Type II cryoglobulinemic vasculitis in the setting of MALT lymphoma

BMJ Case Rep. 2021 Jan 12;14(1):e236267. doi: 10.1136/bcr-2020-236267.

Abstract

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.

Keywords: oncology; rheumatology; vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Acute Kidney Injury / etiology
  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Antineoplastic Agents, Immunological / therapeutic use
  • Bendamustine Hydrochloride / therapeutic use
  • Cryoglobulinemia / etiology*
  • Female
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / complications*
  • Lymphoma, B-Cell, Marginal Zone / drug therapy
  • Rituximab / therapeutic use
  • Splenomegaly / etiology
  • Vasculitis / etiology*

Substances

  • Adrenal Cortex Hormones
  • Antineoplastic Agents, Immunological
  • Rituximab
  • Bendamustine Hydrochloride