Treatment of Systemic Lupus erythematosus-Associated Thrombocytopenia with Intravenous Gamma Globulin

M. Pilar Ruiz-Valverde, Divison of Nephrology, Hospital ‘Vail d’Hebron’, Paseo Vail d’Hebron s/n, E-08035 Barcelona (Spain) Dear Sir, Thrombocytopenia occurs frequently in systemic lupus erythematosus (SLE), and it may sometimes be resistant to the immuno-suppressant treatment [1]. We describe a patient diagnosed as having SLE with antipla-telet antibodies, who had acute thrombocytopenia associated with intravascular hemo-lysis and digestive hemorrhage during the course of an acute lupus relapse. There was no response to the treatment with methyl-prednisolone (MP) and cyclophosphamide (CPM). After the treatment with intravenous γ-globulin (IVGG) there was a recovery of the platelet count and the extrarenal affectation disappeared. A 17-year-old woman was diagnosed as having SLE with skin, articular and renal involvement at 13 (diffuse proliferative glo-merulonephritis type IV according to the WHO classification) and started treatment with prednisone and oral CPM. In August 1992 she was admitted to hospital because of a new lupus relapse and treatment with a 0.7 g/m2 monthly dosis of CPM bolus was started. In December 1992, 15 days after the fifth bolus of CPM, she had a new lupus relapse with polyarthritis, pleuropericarditis, microangiopathic hemolytic anemia, acute thrombocytopenia and rapidly progressive renal insufficiency. Analysis: Serum Crea-tinine 1,325 μmol/l, leukocyte count 2 × 109/1 (4% metamyelocytes, 2% myelocytes, 14% band forms), platelets 10 × 10V1, hemoglobin 51 g/l, hematocrit 0.14, lactic dehydrogenase 1,640 Ul/ml, abundant schistocytes, C3 28 mg/dl (normal 85-170), C3pa 16 mg/dl (normal 11-50), C4 7 mg/dl (normal 9^4-0), antinuclear antibodies ( + ) 1/2,560 (homogeneous pattern), antiDNA 89 U/l (normal < 15), positive antiplatelet antibodies, negative anti-Sm, anti-Ro, anti-La and ANCA antibodies. Although for the next 3 days a new dose of 1,000 mg of MP and 2 mg/kg/day of CPM was given, she was still thrombocy-topenic and had a digestive hemorrhage. Soon after her admission, she needed a sub-stitutive hemodialysis treatment through a femoral catheter. No plasmapheresis treatment was recommended as there was an active hemorrhage. Four days after her admission, IVGG (0.4 g/kg/day for 5 days) was given. The platelet count increased progressively from the 1st day after finishing the treatment and stabilized at 250 × lOVliters 10 days later. The signs of lupus involvement remitted, although the renal function was not recovered. Now she is treated