Intravascular Large B-Cell Lymphoma Presenting as Interstitial Lung Disease

Abstract

Intravascular large B-cell lymphoma (IVLBL) is a rare subtype of diffuse large B-cell lymphoma that resides in the lumen of blood vessels. Patients typically present with nonspecific findings, particularly bizarre neurologic symptoms, fever, and skin lesions. A woman presented with shortness of breath and a chest CT scan showed diffuse interstitial thickening and ground glass opacities suggestive of an interstitial lung disease. On physical exam she was noted to have splenomegaly. The patient died and at autopsy was found to have an IVLBL in her lungs as well as nearly all her organs that were sampled. Although rare, IVLBL should be included in the differential diagnosis of interstitial lung disease and this case underscores the importance of the continuation of autopsies.

Serial chest CT findings of intravascular large B-cell lymphoma of the lungs

“…Serial chest CT revealed exacerbated extension and intensity of GGA without new consolidation, reticulation, traction bronchiectasis, or honeycombing in the whole lung area, especially the periphery of the lung, compared with the initial CT. These serial CT features might correlate with thickened alveolar septa and perivascular spaces because of distension of small vessels such as septal capillaries, venules, and arterioles filled with atypical lymphoid cells, rather than lymphatic channels (3,6). Furthermore, the absence of reticulation, traction bronchiectasis, honeycombing, or architectural distortion on CT images can rule out fibrotic interstitial lung disease (3).…”

When Malignancies Mimic Interstitial Lung Disease: A Case Series and Review of the Literature

“…The typical fibrotic changes in reaction to the proliferation of malignant cells (desmoplastic reaction) can further contribute to misdiagnosis. Lymphoma, another differential diagnosis of ILD with malignant etiology, may show lymphangitic spread with the radio-pathological changes mentioned above [10].…”

Analysis of clinicopathological features and prognostic factors of non‑Hodgkin's intravascular large B‑cell lymphoma

“…Non-Hodgkin's intravascular large B-cell lymphoma (IVL) is an extremely rare subgroup of diffuse large B-cell lymphoma, accounting for only 1% of the total cases of the disease (1). According to the latest histological classification of lymphomas, the World Health Organization (WHO) classified IVL as an extranodal lymphoma in 2001 (1)(2)(3). IVIBCL, together with primary effusion lymphoma and large B cell lymphoma of the mediastinum, was classified as a subtype of DLBCL (2,3).…”

“…The reported incidence rate of men and women worldwide is ~1:1, the median age of onset is ~60 years and the average survival time is ~6-9 months (1-5). Most patients die within 1 year, and it is a malignant tumor with poor prognosis (1)(2)(3)(4)(5). IVL is a highly invasive and extremely rare malignant hematological disease, with poor prognosis and a lack of specificity in the clinical setting (1,2,4).…”

“…Most patients die within 1 year, and it is a malignant tumor with poor prognosis (1)(2)(3)(4)(5). IVL is a highly invasive and extremely rare malignant hematological disease, with poor prognosis and a lack of specificity in the clinical setting (1,2,4). The stable immunohistochemical expression of CD34, CD20 and PAX5 is considered beneficial to the diagnosis and differential diagnosis of IVL (2,3), which may occur in various organs and tissues, though are most commonly found in the adrenal gland and skin tissue (3).…”