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A 55 year old Iraqi female who is aknown case of xeroderma pigmentosa presented with severe pancytopenia. Bone marrow study revealed acute myeloid leukemia with monocytic maturation.
Xeroderma pigmentosum is a rare inherited disease characterized by extreme hypersensitivity to ultraviolet rays and predisposing to cutaneous malignancies that can appear in childhood. These manifestations are often associated with ocular lesions and sometimes with neurological disorders. The association of xeroderma pigmentosum with internal neoplasms such as acute myeloblastic leukemia is not reported with great frequency, which confirms the rarity of this occurrence.