Intravascular Large B-cell lymphoma: A case series and review of literatures

Abstract

Background Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma with uncommon clinical presentations and poor prognosis. The purpose of this study is to report the clinical features and outcome of IVLBCL in a single institution of Taiwan. Methods Ten patients with IVLBCL diagnosed from June 2006 to January 2018 were retrospectively reviewed. Results The median age was 61 (range 39–88) years. The most common presentation was fever (90%), cytopenia (90%), and confusion (50%). For all patients, the median progression free survival (PFS) and overall survival (OS) were 12.6 (95% confidence interval [CI] 0.0–76.1) and 18.8 (95% CI 0–59.3) months, respectively. Six patients received rituximab combined chemotherapy, and the other one patient was treated with chemotherapy alone. Six of seven (85.7%) patients achieved complete response after chemotherapy. The median PFS and OS for six patients who completed treatment were not reached. Three-year PFS and OS rates were 80% and 75%, respectively. Conclusion Our study showed that patients might achieve durable remission after rituximab-based chemotherapy. The outcome of IVLBCL patients may further improve if early diagnosis and prompt treatment were made.

Intravascular lymphoma presenting with hypoxaemia, platypnoea and lactic acidosis

“…4 In a recent review of 10 cases of 'non-mainly cutaneous' IVLBCL the main biological and clinical features were fever, weight loss, night sweats, cytopenia, skin and neurological signs in patients of a median age of 67 years old without significant sex prevalence and a high International Prognosis Index. 6 These findings are consistent with most of the other case series, where elevated LDH levels and splenomegaly are common features. 5 8 9 Our case presents some interesting features.…”

“…As cytopenia of one or more cell lines is a common feature of classical IVLBCL, bone marrow is a frequent site of biopsy and is reported as an affected organ. 6 ¹⁸F-FDG-PET seems to be critical, but not perfect, for evaluation of the extent of IVLBCL. 13 However, standard radiological findings that enable targeted biopsy of liver and lung do not always provide good correlations with involvement of these organs; random biopsies (or autopsies) can sometimes be positive.…”

The challenging diagnosis of intravascular large B-cell lymphoma

“…2 The diagnosis is established by skin biopsy, which reveals large lymphoma cells within the blood vessels, and treatment includes systemic therapy and therapy directed at the central nervous system. 3,4 The overall mortality is associated with the prognosis of the lymphoproliferative disorder itself, but when the disease is restricted to the skin, the prognosis seems to be more favorable. 5 Early diagnosis and treatment could reduce mortality and improve prognosis, although it may not translate into a different outcome given the poor prognosis of this case.…”

Intravascular Large B-Cell Lymphoma

“…40 Recent studies suggest the addition of intrathecal methotrexate as both CNS treatment and prophylaxis; however, impact on survival outcomes remains in need of further investigation. [41][42][43] Autologous stem cell transplants have also been trialed with variable reports of success. 44,45 Despite treatment, disease progression is aggressive and prognosis is generally poor.…”