A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure

الملخص EN

Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma.

It usually presents with CNS and skin involvement.

A 65-year-old Caucasian female presented with fevers and chills of 3-4 months’ duration.

Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates.

The patient later died due to multiorgan failure.

A bone marrow biopsy showed 20–30% CD5+ B cells consistent with infiltrative large B-cell lymphoma.

An autopsy performed revealed diffuse intravascular invasion by lymphoma cells.

Multiorgan involvement by intravascular B-cell lymphoma is very rare.

Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure.

The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis.

Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified.

A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission.