Introduction:
Xeroderma pigmentosum (XP) is an autosomal recessive disorder
characterised by extreme sensitivity to sunlight, resulting in severe
sunburn and pigment changes in the skin. It is caused by defects in
genes involved in the repair of ultraviolet induced photoproducts in DNA
by nucleotide excision repair (NER). It is associated with greatly
elevated incidence of skin cancer.1,2 It is also
associated with malignancies of oral cavity especially squamous cell
carcinoma and haematological malignancy especially acute myeloid
leukaemia (AML). 3
Lymphoblastic leukaemia in XP is extremely rare and there are very few
case reports of the same2-4. We report a case of a
11-year-old boy with XP, who developed B – Acute lymphoblastic
leukaemia (B-ALL).