Lymphohistiocytic tumors

Lymphomatoid granulomatosis

Last author update: 1 September 2012
Last staff update: 12 July 2023

Copyright: 2003-2024,, Inc.

PubMed Search: lymphomatoid granulomatosis

Deepali Jain, M.D.
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Cite this page: Jain D. Lymphomatoid granulomatosis. website. Accessed May 19th, 2024.
Definition / general
  • Also called angioimmunoproliferative lesion
  • Lymphoproliferative disorder that is malignant per se, or likely to become malignant, resembles post-transplant lymphoproliferative disorders
Clinical features
  • Middle aged men and women with well defined, bilateral rounded mass densities on chest X-ray
  • Associated with transplant-related immunosuppression, Sjogren's syndrome, HIV
  • 80% of cases have extrapulmonary involvement (skin, CNS, kidneys, liver, spleen, adrenal glands, heart, GI tract); rarely, there is only extrapulmonary involvement
  • Usually no hilar or mediastinal lymph node involvement
  • May be an EBV related, B-cell proliferation; T-cells present are usually reactive; may have reversal of T-helper / suppressor ratio
  • Large number of atypical lymphoid cells is a poor prognostic factor
  • Median survival 14 months; death due to sepsis, destruction of lung tissue
  • Chemotherapy
Microscopic (histologic) description
  • Nodular inflammatory infiltrate of large atypical lymphoid cells (prominent nuclei, mitotic activity), plasma cells, immunoblasts, involving walls of pulmonary vessels (angioinvasive, angiocentric, angiodestructive)
  • No multinucleated giant cells
  • Similar infiltrates found in other organs
Positive stains
  • EBV (50 - 70%)
Differential diagnosis
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