Intravascular Large B-Cell Lymphoma

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Abstract

Intravascular large B-cell lymphoma (IVBCL) is a very rare and aggressive subtype of extranodal diffuse large B-cell lymphoma (DLBCL) involving the growth of lymphoma cells within blood vessels of all organ types. We present the case of a 55-year-old North-African man with no prior history of neoplastic disease presenting with weight loss and an isolated splenomegaly. Investigations led to the diagnosis of this disease. To the best of our knowledge, this is the first case recorded in Africa. Through this article, we discuss this case and outline the common presentation, paraclinical investigations, and treatment options of IVBCL.

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The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

Rita Alaggio, Catalina Amador, Ioannis Anagnostopoulos … (2022)

We herein present an overview of the upcoming 5th edition of the World Health Organization Classification of Haematolymphoid Tumours focussing on lymphoid neoplasms. Myeloid and histiocytic neoplasms will be presented in a separate accompanying article. Besides listing the entities of the classification, we highlight and explain changes from the revised 4th edition. These include reorganization of entities by a hierarchical system as is adopted throughout the 5th edition of the WHO classification of tumours of all organ systems, modification of nomenclature for some entities, revision of diagnostic criteria or subtypes, deletion of certain entities, and introduction of new entities, as well as inclusion of tumour-like lesions, mesenchymal lesions specific to lymph node and spleen, and germline predisposition syndromes associated with the lymphoid neoplasms.

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Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'.

Andrés Ferreri, Elias Campo, John F Seymour … (2004)

Despite its recognition as a distinct, extremely rare entity, no large studies of intravascular lymphoma (IVL) have been reported. The clinico-pathological characteristics of 38 human immunodeficiency virus-negative patients with IVL diagnosed in Western countries were reviewed to better delineate clinical presentation, clinical variants, natural history and optimal therapy. The IVL is an aggressive and usually disseminated disease (Ann Arbor stage IV in 68% of cases) that predominantly affects elderly patients (median age 70 years, range: 34-90; male:female ratio 0.9), resulting in poor Eastern Cooperative Oncology Group Performance Status (ECOG-PS >1 in 61%), B symptoms (55%), anaemia (63%) and high serum lactate dehydrogenase level (86%). The brain and skin are the most common sites of disease. In contrast to previous reports, hepatosplenic involvement (26%) and bone marrow infiltration (32%) were found to be common features in IVL, while nodal disease was confirmed as rare (11% of cases). Patients with disease limited to the skin ('cutaneous variant'; 26% of cases) were invariably females with a normal platelet count, and exhibited a significantly better outcome than the remaining patients, which deserves further investigation. Overall survival was usually poor; however, the early use of intensive therapies could improve outcome in young patients with unfavourable features. ECOG-PS >1, 'cutaneous variant', stage I and chemotherapy use were independently associated with improved survival.

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Intravascular large B-cell lymphoma: a chameleon with multiple faces and many masks

Maurilio Ponzoni, Elias Campo, Shigeo Nakamura (2018)

Intravascular large B-cell lymphoma (IVLBCL) is a rare, clinically aggressive lymphoma entity characterized by an almost exclusive growth of large cells within the lumen of all sized blood vessels. The reasons for this peculiar localization of neoplastic cells are only partially understood. Clinically, in its classical variant, IVLBCL presents with many nonspecific signs and symptoms such as fever of unknown origin and involvement of the central nervous system and skin. Cases, which show disease limited to the skin, following extensive staging workup, are called cutaneous variants and show a better prognosis. In addition, a hemophagocytic variant associated with hemophagocytic syndrome and often with hepatosplenic involvement and cytopenia has been described. The classical and hemophagocytic variants are present mainly in western or Asian countries, respectively, although exceptions have been increasingly reported in both geographical areas. The cutaneous variant is mostly observed in western countries. Staging of IVLBCL is difficult and still not satisfactory. The often poor prognosis of this type of lymphoma has been substantially improved by immunochemotherapy, in particular with rituximab. Despite improved outcome, a significant proportion of patients relapse, in particular those with central nervous system manifestations. This review focuses on histopathological features, pathogenetic elements, presenting symptoms, clinical variants, disease progression, prognostic factors, therapeutic management, and the outcome of IVLBCL.

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Mehdi Loukhnati:

ORCID: https://orcid.org/0000-0003-0338-2756

Journal

Journal ID (nlm-ta): Case Rep Hematol

Journal ID (iso-abbrev): Case Rep Hematol

Journal ID (publisher-id): CRIHEM

Title: Case Reports in Hematology

Publisher: Hindawi

ISSN (Print): 2090-6560

ISSN (Electronic): 2090-6579

Publication date Collection: 2023

Publication date (Electronic): 15 September 2023

Volume: 2023

Electronic Location Identifier: 5596890

Affiliations

Department of Hematology and Bone Marrow Transplantation Mohammed VI University Hospital, Cadi Ayyad University, Marrakesh 40080, Morocco

Author notes

Academic Editor: Pier Paolo Piccaluga

Author information

Mehdi Loukhnati https://orcid.org/0000-0003-0338-2756

Fatima Ezzahra Lahlimi https://orcid.org/0000-0001-8911-0334

Illias Tazi https://orcid.org/0000-0002-5091-632X

Article

DOI: 10.1155/2023/5596890

PMC ID: 10516690

PubMed ID: 37746518

SO-VID: d986da92-c5f3-4c10-9fef-b2723f1aee62

License:

This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 2 March 2023

Date revision received : 7 June 2023

Date accepted : 4 September 2023

Intravascular Large B-Cell Lymphoma

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Most cited references 10

The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms

Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'.

Intravascular large B-cell lymphoma: a chameleon with multiple faces and many masks

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