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progressive nephromegaly, and refractory temporal lobe epilepsy (detailed clinical history can be found in this article’s supplemental data). Blood analysis showed progressive pancytopenia, progressive elevation of liver enzymes, and chronic kidney failure with a glomerular filtration rate as low as 50 ml/min/1.73 m2, as measured by 51Cr-EDTA clearance. On ultrasound multiple hypodense, focal lesions were evident in the liver, spleen, and kidneys, which were enlarged. Histological examination of a liver biopsy specimen demonstrated severe chronic hepatitis with initial fibrosis. Kidney histology showed pronounced diffuse interstitial oligoclonal cytotoxic T cell infiltrates with changing TCR Vβ repertoires on sequential biopsies (Fig. S1 in this article’s supplemental data). Colon biopsy showed chronic colitis. The patient developed severe growth delay. At the age of 13 years, he underwent splenectomy, which …