A Case of Pulmonary Intravascular Lymphomatosis Diagnosed by Thoracoscopic Lung Biopsy

Abstract

Intravascular lymphomatosis with primary pulmonary lesion is an extremely rare disease. Although the major clinical symptoms include fever, cough, dyspnea and loss of body weight, these are not diagnostic. Chest radiograph findings are also nonspecific and include bilateral reticular shadow, reticulonodular shadow, ground-glass opacity or wedge-shaped subpleural opacities. Therefore, the antemortem diagnosis is relatively difficult. It is considered that intravascular lymphomatosis is a high-grade malignant lymphoma. However, it has been shown recently that a good response and long-term survival may possibly be obtained through systemic combination chemotherapy. We report a case of intravascular lymphomatosis with primary pulmonary lesion where an early diagnosis was obtained through thoracoscopic lung biopsy and subsequent systemic chemotherapy proved to be quite effective. Because the clinical symptoms or chest radiograph findings are usually nonspecific, it was thought that thoracoscopic lung biopsy could be a useful procedure for early and reliable diagnosis of primary pulmonary intravascular lymphomatosis and that it might contribute to an improved prognosis.

Pulmonary Intravascular Lymphomatosis: Clinical, CT, and PET Findings, Correlation of CT and Pathologic Results, and Survival Outcome

“…When the lungs are involved, patients experience shortness of breath, hypoxemia, and, rarely, pulmonary hypertension, and the disease may simulate various lung diseases, particularly diffuse interstitial lung disease or a subacute or chronic infectious condition (6,7). Moreover, due to the rarity of this condition, ready diagnosis of the disease is difficult, and lung lesions in patients with IVL are often detected at autopsy (8).…”

“…To date, there have been several reports (8)(9)(10) about the radiologic findings of lung IVLs. Although the main computed tomographic (CT) finding of lung IVLs is diffuse ground-glass opacity (GGO), the disease also may demonstrate subpleural patchy areas of consolidation and reticulonodular lesions.…”

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

“…The severity of the disease requires early diagnosis. In this setting, a case of IVL was diagnosed early by thoracoscopic lung biopsy, allowing effective treatment by chemotherapy [15]. It was recently proposed that cytologic examination of pulmonary capillary blood aspiration by right-sided heart catheterization could demonstrate the presence of malignant cells in cases presenting as pulmonary embolism and/or primitive pulmonary arterial hypertension [16,17].…”

Intravascular large B‐cell lymphoma with FDG accumulation in the lung lacking CT/67gallium scintigraphy abnormality

“…thoracoscopic lung biopsy [15] and open lung biopsy [16] has also been reported as promising methods for the diagnosis of IVLBCL with lung involvement. Although the lung is not a common diagnostic site of IVLBCL, autopsies have revealed changes in the lung in approximately 60% of reported cases [5,17].…”

“…Even if respiratory symptoms exist, physicians often hesitate to perform TBLB in the absence of convincing radiological findings in the lung field. Indeed, reported cases diagnosed as IVLBCL by the lung biopsy have exhibited apparent lung CT abnormalities [12][13][14][15][16]. We performed TBLB after diffuse uptake of FDG in both lungs was confirmed by FDG-PET, and we were able to obtain a sample sufficient for the diagnosis.…”