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Case report shows MCL presenting as cryoglobulinemic vasculitis

Key clinical point: Patient’s skin biopsy showed occlusive vasculopathy, suggesting cryoglobulinemic vasculitis. Bone marrow biopsy confirmed mantle cell lymphoma.

Major finding: Systemic immunochemotherapy including bortezomib and rituximab resolved the cryoglobulinemia and the MCL remains in complete remission at 2 years.

Study details: Single patient case report of a 60 year old man.

Disclosures: The authors reported that there was no funding source and that they had no conflicts.

Source: Pei S-N et al. J Cancer Res Practice. 2020 7(1):41-4.

Commentary

“Asymptomatic type I cryoglobulinemia is a relatively common finding in patients with lymphomas, likely occurring in 10-15% of small B-cell lymphomas that exhibit plasmacytic differentiation and secrete a monoclonal IgM. Symptomatic type II mixed cryoglobulinemias are less common but should be considered in lymphomas arising in the setting of Sjogren’s syndrome or hepatitis C. Rarely, mantle cell lymphoma (MCL), particularly SOX11-negative MCL, exhibits plasmacytic differentiation and secretes a monoclonal IgM. Undoubtedly, some of these monoclonal IgMs have type I cryoglobulinemic properties and are fortunately asymptomatic. Rarer still are the MCL-associated mixed-type cryoglobulins. Indeed, Pei et al describe the only reported case. These cases serve as a reminder that some MCLs are derived from an antigen-experienced B cell that continue to differentiate and may present differently that the majority of cases. We still have a lot to learn about the variable pathobiology of MCL.”

Peter Martin, MD

Weill Cornell Medicine

Citation:

Pei S-N et al. J Cancer Res Practice. 2020 7(1):41-4.