Angiocentric lymphoproliferative disorders61

This group of disorders, which encompasses the previously nosologic entities of lymphomatoid granulomatosis, represents an uncommon type of vasculitis which combines the angiodestructive and granulomatous features of Wegener's granulomato-sis with the cellular atypicality of lymphoma.62-64 Evidence now indicates that lymphomatoid granulo-matosis is a T-cell lymphoma.65 Jaffe has also described a histological grading for angiocentric lymphoproliferative disorders.66 Grade I is characterized by a predominantly polymorphous lympho-cytic infiltrate without cytological atypia; grade II is characterized by the polymorphous infiltrate but with clear cytological atypia; grade III, also termed angiocentric lymphoma, is clearly malignant histo-logically. This type of vasculitis seems to carry a more severe prognosis than most cases of NA, with frequent involvement of the CNS. Still, this entity associates an angiocentric proliferation of T-lympho-cytes with lesions of blood vessels that induce nerve ischemia. Neuropathy associated with angiocentric proliferation of lymphocytes and necrosis of blood vessels, and infiltration predominantly composed of CD8+ lymphocytes, occur in the course of infection with HIV.67 Occasionally, vasculitis occurs in the context of a malignant monoclonal gammopathy, including multiple myeloma or malignant lymphoma with monoclonal gammopathy.

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