The Warren Alpert Medical School of Brown University Providence, Rhode Island, United States
Background/Case Studies: Cryoglobulinemia is an immune-mediated disorder caused by the presence of abnormal immunoglobulins called cryoglobulins that precipitate at lower than core body temperatures. Cryoglobulinemia has been associated with chronic infections, lymphoproliferative disorders, and autoimmune diseases although it can be idiopathic in 10-15% of the cases. The mainstay of treatment involves treating the underlying disease which is not always possible as well as immunosuppression and plasmapheresis when there is an organ or life-threatening disease.
Study
Design/Methods: A 23-year-old female presented to the emergency department (ED) after her routine blood work revealed anemia (6.8 g/dL). She also complained of diffuse weakness and lightheadedness. Past medical history was significant for diabetes mellitus type 2, previous parvovirus infection, intravenous drug use, back pain for 1 year, and mild, painless hematuria of 6 months duration that had progressively been worsening.
Results/Findings: Her bone marrow biopsy and aspirate were unremarkable. Autoimmune workup was significant for decreased complement C3 (56 mg/dL) and C4 (< 3 mg/dL) levels. A renal biopsy was performed due to hematuria and proteinuria which was suggestive of cryoglobulinemic glomerulonephritis. Her serum protein electrophoresis and immunofixation electrophoresis revealed IgM lambda paraproteins. Infectious studies were negative. She was put on steroids and Rituximab for cryoglobulinemia and then was discharged. A few months later, she presented again with left upper quadrant abdominal and bilateral flank pain. Computed tomography showed hepatosplenomegaly for which splenectomy was performed to resolve the pain. Splenectomy was curative for her abdominal pain, paraproteinemia and cryoglobulinemia. Surgical pathology report of the spleen revealed focal involvement by a lambda-restricted IgM positive plasma cell population (Figure A). Conclusions: A very subtle and easy to miss plasma cell neoplasm of the spleen associated with cryoglobulinemia has been identified for which splenectomy appears curative.
