Gorham's disease and diffuse lymphangiomatosis in children and adolescents

Rajkumar Venkatramani, Nina S. Ma, Pisit Pitukcheewanont, Marcio Malogolowkin, Leo Mascarenhas

Research output: Contribution to journalArticle

29 Scopus citations

Abstract

Gorham's disease is a rare disorder of unknown etiology and variable clinical presentation that is characterized by proliferation of thin-walled vascular channels resulting in destruction and resorption of osseous matrix. The condition is frequently under recognized or misdiagnosed. There is no standard treatment defined for this disease. Here we report on eight children diagnosed with Gorham's disease at our institution over a ten-year period. Soft tissue lymphangioma was present in seven and six children had splenic involvement. Disease stabilization and improvement was observed on treatment with interferon alpha-2b and bisphosphonate therapy.

Original languageEnglish (US)
Pages (from-to)667-670
Number of pages4
JournalPediatric Blood and Cancer
Volume56
Issue number4
DOIs
StatePublished - Apr 1 2011
Externally publishedYes

Keywords

  • Bisphosphonates
  • Chylothorax
  • Interferon
  • Lymphangioma
  • Lymphatic malformation
  • Osteolysis
  • Vanishing bone disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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