Treatment of Primary Pulmonary Lymphoma in Lymphomatoid Granulomatosis with Diffuse and/or Extrathoracic Involvement

This protocol addresses patients with lymphomatoid granulomatosis presenting with diffuse pulmonary involvement and/or spread to extrathoracic sites, including potential cerebral involvement — a distribution that defines specific management considerations.

Management is typically steroid-based, with systemic options and, notably for diffuse or extrathoracic forms, radiotherapy among the approaches considered. The complete regimen selection and sequencing are detailed in the structured protocol.

References

DOI: 10.1183/09031936.02.00404102

It is usually based on steroid therapy, alone or combined with cyclophosphamide (CPM), and combination chemotherapy.

Radiotherapy has also been used to treat more diffuse and/or extrathoracic forms (especially cerebral involvement).

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