Low-grade lymphoma of immature T-cell phenotype in a case of lymphocytic interstitial pneumonia and Sjögren's syndrome

Histopathology. 1987 Nov;11(11):1193-204. doi: 10.1111/j.1365-2559.1987.tb01859.x.


A 19-year-old male patient presented with lymphocytic interstitial pneumonia and Sjögren's syndrome, confirmed by histopathology. He was treated with prednisone; 4 months later, cyclophosphamide was added. A lymph node taken at presentation revealed no histological signs of malignancy. Lymph nodes obtained 1 and 2 years later exhibited an effaced structure and a diffuse infiltration of small-sized lymphocytic cells compatible with a low-grade non-Hodgkin's lymphoma. The immunological phenotype of the lymphoma resembled that of immature T-cells present in the normal thymus cortex--positivity for CD1, CD2, CD4, CD7, CD38 and terminal deoxynucleotidyl transferase; faint positivity for CD5 and in the second specimen for CD3; negativity for CD6 and MHC class 1 antigen. The occurrence of such a peculiar lymphoma in Sjögren's syndrome has not been reported thus far. Small numbers of putative malignant cells were found on immunohistochemistry in a lymph node and a lung biopsy obtained at presentation. This is suggestive of one underlying pathogenetic event in the development of lymphocytic interstitial pneumonia, Sjögren's syndrome and non-Hodgkin's lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Humans
  • Immunoglobulins / analysis
  • Lung / pathology
  • Lymph Nodes / immunology
  • Lymph Nodes / pathology
  • Lymph Nodes / ultrastructure
  • Lymphoma / complications
  • Lymphoma / diagnosis
  • Lymphoma / pathology*
  • Male
  • Microscopy, Electron
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / pathology
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / pathology
  • T-Lymphocytes / cytology
  • T-Lymphocytes / immunology*
  • T-Lymphocytes / ultrastructure


  • Immunoglobulins