Malignant pulmonary lymphoproliferative angiitis. A monoclonal neoplasm

Area of Science:

• Pulmonary Pathology
• Hematopathology
• Oncology

Background:

• Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder.
• Malignant transformation of LYG into lymphoma is uncommon.
• Primary pulmonary lymphoma can present with infiltrative lung disease.

Observation:

• A 55-year-old female presented with rapidly progressive, bilateral pulmonary infiltrates.
• Open lung biopsy and autopsy revealed diffuse malignant lymphoproliferative disease.
• The condition exhibited a striking angiocentric and angioinvasive pattern.

Findings:

• Microscopic involvement of hilar lymph nodes, bone marrow, spleen, and viscera was noted.
• The findings suggested lymphomatous transformation of LYG.
• Plasmacytoid cells were identified via light and electron microscopy.
• Immunohistochemistry confirmed a monoclonal lymphoproliferative malignancy.

Implications:

• This case highlights a rare presentation of lymphomatous transformation of LYG.
• The angioinvasive pattern is a key diagnostic feature.
• Understanding this entity is crucial for accurate diagnosis and patient management.