Lymphomatoid Granulomatosis Treatment & Management

Updated: Dec 31, 2015
  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Treatment

Medical Care

The therapeutic approach and optimal management have not been well defined. In several studies, therapy has ranged from observation to treatment with prednisone or chemotherapy. In the largest reported study of 152 patients, no significant difference in mortality or disease-free survival was found in treatment options, and the mortality rate exceeded 50%. New therapeutic approaches are necessary. In view of the association of lymphomatoid granulomatosis (LYG) with EBV and the similarity to posttransplant lymphoma, the use of antiviral drugs with minimal immunosuppressive therapy is advocated.

  • Patients with a benign course require no treatment. Spontaneous remission has been reported.

  • Corticosteroids, with or without chemotherapy, may be recommended.

    • Treat symptomatic or progressive disease.

    • In general, therapy involves prednisone with antineoplastic agents (eg, cyclophosphamide).

    • More than 50% of patients with lymphomatoid granulomatosis respond to treatment.

    • Recurrence is usual and may include refractory disease or progression to high-grade lymphoma (13-47%).

    • When lymphomatoid granulomatosis progresses to high-grade lymphoma, combination antilymphoma regimens are used, but response rates are poor at this stage.

  • Localized disease may respond to radiotherapy.

  • Surgical resection of isolated pulmonary masses followed by chemotherapy has been associated with disease-free survival for at least 2 years.

  • Other treatment options include ganciclovir, interferon alfa-2, or, depending on histologic grade, combination chemotherapy. [7, 8]