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Gorham's disease and diffuse lymphangiomatosis in children and adolescents

Rajkumar Venkatramani1, Nina S Ma, Pisit Pitukcheewanont

  • 1Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, California 90027, USA.

Pediatric Blood & Cancer
|February 8, 2011
PubMed

Insights

Gorham's disease, a rare vascular disorder, can affect children. Treatment with interferon alpha-2b and bisphosphonate therapy showed disease stabilization and improvement in eight pediatric patients.

Area of Science:

  • Vascular Biology
  • Pediatric Oncology
  • Rare Diseases

Background:

  • Gorham's disease is a rare, idiopathic disorder characterized by vascular proliferation and bone resorption.
  • It is often underdiagnosed or misdiagnosed due to variable clinical presentations.
  • No established treatment guidelines exist for Gorham's disease.

Purpose of the Study:

  • To report on the clinical characteristics and treatment outcomes of pediatric patients with Gorham's disease.
  • To evaluate the efficacy of interferon alpha-2b and bisphosphonate therapy in managing this condition.
  • To highlight the association of Gorham's disease with soft tissue lymphangioma and splenic involvement in children.

Main Methods:

  • Retrospective review of eight pediatric patients diagnosed with Gorham's disease over a ten-year period.
  • Analysis of clinical data, including presence of lymphangioma and splenic involvement.
  • Assessment of treatment response to interferon alpha-2b and bisphosphonate therapy.

Main Results:

  • Seven out of eight children presented with soft tissue lymphangioma.
  • Six out of eight children had splenic involvement.
  • Disease stabilization and improvement were observed in patients treated with interferon alpha-2b and bisphosphonate therapy.

Conclusions:

  • Interferon alpha-2b and bisphosphonate therapy may be effective in managing Gorham's disease in children.
  • Early recognition and multidisciplinary management are crucial for improving outcomes.
  • Further research is warranted to establish standardized treatment protocols for Gorham's disease.

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