Multicentre retrospective study of intravascular large B‐cell lymphoma treated at academic institutions within the United States

Abstract

Summary Intravascular large B‐cell lymphoma (IVLBCL) is a rare entity, with a generally aggressive course that may vary based on geographic presentation. While a United States (US) registry study showed relatively good outcomes with IVLBCL, clinicopathological and treatment data were unavailable. We performed a detailed retrospective review of cases identified at 8 US medical centres, to improve understanding of IVLBCL and inform management. We compiled data retrieved via an Institutional Review Board‐approved review of IVLBCL cases identified from 1999 to 2015 at nine academic institutions across the US. We characterized the cohort's clinical status at time of diagnosis, presenting diagnostic and clinical features of the disease, treatment modalities used and overall prognostic data. Our cohort consisted of 54 patients with varying degrees of clinical features. Adjusting for age, better performance status at presentation was associated with increased survival time for the patients diagnosed in vivo (hazard ratio: 2·12, 95% confidence interval 1·28, 3·53). Based on the data we have collected, it would appear that the time interval to diagnosis is a significant contributor to outcomes of patients with IVLBCL.

Prognostic Value of Concurrent Expression of C-MYC and BCL2 in Intravascular Large B-Cell Lymphoma: A 10-Year Retrospective Study

“…This emphasizes that skin biopsy, particularly random skin biopsy, which is a minimally invasive method, appears to be a useful diagnostic tool even in the absence of BM invasion and sometimes can preclude unnecessary biopsy of other vital organs. Therefore, we agree with previous reports that [9][10][11][12][13][14]. Sections from the lower extremities and trunk are important and may yield a better chance to detect lymphoma cells since our results suggest that cutaneous lesions in IVLBCL commonly affected these areas.…”

“…Nonetheless, random skin biopsy (RSB) has been proposed as a useful diagnostic tool in IVLBCL by Gill et al [7]. Subsequent studies also confirmed the potential of RSB in establishing the diagnosis of IVLBCL [8][9][10][11][12][13][14].…”

Current progress and future perspectives of research on intravascular large B‐cell lymphoma

“…21 Before being listed in the WHO classification, IVLBCL reports from the USA were extremely limited, but a retrospective study from academic centers in the USA has been recently reported. 27 In respectively. 27 Although skin eruption and neurological symptoms were more common in the USA than in Japan, the clinical aggressiveness of IVLBCL did not differ much (Table 1).…”

“…27 In respectively. 27 Although skin eruption and neurological symptoms were more common in the USA than in Japan, the clinical aggressiveness of IVLBCL did not differ much (Table 1).…”

Intravascular lymphoma: from vessels to genes

“…2,3 Remarkably, the blood vessel is the site of active cell replication and also serves as vehicle for disease spread. The clinical manifestations of IVLBCL are extremely heterogeneous, and the disease is frequently regarded as a great imitator, which poses diagnostic challenges, 3,4 but increasing clinical awareness of it has steadily improved recognition and diagnosis. 5 A skin biopsy is generally used to reach a diagnosis in cutaneous IVLBCL and in a fraction of patients with the classical variant of the disease.…”