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 immunoproliferative 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 immunoproliferative 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.
Kueck
B D
BD
Department of Pathology, Medical College of Wisconsin, Milwaukee 53226.
Hanson
C A
CA
Weissman
D E
DE
Bayliss
K
K
D000328:Adult; D001773:Blood Cells / Q000473:pathology; D006402:Hematologic Diseases / Q000150:complications* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology*; D008223:Lymphoma / Q000150:complications / Q000473:pathology*; D008297:Male; D009336:Necrosis; D010587:Phagocytosis; D013577:Syndrome