Renal Transplantation in a Patient with Idiopathic Thrombocytopenic Purpura
A 35-year-old man was hospitalized for generalized weakness and exertional dyspnea. He had developed ITP at 15 years of age and chronic glomerulonephritis at 27 years of age. However, he had been well without receiving a definite treatment.\nUpon admission, his vital signs were stable except for an increased blood pressure (150/100 mmHg), and a physical examination was unremarkable. His laboratory data were as follows: hematocrit, 24.4% (normal range, 42% to 52%); hemoglobin, 8.1 g/dL (normal range, 13 to 18 g/dL); white blood count, 6,100/µL (normal range, 4,000 to 10,500/µL); platelet count, 12,000/µL; serum blood urea nitrogen, 86.9 mg/dL (31.0 mmol/L) (normal range, 9 to 20 mg/dL [3.2 to 7.1 mmol/L]); and serum creatinine, 7.71 mg/dL (681.6 µmol/L) (normal range, 0.8 to 1.5 mg/dL [71 to 133 µmol/L]). His 24-hour urine protein excretion was 7.6 g. Blood chemistry showed a serum sodium level of 142 mmol/L (normal range, 135 to 145 mmol/L); potassium, 4.6 mmol/L (normal range, 3.5 to 5.5 mmol/L); albumin, 3.4 g/dL (normal range, 3.8 to 5.0 g/dL); calcium, 7.9 mg/dL (normal range, 8.3 to 10 mg/dL); and phosphate 4.6 mg/dL (1.49 mmol/L) (normal range, 2.5 to 5.0 mg/dL [0.80 to 1.60 mmol/L]). Liver function tests were all within normal ranges. All serologies were negative or normal, including antinuclear antibodies, anti-neutrophil cytoplasmic antibodies, hepatitis B surface antigen and hepatitis C antibody. Serum immunoglobulin levels were normal and C3 and C4 complement levels were normal.\nUrinalysis revealed a specific gravity of 1.010, pH 7.5, 3+ protein, and macrohematuria. A chest X-ray was negative and a renal ultrasound revealed normal-sized kidneys. Bone marrow biopsy findings were compatible with ITP.\nThe patient was initially treated with steroids (1 mg/kg/day) for two weeks, but there was no increase in his platelet counts. Meanwhile, the blood urea nitrogen level increased gradually (to 55.3 mmol/L), and his uremic symptoms (nausea, poor oral intake and generalized weakness) became aggravated. Hemodialysis was initiated, and renal transplantation and a splenectomy were scheduled to be performed simultaneously as the optimal management of end stage renal disease and ITP.\nThe donor was the patient's 28-year-old sister, and HLA typing confirmed a haploidentical donor-recipient match. Before renal transplantation, a prophylactic pneumococcal vaccination was performed and intravenous immunoglobulin (IVIG, 1 g/kg) was given one day before transplantation. Cyclosporine, prednisolone, and mycophenolate mofetil (MMF) were used as initial immunosuppressive agents. At the time of renal transplantation, his platelet count was 89,000/µL.\nThe patient's initial graft function was good. Intraabdominal bleeding had developed just after transplantation, but bleeding was not observed thereafter (). His platelet count gradually increased on the tenth post-transplant day and the patient was discharged on the 37th post-transplant day with a normal serum creatinine level (114 µmol/L) and a platelet count of 124,000/µL (). The native kidney biopsy findings were consistent with IgA nephropathy ().\nTwo months later, the patient was readmitted because of fever, chill and headache. Cryptococcus neoformans was recovered from the blood, cerebrospinal fluid and pleural fluid. Systemic cryptococcosis was well-controlled with the reduction of immunusuppressants and an intravenous amphotericin B treatment.\nA two-year follow-up of the patient showed a favorable clinical course. His platelet count was stable and the graft function was well maintained. No further overwhelming sepsis was observed.