Intravascular Large B-Cell Lymphoma: Clinicopathological Characteristics and Outcomes

Autores de IIS La Fe

• Carmen Rodriguez Garcia

  Autor

• David Martínez Campuzano

  Autor

• Ana Belén Gálvez Ruiz

  Autor

• Eloy Sempere Moreno

  Autor

• Sara Montesinos González

  Autor

• Juana Clavel Pia

  Autor

• Rafael Andreu Lapiedra

  Autor

• Nuria Rausell Fontestad

  Autor

Grupos

• Hematología y Hemoterapia

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma with a poor prognosis. This retrospective study reports the clinicopathological characteristics and outcomes of six cases of IVLBCL diagnosed in a period of 8 years in a tertiary hospital. The mean age was 66 years, with no sex predilection. Dizziness, gait instability, and hepatosplenomegaly were the most common presentations. Laboratory and brain imaging findings were inconclusive for lymphoma in all cases. Brain biopsies were diagnostic in two cases, while skin biopsies led to diagnosis in three patients. Only one patient with hemophagocytic variant was diagnosed at autopsy. Histologically, atypical lymphocytes infiltrated deep small- and medium-sized vessels, showing positive staining for B-cell markers. All patients who received combined chemotherapy achieved a complete response. After a median follow-up of 4 years, four of six patients remain alive. This lymphoma represents a diagnostic challenge, requiring a high index of suspicion and a multidisciplinary approach. © 2026 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

© 2026 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Keywords

• autopsy; intravascular large B-cell lymphoma; multidisciplinary approach; skin biopsy