angioendotheliomatosis

Etiology

• malignant phenotype most common
• benign reactive variant (reactive angioendotheliomatosis is a rare, poorly defined disorder
  • etiololgy depends on offending disease

Epidemiology

• 100 cases of malignant angioendotheliomatosis & ~40 cases reactive angioendotheliomatosis have been described worldwide

Pathology

• proliferation of histiocytes within vascular lumina
• sludging effect of circulating malignant lymphoid cells
• secondary intravascular thrombi & obliteration of the vessels

Laboratory

• complete blood count
  • anemia
  • thrombocytopenia
  • leukocyte count variable
• peripheral blood smear
• serum chemistries
  • serum LDH elevated
  • serum AST & serum ALT elevated
  • serum alkaline phosphatase elevated
• cryofibrinogen in plasma may be elevated

Management

• treat as B-cell lymphoma
• prognosis
  • mortality 80% within 1 year (malignant form)
  • reactive angioendotheliomatosis has a good prognosis -regression common when underlying disease, if discovered, is successfully treated

Notes

• classification of diffuse large B-cell lymphoma derived from Snomed (otherwise would be classified as B-cell lymphoma)

More general terms

• diffuse large B-cell lymphoma (DLBCL)