R.G.J. Klein Wolterink, P.T. Werner, J. Potjewijd, P. van Paassen
Woensdag 20 april 2016
15:40 - 15:50u in Auditorium 1
Parallel sessie: Parallelsessie 1: Case reports/research
Introduction: Traumatic tissue injury causes a proinflammatory immune response, normally followed by immediate downregulation to prevent from tissue damage. Here, we present a case in which trauma induced an undiscovered autoimmune disease with a severe phenotype.
Case: A 86-year old otherwise healthy woman was referred because of acute pain and blue discoloration of her fingers and feet after a traumatic shoulder dislocation three days prior to hospitalization. She was diagnosed with necrotizing vasculitis. As she tested positive for antinuclear antibodies (titer 1/320) and the peripheral blood smear showed amorphous extracellular light blue globules, we suspected cryoglobulinemia and initiated methylprednisolone, cyclophosphamide and plasmapheresis. Laboratory tests confirmed type I cryoglobulinemia and in addition showed a positive IgM rheumatoid factor, a monoclonal IgM kappa population and >20% monotypic plasma cells in her bone marrow, suggesting a lymphoplasmocytic lymphoma. After a favorable response to the immunosuppressive therapy, the patient was discharged from the hospital. Unfortunately, she returned one day later complaining of a cold right leg. We restarted plasmapheresis and added bortezomib and dexamethasone. Later, during stable remission of her vasculitis with dry necrotic lesions, an amputation of the left foot and of most fingers and toes was performed. The patient was discharged to a rehabilitation clinic and will continue chemotherapy.
Conclusion: Trauma can induce a profound inflammatory immune response that may trigger certain autoimmune diseases. It may also provoke a smoldering disease. In this case, shoulder dislocation induced a necrotizing vasculitis as a presenting symptom of lymfoplasmocytic lymphoma with cryoglobulinemia.