Abstract
Background Intravascular large B-cell lymphoma (IVLBCL) is a variant of extranodal diffuse large B-cell lymphoma (DLBCL), characterized by the presence of a B-lymphoma cell in the lumina of small blood vessels or capillaries. Due to its extremely variable clinical manifestations, IVLBCL typically results in a delayed diagnosis and poor disease prognosis. Skin biopsy, particularly random skin biopsy, has shown a potential role in the diagnosis of IVLBCL. However, information of clinicopathological features in patients with IVLBCL diagnosed by skin biopsy is limited. Objectives To study the clinicopathological features in relation to immunohistochemical features and to identify prognostic factors in IVLBCL patients diagnosed by skin biopsy. Materials and Methods Clinical characteristics; laboratory, histological, and immunohistochemical findings; and therapeutic response of all biopsy-confirmed IVLBCL patients during the years 2008-2017 were retrospectively reviewed. Results The mean age was 67.4 (±9.8) years. Fever was the most common presenting symptom, accounting for 64.7%. Cutaneous and bone marrow involvement was found in 23.5% and 35.3% of patients, respectively. Patients receiving R-CHOP showed more favorable therapeutic outcome. C-MYC/BCL2 double expressors showed significantly higher incidence rate to mortality compared with nondouble expressors (p = 0.042). One-year and two-year overall survival rates were 67.2% and 53.8%, respectively. Conclusions Skin biopsy is an effective diagnostic method for IVLBCL. Concurrent expression of C-MYC and BCL2 may be a useful prognostic indicator and should be performed in order to predict the prognosis in IVLBCL patients.
Highlights
Intravascular large B-cell lymphoma (IVLBCL), first described as “angioendotheliomatosis proliferans systemisata” [1], is a rare variant of extranodal diffuse large B-cell lymphoma (DLBCL)
Given the fact that IVLBCL can present with a wide range of signs and symptoms depending on organ involvement, diagnosis and treatment are often delayed, resulting in poor disease prognosis [2]
A recent series of IVLBCL patients diagnosed by random skin biopsy showed no bone marrow (BM) involvement [11], we found approximately 35% with BM infiltration which is consistent with previous studies ranging from 26.7% to 100% [4, 6, 20, 22, 24, 26, 27]
Summary
Intravascular large B-cell lymphoma (IVLBCL), first described as “angioendotheliomatosis proliferans systemisata” [1], is a rare variant of extranodal diffuse large B-cell lymphoma (DLBCL) It is characterized by the presence of lymphoma cells in the lumina of small blood vessels and an aggressive clinical course involving multiple organs [2, 3]. Given the fact that IVLBCL can present with a wide range of signs and symptoms depending on organ involvement, diagnosis and treatment are often delayed, resulting in poor disease prognosis [2]. Information of clinicopathological features in patients with IVLBCL diagnosed by skin biopsy is limited. To study the clinicopathological features in relation to immunohistochemical features and to identify prognostic factors in IVLBCL patients diagnosed by skin biopsy. Concurrent expression of C-MYC and BCL2 may be a useful prognostic indicator and should be performed in order to predict the prognosis in IVLBCL patients
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