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In kidney transplantation, it is essential to avoid acute vascular complications, such as hemorrhage and renal vascular thrombosis, which may often lead to allograft loss. Inherited dysfibrinogenemia is a rare coagulation disorder with a wide spectrum of clinical manifestations, such as excessive bleeding and thrombosis. A 12-yr-old boy, previously diagnosed with renal hypodysplasia, was found to have reduced fibrinogen concentrations. Coagulation tests assessing surgical risk during kidney transplantation showed a discrepancy between functional and immunologic fibrinogen concentrations. Gene analysis confirmed inherited dysfibrinogenemia, with a heterozygous mutation in FGA (Aα Arg16His) in the patient and his mother. Based on the molecular and functional properties of the mutation, and a familial phenotype, in which his aunt had experienced a previous bleeding episode, the patient was considered at greater risk of bleeding than of thrombosis. The patient was administered fibrinogen concentrate before surgery, and kidney transplantation was performed with his father as the organ donor. The patient received additional prophylactic infusions of fibrinogen concentrate postoperatively, and his postoperative course was uneventful. Accurate diagnosis of dysfibrinogenemia, including gene analysis, is important for correctly managing patients with this coagulation disorder who are undergoing kidney transplantation. [ABSTRACT FROM AUTHOR]

We report here the case of a girl who developed plasma cell-rich acute rejection ( PCAR), a condition characterized by the presence of mature plasma cells infiltrating a renal allograft. The patient's creatinine level increased sharply to 4.3 mg/dL from 0.9 mg/dL at 19 months post-renal transplantation. She showed no response to methylprednisolone pulse therapy at a dose of 500 mg for three d but did show an immediate clinical and histopathological response to muromonab- CD3 ( OKT3) administration. She had two episodes of PCAR recurrence and subsequently lost her graft. She had no evidences of antibody-mediated rejection including C4d deposition in peritubular capillaries and donor-specific antibodies during the entire follow-up period. To elucidate the pathogenesis of PCAR, immunohistological examination of infiltrating cells was performed. CD3-positive cells infiltration seemed to be associated with the CD138-positive cells infiltration, and the number of CD3-positive cells was increased preceding PCAR recurrence. Additionally, a rapid decrease in the number of CD138-positive cells and CD3-positive cells following the OKT3 administration was observed. This case suggests that T-cell mediated immune mechanisms might play a role in the development of PCAR. [ABSTRACT FROM AUTHOR]