Successful treatment of monomorphic primary central nervous system post-transplantation lymphoproliferative disorder 5 years after kidney transplantation.

A 31-year-old man underwent living-related kidney transplantation in 2004 as a consequence of primary focal segmental glomerulosclerosis (FSGS). Four years after the transplantation, we confirmed nephrotic syndrome caused by recurrent FSGS. We performed plasmapheresis and low-density lipoprotein adsorption. We also combined steroid therapy with a reduction in the dose of tacrolimus and an increased dose of mycophenolate mofetil. The nephrotic syndrome improved dramatically with this combined therapeutic approach. However, 10 months after these treatments, he revisited our hospital because of altered consciousness. We detected multiple tumor masses in his brain that were ring enhanced on contrast magnetic resonance imaging. Consequently, we suspected primary central nervous system post-transplantation lymphoproliferative disorder (CNS-PTLD). We performed a craniotomy to biopsy the brain tumors. The biopsy specimen showed Epstein-Barr virus-associated diffuse large B-cell lymphoma. There is no definitive treatment for CNS-PTLD. Therefore, we treated the primary CNS-PTLD successfully with whole-brain radiation and discontinuation of immunosuppression therapy.

[Clinical evaluation of cases with massive ascites accumulation after discontinuation of CAPD: an attempt to establish the concept of the pre-sclerosing encapsulating peritonitis(pre-SEP) state].

Sclerosing encapsulating peritonitis(SEP) is a most serious complication of continuous ambulatory peritoneal dialysis(CAPD). Although the criteria of diagnosis and guidelines for therapy of SEP have been proposed by the Japanese SEP Study Group already, SEP is refractory to treatment when the disease process is complete. It is important to detect the latent phase of SEP(pre-SEP state) in order to treat patients at an early stage. We evaluated the characteristics of ascites in four patients with massive ascites accumulation after discontinuation of CAPD. Age and the duration of CAPD of the subjects were 53.3 +/- 9.7 years and 126.5 +/- 6.8 months, respectively. However, the patients were withdrawn from CAPD because of peritonitis or ultrafiltration failure. We also followed cytokines and parameters of collagen metabolism of ascites in two patients during adrenocorticosteroid therapy and conducted a histopathological evaluation of the peritoneum of an autopsy case who had died of pneumonia. Ascites seems to be exudative because of the high concentration of protein, cytokines and parameters of collagen metabolism such as interleukin-1 beta, interleukin-6, transforming growth factor-beta 1, procollagen 3 peptide, and type IV collagen 7S, the levels of which were 21.3 +/- 9.3 pg/ml, 8,153 +/- 7,327 pg/ml, 6.7 +/- 3.6 ng/ml, 89.3 +/- 67.8 U/ml, and 59.0 +/- 36.2 ng/ml, respectively. The histopathological findings of the peritoneum from the autopsy case showed dense fibrous tissue permeated with inflammatory infiltration and widespread infiltration of fibrin. These findings suggested that the peritoneum was inflamed when massive ascites accumulated. The amount of ascites and concentration of cytokines and parameters of collagen metabolism of ascites diminished during adrenocorticosteroid therapy. We concluded that massive and refractory accumulation of ascites appearing after the discontinuation of CAPD should be regarded as a sign of the pre-SEP state, and prophylactic treatment should be started at this stage of disease.

[Hyperuricemia and the kidney].

The risk for renal insufficiency by uric acid precipitation in medulla of kidney correlates with the degree of uric acid supersaturation in the urine, depending on uric acid concentration and urinary pH. The patients with gout or hyperuricemia have sometimes acidic urine and increased uric acid excretion. Accordingly, these patients frequently accompany by renal insufficiency. Improvement of hyperuricosuria, increasing of urine volume, and alkalinization of urine to pH6 6.5, are effective for the prevention from renal insufficiency. Acute renal failure related to hyperuricemia, can also occured secondary to cell lysis. Tumor lysis syndrome is a critical illness characterized by massive tumor cell death leading to severe hyperuricemia, hyperphosphatemia, hypocalcemia, and acute renal failure after starting chemotherapy to cancers, especially lymphoproliferative malignancies. Administration of allopurinol 500-600 mg and adequate hydration and alkalinization of urine are advocated to prevent acute renal failure. Intensive care with hemodialysis is often required to treat renal failure, because renal failure is reversible in most cases.