Skip to main content
Log in

A case of nasal T cell lymphoma with lethal midline granuloma which is clinically indistinguishable from Wegener’s granulomatosis

  • Case Report
  • Published:
Japanese Journal of Rheumatology

Abstract

We describe a patient who developed fever, bilateral otitis media, destructive necrosis of the nasal cavity and multiple lung nodules. The patient fulfilled the American College of Rheumatology (ACR) classification criteria for Wegener’s granulomatosis (WG) and was also diagnosed as having WG by using the ACR classification tree. However, the diagnosis of T cell lymphoma was finally made by cervical lymph node biopsy, 2 years after disease onset. Rheumatologists should therefore aware of the pitfall of using diagnostic criteria and repetitive biopsy is strongly recommended for accurate diagnosis of WG.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Burston H: Lethal midline granuloma: is it a pathologic entity?Laryngoscope 69: 1–43, 1959.

    Article  CAS  PubMed  Google Scholar 

  2. Tsokos M, Fauci AS, Costa J: Idiopathic midline destructive disease (IMDD): a subgroup of patients with the ‘midline granuloma’ syndrome.Am J Clin Pathol 77: 162–168, 1982.

    CAS  PubMed  Google Scholar 

  3. Robinson AC, Fraser I, Bailey Det al.: Idiopathic midline destructive disease—case report and review of the literature.Postgrad Med J 60: 471–473, 1984.

    Article  CAS  PubMed  Google Scholar 

  4. Hirota J, Osaki T, Yoneda Ket al.: Midline malignant B-cell lymphoma with leukemic transformation.Cancer 70: 2958–2962, 1992.

    Article  CAS  PubMed  Google Scholar 

  5. Leavitt RY, Fauci AS, Bloch DAet al.: The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis.Arthritis Rheum 33: 1101–1107, 1990.

    CAS  PubMed  Google Scholar 

  6. Harabuchi Y, Yamanaka N, Kataura Aet al.: Epstein-Barr virus in nasal T-cell lymphomas in patients with lethal midline granuloma.Lancet 335: 128–130, 1990.

    Article  CAS  PubMed  Google Scholar 

  7. Kanavaros P, Lescs MC, Briere Jet al.: Nasal T-cell lymphoma: a clinicopathologic entity associated with peculiar phenotype and with Epstein-Barr virus.Blood 81: 2688–2695, 1993.

    CAS  PubMed  Google Scholar 

  8. Borisch B, Hennig I, Laeng RHet al.: Association of the subtype 2 of the Epstein-Barr virus with T-cell non-Hodgkin’s lymphoma of the midline granuloma type.Blood 82: 858–864, 1993.

    CAS  PubMed  Google Scholar 

  9. Mishima K, Horiuchi K, Kojya Set al.: Epstein-Barr virus in patients with polymorphic reticulosis (lethal midline granuloma) from China and Japan.Cancer 73: 3041–3046, 1994.

    Article  CAS  PubMed  Google Scholar 

  10. Aozasa K, Ohsawa M, Tomita Yet al.: Polymorphic reticulosis is a neoplasm of large granular lymphocytes with CD3+ phenotype.Cancer 75: 894–901, 1995.

    Article  CAS  PubMed  Google Scholar 

  11. Prokocimer M, Modan M, Rusoshansky Set al.: ProMACE-CytaBOM: combination chemotherapy for diffuse large cell lymphoma.Anticancer Res 9: 1233–1235, 1989.

    CAS  PubMed  Google Scholar 

  12. Pavlovsky S, Corrado C, Santarelli MTet al.: An update of two randomized trials in previously untreated multiple myeloma comparing melphalan and prednisone versus three- and five-drug combinations: an Argentine Group for Treatment of Acute Leukemia Study.J Clin Oncol 6: 769–775, 1988.

    CAS  PubMed  Google Scholar 

  13. Fauci AS, Haynes BF, Katz Pet al.: Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years.Ann Intern Med 98: 76–85, 1983.

    CAS  PubMed  Google Scholar 

  14. Wiik A: Antineutrophil cytoplasmic antibodies in Wegener’s granulomatosis.Clin Exp Rheumatol 11: 191–201, 1993.

    CAS  PubMed  Google Scholar 

  15. Hausen HZ, Schulte HH, Klein Get al.: EBV DNA in biopsies of Burkitt tumours and anaplastic carcinomas of the nasopharynx.Nature 228: 1056–1058, 1970.

    Article  PubMed  Google Scholar 

  16. Cabane J, Raphael M, Lamas Get al.: Origin of malignant centrofacial granulomas: surface markers and gene rearrangement of malignant cells.Laryngoscope 101: 998–1001, 1991.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

About this article

Cite this article

Nanki, T., Koike, R., Mizushima, N. et al. A case of nasal T cell lymphoma with lethal midline granuloma which is clinically indistinguishable from Wegener’s granulomatosis. Japanese Journal of Rheumatology 7, 183–188 (1997). https://doi.org/10.1007/BF03041240

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03041240

Key words

Navigation