Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years

Abstract

This study aimed to clarify the comprehensive clinical, laboratory, pathological and imaging features of intravascular large B-cell lymphoma (IVLBCL) using data on 42 IVLBCL patients diagnosed at our hospital over the past 20 years. The majority of patients were diagnosed via random skin biopsy (29/42, 69·0%) followed by bone marrow biopsy alone (8/42, 19·0%). Characteristic features included persistent fever (41/42, 97·6%), decreased performance status (≥2) (100%), hypoxaemia (32/40, 80·0%), impaired consciousness (19/42, 45·2%), hypoalbuminemia (42/42, 100%) and extreme elevation of lactate dehydrogenase and soluble interleukin 2 receptor levels. Brain magnetic resonance imaging showed abnormal findings in 32/37 patients (86·4%). Hyperintense lesion in the pons was a peculiar finding that was unrelated to the neurological deficits. Positron emission tomography-computed tomography revealed a high incidence of bone marrow (26/34, 76·5%), spleen (19/34, 55·9%) and adrenal gland (9/34, 26·5%) involvement. Neurolymphomatosis was noted in 6 patients during the course of the disease. About 60% of IVLBCL patients in whom in vivo diagnosis was possible survived more than 5 years with combination chemotherapy. Our observations provide additional insight into the diagnosis of IVLBCL and indicate that early disease recognition via random skin biopsy combined with imaging, enables in vivo diagnosis of the disease and improved survival for many patients.

Keywords: MRI; PET-CT; bone marrow biopsy; intravascular large B-cell lymphoma; random skin biopsy.

Figure 1

Bone marrow accumulation pattern of FDG in patients with IVLBCL. Bone marrow accumulation of fluorodeoxyglucose (FDG) was divided into three patterns; no uptake (A), focal (B) and diffuse (C), according to the positivity of uptake in central and appendicular bones

Figure 2

Bone marrow infiltration pattern in patients with IVLBCL. (A) Pure intrasinusoidal infiltration. Neoplastic cells are confined within the intrasinusoidal spaces depicted by CD31 immunostaining. Green arrows indicate lymphoma cells. (B) Intrasinusoidal infiltration with extravasation. Neoplastic cells proliferate within the intrasinusoidal space but extravasation was also seen. (C) Diffuse interstitial infiltration pattern. Neoplastic cells proliferate diffusely within the bone marrow