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

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of tumor cells in the lumina of systemic organ small vessels (Figure 1). 1 The disease mainly affects middle-aged and elderly adults, similar to other types of malignant lymphoma. A peculiar characteristic of the disease is the lack of lymphadenopathy, which is a general characteristic of malignant lymphoma, and the disease develops with nonspecific symptoms such as fever, general fatigue, and respiratory symptoms, which hamper timely and precise diagnosis. The existence of the disease is suspected only after the appearance of systemic symptoms mentioned above, and the delay in diagnosis directly leads to deteriorating general conditions due to progression. Since being listed in the 4th edition of the World Health Organization (WHO) classification as a distinct disease entity, 2 awareness of the disease has improved, leading to increased timely diagnosis. In this review, we will discuss recent progress in research and treatment of the disease and future perspectives.

Recommendations for an Approach to Random Skin Biopsy in the Diagnosis of Intravascular B-Cell Lymphoma

“…3,29,31 Shimada and Kiyoi recommend work-up by both RSB and BMB if IVBCL is suspected. 38 Another diagnosis may also be responsible for the patient's presentation. Review of studies where RSB did not yield a diagnosis of IVBCL revealed numerous other neoplastic, auto-immune, and infectious etiologies of a patient's symptomatology including various other types of lymphoma, acute disseminated encephalomyelitis, Schnitzler syndrome, adult-onset Still's disease, neuro-Behcet disease, sarcoidosis, graft-versus-host disease, POEMS syndrome, systemic lupus erythematosus, polyarteritis nodosa, bacterial infection and tuberculosis.…”

Intravascular Lymphoma as a Cause of Recurrent Strokes – Case Report and Review of the Literature

“…IVL does not typically cause lymphadenopathy, unlike diffuse large B-cell lymphoma (DLBCL) with which it shares molecular and genetic signatures. 3 IVL can rarely cause strokes: 4 In an analysis of all published cases from 1957 to 2012 of 654 patients, 5 around half presented with neurological symptoms. Within this group, cognitive impairment/dementia was the most common CNS symptom.…”

“…9 Prior to the introduction of rituximab, the median overall survival of patients with IVL with anthracycline-based therapies was 3 years. 3 Japanese and European retrospective studies indicate that progression-free survival was significantly improved with rituximab. 3 PRIMEUR-IVL, a phase II trial involving 38 patients, demonstrated safety of rituximab with CHOP, high-dose methotrexate, and intrathecal chemotherapy for intravascular lymphoma without CNS involvement, with a progression-free survival of 76% over 2 years (95% CI 58-87%).…”

Intravascular Large B Cell Lymphoma with CNS Involvement Successfully Treated with High-Dose Methotrexate and High-Dose Ara-C Based CNS-Directed Chemoimmunotherapy Alternating with Anthracycline Based Chemoimmunotherapy

“…The optimal treatment for CNS-IVL is not established. 7 , 15 , 16 Most patients with IVL with or without CNS involvement are treated with anthracycline-based chemoimmunotherapy (CIT), frequently rituximab, cyclophosphamide, hydroxydoxorubicin, vincristine and prednisone (RCHOP), and without any CNS-directed therapy. 15 , 17 , 18 In the European patients with IVL with both CNS and non-CNS involvements, RCHOP has resulted in 88% complete remission rate (CRR), 91% overall response rate (ORR) and a 3-year overall survival (OS) in 81% of patients.…”

“… 15 However, the experience with CNS-directed therapy in management of IVL patients is limited and further investigation and clinical experience are required. 16 …”