Type II cryoglobulinemic vasculitis in the setting of MALT lymphoma

The objectives of this article are to present a case of type II cryoglobulinemic vasculitis, explain why mucosa-associated lymphoid tissue (MALT) lymphoma is an unusual cause of type II cryoglobulins and to discuss the aetiology, epidemiology, pathophysiology and treatment of cryoglobulinemic vascul...

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Bibliographic Details
Published in:BMJ case reports 2021-01, Vol.14 (1), p.e236267
Main Authors: Reed, Griffin J, Hazim, Antonious Z, Sanchez-Alvarez, Catalina, Warrington, Kenneth J
Format: Article
Language:English
Subjects:
Abdomen
Acute Kidney Injury - etiology
Adrenal Cortex Hormones - therapeutic use
Aged
Antibodies
Antineoplastic Agents, Immunological - therapeutic use
Bendamustine Hydrochloride - therapeutic use
Biopsy
Case Report
Case reports
Connective tissue diseases
Creatinine
Cryoglobulinemia - etiology
Epistaxis
Female
Granulomas
Hepatitis C
Humans
Immunoglobulins
Immunotherapy
Laboratories
Lymphoma
Lymphoma, B-Cell, Marginal Zone - complications
Lymphoma, B-Cell, Marginal Zone - drug therapy
Medical imaging
Microscopy
Monoclonal antibodies
Patients
Peptides
Proteins
Purpura
Rituximab - therapeutic use
Splenomegaly - etiology
Targeted cancer therapy
Vasculitis - etiology
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Summary:The objectives of this article are to present a case of type II cryoglobulinemic vasculitis, explain why mucosa-associated lymphoid tissue (MALT) lymphoma is an unusual cause of type II cryoglobulins and to discuss the aetiology, epidemiology, pathophysiology and treatment of cryoglobulinemic vasculitis. A 67-year-old woman presented with 4 months of weight loss, intermittent epistaxis and a purpuric skin rash. Prior to presentation, she was found to have an elevated rheumatoid factor. Further investigation revealed an acute kidney injury and elevated type II cryoglobulins suspicious for cryoglobulinemic vasculitis, which was confirmed by kidney biopsy. Additional workup for the weight loss included biopsy of newly found splenomegaly. Pathology revealed MALT lymphoma, a rare cause of type II cryoglobulinemic vasculitis. Successful medical therapy required treating the underlying malignancy with rituximab and high-dose steroids. After initial resolution of symptoms with this regimen, the patient’s vasculitis worsened, which was thought to be secondary to undertreatment of the lymphoma. Bendamustine was added to further treat the lymphoma, after which the patient recovered and was able to discharge without recurrence of symptoms at 6 months.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-236267