Multiple basal cell carcinomas associated with hairy cell leukaemia

Abstract

We report the case of a caucasian woman who, between the ages of 49 and 51 years, developed multiple (> 20) basal cell carcinomas (BCC). There was no family history of BCC. No abnormalities in the human homologue of the Drosophila segment polarity gene patched (PTCH), glutathione S-transferases T1 and M1, or cytochrome P450 1A1 were detected by polymerase chain reaction (PCR)-based molecular analysis. There was, however, actinic damage of the skin in sun-exposed areas. The patient was diagnosed as having hairy cell leukaemia (HCL) at the age of 51 years, based upon leucocyte morphology as assessed by light and electron microscopy, tartrate-resistant acid leucocyte phosphatase (TRAP) staining, fluorescence activated cell scanning of peripheral blood leucocytes and bone marrow histology. As the leukaemia slowly progressed over a 3-month period, the patient developed four further BCCs. Given that HCL is characterized by a profound defect in T-cell function, it is conceivable that T-cell immune dysregulation can contribute to the pathogenesis of BCC, possibly enhancing the aetiological effect of ultraviolet irradiation.

Basal cell carcinoma

“…We speculate that age‐related changes in immune surveillance are determinants of clustering and accrual 12, 13. Effects of UVB include impairment of induction of contact hypersensitivity, promotion of hapten‐induced tolerance, and creation of a sustained immunosuppressive environment.…”

“…Most cases, particularly after first presentation, had noted lesions and believed them to be contemporaneous. Clearly, the separate BCC in a cluster did not appear at precisely the same time but rather the phenotype reflects fast accrual of lesions 11, 12. In a few cases, the appearance of each BCC could have been separated in time but been unnoticed until clinical examination.…”

Multiple basal cell carcinoma in lymphoedema

“…Immunosuppression might also playarole for the development of MBCC, as in malignancieslikechroniclymphocyticleukaemia andh airy celll eukaemia (23,28). Apart from immunosuppression relatedt on eoplasms, transplantation andf ollowing immunosuppressive therapy regimesp redisposefor the development of non-melanoma skin cancer,suchasMBCC (18).…”

Skin changes in hairy cell leukemia

“…The association between HCL and second primary malignancies remains controversial [37][38][39][40][41]. The overall incidence of second primary tumors in patients with HCL ranges from 19.9 to 24% [41][42][43][44][45]. The most common include second primary malignancies of the skin, melanoma and non-melanoma skin cancer [43,44].…”

“…The overall incidence of second primary tumors in patients with HCL ranges from 19.9 to 24% [41][42][43][44][45]. The most common include second primary malignancies of the skin, melanoma and non-melanoma skin cancer [43,44]. Existing data suggests that patients with HCL have a higher risk of nonmelanoma skin cancers [45]; therefore, these patients should be closely examined, especially when secondary risk factors exist like fair skin and extensive sun burden.…”