<p>Prognosis of Intravascular Large B Cell Lymphoma (IVLBCL): Analysis of 182 Patients from Global Case Series</p>

Abstract

Purpose This study provides an overview of the prognosis of intravascular large B cell lymphoma (IVLBCL) over the past 10 years and analyzes the possible relevant factors. Patients and Methods We conducted a literature search of case reports, case series, and retrospective studies of IVLBCL published from January 2008 to July 2018. After excluding inappropriate data, 103 publications were selected for the analysis. Statistical analyses of different treatment modalities, the effect of blood–brain barrier (BBB)-penetrating drugs, and prognostic factors for outcomes were performed. Results In total, 182 pathologically confirmed cases of IVLBCL were included in our study. The results revealed that the 1- and 3-year overall survival rates were 42.3 and 11.5%, respectively, whereas the median overall survival was 340 days. Overall survival (450 days vs 180 days) and progression-free survival (420 days vs 150 days) were significantly longer in patients who received rituximab-containing regimens than in those treated with other regimens. For IVLBCL involving the CNS, regimens containing BBB-penetrating drugs failed to provide an additional survival benefit. In addition, lactic dehydrogenase levels ≥700 U/L, CNS involvement, and hemophagocytic syndrome were identified as unfavorable risk factors in patients with IVLBCL, whereas skin involvement appeared to be a protective factor. Conclusion Rituximab-containing chemotherapy can improve the outcomes of patients with IVLBCL, but the prognosis remains unsatisfactory. Treatment regimens containing BBB-penetrating drugs failed to improve outcomes in patients with CNS-involved IVLBCL. Several factors affect the prognosis of patients with IVLBCL, and further research on the underlying mechanisms is needed.

Hemophagocytic syndrome and neurological involvement in a case of intravascular large B-cell lymphoma

“…A global case series reported that the median patient age of IVLBCL was 64 ± 12.7 years, and that 51.1% of patients were men. 5 The patient described in the present case was 32 years old, which is much younger than the average age of illness. Two recent case series within China reported an average age of onset of 56 6 and 58.5 7 years, suggesting a lower age of onset in China compared with other countries.…”

“…IVLBCL generally has a poor prognosis, with 1- and 3-year overall survival rates of 42.3% and 11.5%, respectively. 5 It has naturally highly aggressive and infiltrative tumors and a poor response to chemotherapy. Furthermore, delays in diagnosis are common because of a lack of understanding of the disease.…”

Rapid deterioration of intravascular large B-cell lymphoma with mass formation in the trigeminal nerve and multiple organ infiltration: An autopsy case report

“…2,3 Among the many clinical presentations of IVLBCL, B symptoms are a common manifestation, occurring in 54-76% of patients. [4][5][6] Although the prognosis of IVLBCL is poor due to the frequent delay in early diagnosis, random skin biopsy (RSB) and BM biopsy (BMB) have improved the accuracy of diagnosis, resulting in the prompt initiation of chemotherapy and improved outcomes. 1,3,5,6 However, the results, including flow cytometry and pathological findings of these samples, are often unhelpful in making a diagnosis.We present the case of a patient with IVLBCL resulting in mass formation in the trigeminal nerve (TN).…”

“…[4][5][6] Although the prognosis of IVLBCL is poor due to the frequent delay in early diagnosis, random skin biopsy (RSB) and BM biopsy (BMB) have improved the accuracy of diagnosis, resulting in the prompt initiation of chemotherapy and improved outcomes. 1,3,5,6 However, the results, including flow cytometry and pathological findings of these samples, are often unhelpful in making a diagnosis.We present the case of a patient with IVLBCL resulting in mass formation in the trigeminal nerve (TN). This case had concurrent abnormal clinical manifestations, such as failure to detect IVLBCL by RSB and BMB, resulting in death due to rapid deterioration.…”

“…[4][5][6] Although the prognosis of IVLBCL is poor due to the frequent delay in early diagnosis, random skin biopsy (RSB) and BM biopsy (BMB) have improved the accuracy of diagnosis, resulting in the prompt initiation of chemotherapy and improved outcomes. 1,3,5,6 However, the results, including flow cytometry and pathological findings of these samples, are often unhelpful in making a diagnosis.…”