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

Am J Hematol. 1989 Feb;30(2):104-11. doi: 10.1002/ajh.2830300210.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blood Cells / pathology
  • Hematologic Diseases / complications*
  • Hematologic Diseases / pathology
  • Humans
  • Liver / pathology
  • Lymph Nodes / pathology*
  • Lymphoma / complications
  • Lymphoma / pathology*
  • Male
  • Necrosis
  • Phagocytosis*
  • Syndrome