Cutaneous TCell Lymphoma

Immunological disorders frequently occur in patients with cutaneous T-cell lymphoma and include monoclonal gammopathy, haemolytic anaemia, cold agglutinins, acquired factor VII inhibitor, warm and cold erythrocyte antibodies and plasma cell myeloma.

Mycosis Fungoides characterised by a clonal proliferation of CD4 and CDW29 positive cells, may be associated with B-cell proliferation and the production of a variety of antibodies, including those directed against phospholipids. Clinically the condition exhibits erythroderma. One such patient who developed cutaneous necrosis associated with a low free protein S, was reported by Hill et al. [82], Protein S acts as a cofactor to protein C, a natural inhibitor of clotting and is vitamin K dependent, as is protein C itself. Spontaneous cutaneous necrosis has been seen with the use of warfarin, which reduced protein S in patients with a heterozygous deficiency of this compound.

Some patients with aPL have been shown to have a deficiency of their protein S accompanied by high levels of C4b binding protein. A previous case of cutaneous T-cell lymphoma also of the mycosis fungoides type with APS was documented by Allegue et al. [83]. One patient with Sezary syndrome (erythroderma associated with abnormally hyperconvo-luted lymphocyte T cells in peripheral blood) with positive LA and elevated IgG aCL but without clinical complications was reported by Carrascosa et al. [84],

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