration, which was not considered clinically, determined a restrictive respiratory impairment. In our opinion, this was the direct cause of death.
Assuntos
Palavras-chave

Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Assunto principal: Xantogranuloma Juvenil / Hepatopatias Tipo de estudo: Prognostic_studies Limite: Female / Humans / Newborn Idioma: En Ano de publicação: 2019 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: LILACS Assunto principal: Xantogranuloma Juvenil / Hepatopatias Tipo de estudo: Prognostic_studies Limite: Female / Humans / Newborn Idioma: En Ano de publicação: 2019 Tipo de documento: Article