Primary lymph node presentation of angiocentric lymphoma associated with features of a hemophagocytic syndrome

The spectrum of post‐thymic T‐cell neoplasia includes the angiocentric immunoproliterative lesions, a group of disorders histologically exhibiting vascular infiltration and destruction; included among these disorders is angiocentric lymphoma. In contrast to the typical extranodal presentation seen in the angiocentric immunoproiiferative lesions, this report describes a case of angiocentric lymphoma presenting as primary lymph node disease with clinicopathologic findings mimicking a hemophagocytic syndrome. Rearrangement of the T‐cell receptor beta chain documents this case to be a clonal T‐cell neoplasm. The association of this distinct histologic type of T‐cell malignancy with hemophagocytic syndromes is reviewed.