Letterer-Siwe syndrome

Epidemiology

• children & young adults
• predominance of males

Pathology

Multiple sites, multiple organ systems

Clinical manifestations

• systemic illness resembling an infection or malignancy
• hepatosplenomegaly
• petechiae
• purpura
• generalized skin eruption

Management

• prognosis:
  • commonly fulminant & fatal
  • spontaneous remissions uncommon
  • worst prognosis is in the very young, with multifocal involvement & organ dysfunction
• pharmaceutical agents
  • vinblastine
  • combination chemotherapy

More general terms

• malignant histiocytosis
• Langerhans cell histiocytosis (LCH); histiocytosis X
• genetic syndrome (multisystem disorder)

Database

• OMIM: https://mirror.omim.org/entry/246400