Association With Autoimmune Phenomena

The association between HCL and autoimmune disorders such as scleroderma, polymyositis, Raynaud's phenomenon, and various vasculitis, has been described.22 23 Among the vasculopathic syndromes polyarteritis nodosa represents the most frequently associated disorder, with 18 cases reported so far. It has been suggested that a common membrane antigen expressed in leukemia cells and vascular endothelium may lead to a vasculitis by generating cross-reacting antibody.24 Four cases of scleroderma in the setting of HCL have been reported.25-27 It has been postulated that sarcoidosis may be induced directly through hairy cell antigen-mediated activation of T cells or indirectly through promotion of the nonspecific inflamatory response.27 The presence of cutaneous lesions, such as erythematous maculopapules and pyoderma gan-grenosum, have been noted in patients with HCL.28-30

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