Intravascular lymphomatosis presenting in the lung

Area of Science:

• Oncology
• Pulmonary Medicine
• Pathology

Background:

• Intravascular lymphomatosis (IVL) is a rare form of non-Hodgkin lymphoma.
• IVL typically presents with diverse systemic symptoms, often complicating diagnosis.
• Pulmonary involvement in IVL can manifest as interstitial lung disease, mimicking other conditions.

Purpose of the Study:

• To describe the clinical and pathological features of four patients with IVL presenting as interstitial lung disease.
• To highlight the diagnostic challenges and differential considerations for IVL in the pulmonary context.

Main Methods:

• Case series reporting on four patients diagnosed with IVL.
• Clinical data collection including symptoms, imaging, and biopsy results.
• Histopathological examination of open lung biopsy specimens.

Main Results:

• All four patients exhibited progressive dyspnea, weight loss, and fever.
• Chest radiographs revealed diffuse interstitial infiltrates.
• Lung biopsies showed interstitial pneumonia with intravascular proliferation of large lymphoid cells, congestion, microthrombi, and intimal proliferation, consistent with intravascular large cell lymphoma (malignant angioendotheliomatosis/angiotropic lymphoma).

Conclusions:

• Intravascular lymphomatosis should be considered in the differential diagnosis of interstitial lung disease with systemic symptoms.
• Histopathological confirmation of intravascular lymphoma is crucial for accurate diagnosis and management.
• Distinguishing IVL from other pulmonary lymphoproliferative and intravascular malignancies is essential.