Diagnosis

Diagnosis

Intravascular Large B-Cell Lymphoma.

Microscopy

Postmortem histologic examination revealed an intravascular neoplastic lymphocytic infiltrate, composed of large cells with irregular nuclear contours, vesicular chromatin, and prominent nucleoli, involving multiple organs, including the spinal cord, lungs, heart, kidneys, bladder, testes, thyroid, pancreas, and the gastrointestinal tract. The neoplastic cells were positive for CD20, BCL6, and MUM1 expression, with a high Ki67 proliferation index, while negative for CD10 expression. These findings supported the diagnosis of intravascular large B cell lymphoma (IVLBCL).

Discussion

IVLBCL is an exceedingly rare extra-nodal large B cell lymphoma characterized by a selective growth within the lumina of small blood vessels, particularly capillaries. Three variants: Classic /Western, cutaneous, and hemophagocytic syndrome-associated variants of IVLBCL are currently recognized. Patients usually present in their 7th and 8th decades; however, the disease can occur over a wide age range without significant male or female predominance. Due to the heterogeneity in presentation and lack of specific signs and symptoms, the majority of IVLBCL cases are diagnosed at autopsy. The prognosis of IVLBCL is generally poor but has improved significantly over the years with the addition of rituximab1. Some studies reported that IVLBCL patients exhibiting central nervous system involvement, similar to the presented case, tend to have shorter overall survival; A better prognosis has been observed in isolated cutaneous involvement2, 3.

In summary, IVLBCL is a multifaceted disease with a generally dismal outcome. This case highlights the importance of considering IVLBCL in the setting of non-specific clinic presentation, particularly progressive neurologic decline, as a rapid intervention may improve the chances of survival.

References

[1] Ferreri AJ, Dognini GP, Govi S, Crocchiolo R, Bouzani M, Bollinger CR, D'Incan M, Delaporte E, Hamadani M, Jardin F, Martusewicz-Boros M, Montanari M, Szomor A, Zucca E, Cavalli F, Ponzoni M: Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? J Clin Oncol 2008, 26:5134-6; author reply 6-7.

[2] Ferreri AJ, Campo E, Seymour JF, Willemze R, Ilariucci F, Ambrosetti A, Zucca E, Rossi G, Lopez-Guillermo A, Pavlovsky MA, Geerts ML, Candoni A, Lestani M, Asioli S, Milani M, Piris MA, Pileri S, Facchetti F, Cavalli F, Ponzoni M, International Extranodal Lymphoma Study G: Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'. Br J Haematol 2004, 127:173-83.

[3] Ponzoni M, Campo E, Nakamura S: Intravascular large B-cell lymphoma: a chameleon with multiple faces and many masks. Blood 2018, 132:1561-7.

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