ABSTRACTS POSTERS WETENSCHAPSDAG SFG 2012 INTRODUCTION: Cryoglobulinemia is defined as one or more immunoglobulins in the serum which precipitate at temperatures below 37C and dissolve again on rewarming. Criteria for the classification of cryoglobulinemia patients are based on serologic, pathologic and clinical findings. We present a patient with hepatitis C negative mixed type cryoglobulinemia. CASE REPORT: A 58-year old Caucasian man was admitted to the Intensive Care because of an arterial occlusion and was treated with urokinase and stenting. During admission he was successfully treated with clarythromycin because of pneumonia. One month after discharge the patient returned with hypoxemia with massive hemoptysis. He was readmitted to the Intensive Care for intubation and mechanical ventilation. Physical examination showed erythematous lesions on his lower extremities, biopsy showed a leukocytoclastic vasculitis. We found an increased level of creatinine with dysmorphic erythrocytes in the urinanalysis, suggesting glomerulonephritis. Decreased levels of C3, C4 and an elevated C1q-bindingtest suggested activation of the immune system. Over time the patient developed progressive renal failure, all tests for auto-immune diseases (ANA, ENA, ANCA, anti-CCP) were negative except for a positive IgM rheumatoid factor. Also viral tests including hepatitis C RNA were negative. Because of a possible seronegative Wegener granulomatosis he started with prednisone, cyclophosphamide and plasmapheresis, with poor result. Additionally a monoclonal IgM-kappa paraprotein and cryoglobulins were present in the serum. Bone marrow biopsy (performed after initial treatment) showed 1% plasmacells and 15% B-lymphocytes with an increased kappa/lambda ratio of 47, the ratio of kappa/lambda free light chains in the serum was also increased (ratio 23). We concluded that the patient had a hepatitis C virus negative mixed cryoglobulinemia (type 2) with diffuse alveolar haemorrhage, leukocytoclastic vasculitis of the skin and glomerulonephritis. He was treated with prednisone 1mg/kg daily and rituximab 375 mg/m2 weekly for 4 weeks. Currently he is doing well and is treated with prednisone (tapering scheme) and rituximab every three months (for 2 years according to non Hodgkin lymphoma protocols). Kappa/lambda ratios, IgM, complement levels etc. are normalised and cryoglobulins can not be detected anymore. CONCLUSION: Cryoglobulinemia mixed type 2 is an immune complex-mediated systemic vasculitis. In a majority of cases it is induced by B-cell activation by the hepatitis C virus, therefore HCV-negative cryoglobulinemia is very rare. It is unknown what the causative factors are involving HCV-negative cryoglobulinemia mixed type 2, but this diagnosis should always be considered in HCV-negative patients. Diagnose in beeld: Een anefrische vrouw met ‘urine’-incontinentie. G. Prins en Dr. Y.C.Schrama, Interne Geneeskunde Afdeling nefrologie Sint Franciscus Gasthuis Rotterdam CASUS: Een 55-jarige vrouw werd opgenomen vanaf de dialyse afdeling in verband met koorts en algehele malaise. Haar voorgeschiedenis vermeldt: chronische nierinsufficiëntie stadium V; dialyse afhankelijkheid bij postrenale problematiek bij congenitaal spina bifida; recidiverende pyelonefritiden bij neurogene blaasstoornissen. Ter voorbereiding op een niertransplantatie, werd in het verleden een bilaterale nefrectomie verricht. Patiënte had bij opname een CRP van 405 mg/ liter en een temperatuur van 38.0. Initieel leek het focus een septische arthritis van haar schouder. Onder therapie hiervoor daalde het CRP gestaag, maar patiënte knapte onvoldoende op. 125 WETENSCHAPPELIJK jaarverslag 2012 Pagina 124
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