Lists > Genodermatoses > Disorders with Increased Malignancy

Disorders with Increased Malignancy

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•Wiskott-Aldrich syndrome (few that survive to adulthood = highly prone to develop hematologic malignancy)
•ataxia-telangiectasia (30-40% develop malignancy - hematologic mostly; defective repair of radiation induced damage - ionizing radiation, not UV)
•Chediak-Higashi syndrome lymphoma-like phase in late childhood

 

chromosome instability –

•Bloom syndrome (~1/4 develop malignancy by age 20 -  hematologic and GI)

 

Genodermatoses treated by bone marrow transplant:

•Wiskott-Aldrich syndrome (BMT = cure)
•dyskeratosis congenita (BMT = only long-term treatment option;  but cure???)