An Unusual Presentation of Cryoglobulinemia in a Patient With Undiagnosed Sjögren’s Syndrome and Treated Marginal Zone Lymphoma

Our case details a 47-year-old female who presented to our cancer hospital with a petechial rash of the lower extremities as well as a headache and blurred vision for the prior two days. She was found to have systolic pressures in the 200s in the emergency department and was admitted for a hypertensive emergency. Notable medical history includes marginal zone lymphoma (MZL) status post-submandibular resection at an outside institution in 11/2017 (thought to be in remission). With her history in mind, she also reported subjective submandibular swelling on admission and an unintentional 25-pound weight loss over the eight weeks prior to admission. A PET scan was completed, which showed diffusely increased reticuloendothelial activity, and a follow-up bone marrow biopsy was without residual lymphoma activity. Creatinine was markedly elevated with significant proteinuria, and a renal biopsy revealed thrombotic microangiopathy, acute tubular injury, and moderate interstitial fibrosis. Remarkable laboratory tests included positive quantitative cryoglobulins (“cryocrit”) and low complement 4 (C4). Qualitative cryoglobulins were never obtained, unfortunately. She was started on prednisone and transferred to a nearby academic hospital for formal rheumatologic evaluation. Importantly, testing at this facility showed elevated Sjögren’s syndrome-related antigen A (SSA/Ro) antibodies. Also elicited at the academic hospital was that she had been experiencing symptoms of chronic dry eyes and mouth years even before her diagnosis of MZL. She was diagnosed with primary Sjögren’s syndrome, which was thought to be the cause of her likely mixed cryoglobulinemia and the precipitant of her acute renal failure with hypertensive emergency, her skin changes, her anemia, and her hypocomplementemia. Of note, prior to discharge from the academic hospital, the patient’s cryoglobulin testing was negative after prolonged steroid treatment, and she was placed on rituximab for maintenance. Our case is important as it helps illustrate one of the myriad precipitants of mixed cryoglobulinemia, in this case possibly untreated Sjögren’s syndrome.