Analysis of protein removal properties during cryofiltration apheresis using the Evaflux-5A plasma fractionator in a patient with hepatitis C virus-associated cryoglobulinemic glomerulonephritis.

Cryofiltration (CF) is a technique in which separated plasma is chilled before being subjected to a plasma fractionator (PF), leading to cryoglobulin precipitation or cryogel formation. In CF using the Evaflux-5A as the PF, there is no consensus on the necessity of albumin supplementation, and when or how often the PF column should be washed. We analyzed the sieving effects of various solutes (albumin, IgG, IgM, LDL, HCV-RNA, and cryoglobulin) depending on transmembrane pressure (TMPPF ) during CF using the Evaflux-5A in a patient with hepatitis C virus-associated cryoglobulinemic glomerulonephritis. Five CF treatments were initially performed and a sixth one later, at disease recurrence. Quantitative detection of cryoglobulin and a marked rise in TMPPF to 400 mm Hg were observed only at the first and sixth treatment, and albumin losses during these treatments were very high, at 16.8 g, and 14.6 g, respectively, while those of others (from the second to fifth) were 6.7 g, 6.4 g, 5.9 g, and 7.0 g, respectively. The sieving coefficients (SCs) of both albumin and IgG were stable (0.8-1.0) at TMPPF < 200 mm Hg, but significantly decreased at TMPPF ≥ 200 mm Hg (P < 0.01). The SC of IgM tended to decrease at TMPPF ≥ 200 mm Hg, but not significantly, while that of LDL was zero regardless of the TMPPF . Albumin loss per treatment likely depends on degree of TMPPF rise, which is mainly affected by the patient's cryoglobulinemic status. In CF using Evaflux-5A, washing the PF column to keep TMPPF < 200 mm Hg during treatment may be a recommended for selective removal and albumin salvage.

Preconditioning regimen consisting of anti-CD20 monoclonal antibody infusions, splenectomy and DFPP-enabled non-responders to undergo ABO-incompatible kidney transplantation.

Patients undergoing ABO-incompatible kidney transplantation must have their anti-donor blood-type antibody titer (ADBT) reduced to below 1:16 by using either plasma-exchange (PEX) or double filtration plasma exchange (DFPP) before they can safely undergo a transplantation. The ADBT can be reduced to under 1:16 in most cases; however, some cases (non-responders) do not respond to PEX or DFPP treatment. To enable kidney transplantations to be performed in non-responders, we developed a new preconditioning regimen consisting of anti-CD20 monoclonal antibody (rituximab) infusions, a splenectomy, and DFPP. Four non-responders were infused with rituximab at a dose of 375 mg/m(2) weekly for 3-4 wk and splenectomized 1 or 2 wk before transplantation. Four to five DFPP-sessions were then performed after the splenectomy. Using this preconditioning regimen, the ADBT was reduced to below 1:16, enabling kidney transplantations to be successfully performed in all patients. After the kidney transplantation, no episodes of humoral rejection were observed, and only one episode of cellular rejection was encountered. The cellular rejection was associated with a reduction in immunosuppressant administration because of CMV infection that occurred 80 d after the kidney transplantation. The renal allografts were functioning well in all patients after a mean follow-up period of 390 d. No serious complications or side effects were encountered. We have developed a new preconditioning regimen that enables PEX and DFPP non-responders to undergo ABO-incompatible kidney transplantations.

Living related liver transplantation for polycystic liver disease.

Polycystic liver disease (PCLD) is a rare inherited disorder, often associated with polycystic disease of the kidney. Although liver failure is unusual, some patients suffer from hepatic enlargement associated with severe complications such as abdominal distention, cachexia and dyspnea. Until recently, many surgical attempts had been made to reduce hepatic size, however, results have been unsatisfactory [3, 9, 10]. Today, liver transplantation is recommended as a therapeutic option, and excellent outcome has been demonstrated [1, 2, 4, 5, 6, 8, 11]. In this paper, we present the first case study of total hepatectomy and partial liver transplantation for PCLD, from a living, related donor. The patient is a 38-year-old man with PCLD who underwent living related liver transplantation (LRLT). He is alive and well 21 months after the operation, with complete resolution of the symptoms. He has returned to his previous job, with a marked improvement in his quality of life. Our experience demonstrates that LRLT can be an option for treatment of PCLD.