Clinical features

Patients are mostly elderly adults although cases in younger individuals, including small children, have been reported [8,18-21,25]. There is a predominance of males. Clinically, they present with solitary (rarely), localized or, more commonly, generalized plaques and tumours with a characteristic 'bruise-like' violaceous aspect (Figs 16.1 & 16.2). Ulceration is uncommon. Mucosal regions may be involved (Fig. 16.3). The morphology of cutaneous lesions is similar to that of the skin manifestations of myelogenous leukaemia. In a distinct proportion of patients (approximately 30-40%), skin lesions are accompanied by general symptoms and extracutaneous manifestations in the blood, bone marrow and/or other organs. Lymph nodes are involved in approximately half of cases at presentation. Thrombocytopenia, anaemia and neutropenia are commonly found [2].

Fig. 16.1 Blastic NK-cell lymphoma. Small solitary tumour on the flank. Note deep red colour.

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Fig. 16.2 Blastic NK-cell lymphoma. Multiple plaques and tumours on the chest. Note 'bruise-like' violaceous aspect. (Reprinted with permission from The American Journal of Surgical Pathology, in press.)

Fig. 16.3 Blastic NK-cell lymphoma. Involvement of the oral mucosa.

Skin lesions are the first manifestation of the disease in over 90% of patients. In patients with primary cutaneous disease, the time interval between the onset of skin lesions and leukaemic spread is variable, usually between a few weeks and several months. In one exceptional case there was a 15-year history of multiple cutaneous nodules [26].

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