Short Course of Eculizumab May Be Effective in Dialysis-Dependent Transplantation-Associated Thrombotic Microangiopathy After Hematopoietic Stem Cell Transplantation: A Case Report.

BACKGROUND: Allogeneic hematopoietic stem cell transplantation (alloHSCT) could induce several complications. The most frequent viral infections and graft-vs-host disease (GvHD) sometimes lead to thrombotic microangiopathy (TMA). It is associated with significant morbidity and mortality with the risk of death reaching 90%. Effective prevention and treatment are not available to date. Recent attempts at using antibody against C5 have been made. CASE REPORT: A 19-year-old girl with acute myeloid leukemia twice underwent alloHSCTs from her 10/10 HLA-matched sister. After the second HSCT severe acute steroid-resistant grade 4 GvHD occurred. Despite treatment with high doses of steroids, mycophenolate mofetil, biological therapy, and extracorporeal photopheresis, the patient developed TMA with acute kidney injury and the need for renal replacement therapy. The concentration of complement component 3 and activity of ADAMTS 13 were normal, and infection with Escherichia coli (E. coli) 0157H7 was excluded. Due to failure of all ordered therapies and severity of the condition, an attempt was taken to use eculizumab. Two 900-mg doses of eculizumab (Soliris) were administered at an interval of 2 weeks, which resulted in the improvement of renal function and amelioration of hemolysis and thrombocytopenia. Dialysis therapy was finished after 5 weeks, and then a third dose of the drug was administered. Eighteen months later, the patient is alive and well, with limited chronic GvHD. eGFR remains stable at 40 to 46 mL/min/1.73 m2, and mild hypertension requires treatment with angiotensin converting enzyme inhibitors and furosemide. CONCLUSION: Even a short course of eculizumab can be sufficient in controlling the TMA after HSCT, provided that the TMA-triggering factors are well controlled.

Kaposi's sarcoma in renal transplant recipients.

Kaposi's sarcoma (KS) is a spindle-shaped vascular cell tumor that occurs in the skin, lymphoid, respiratory and gastrointestinal tissues. It may resemble aggressive malignant neoplasm in HIV-related or in post-transplant types but classic form may behave as benign, potentially controllable and reversible hyperplasia. KS lesions from the onset are dispersed and multicentric. KS probability increases in solid organ transplant recipients (approximately 3/1000 patients). KS occurrence is associated with: type and dose of immunosuppression, chronic stimulation by foreign allograft antigens, viral infections (Herpes virus 8), anti rejection and induction therapy, etc. 90% of KS cases appear as dark blue or purplish macular lesions that may form nodular tumors. Histological picture shows networks of spindle shaped cells and vascular spaces surrounded by an endothelial cell layer. There is no uniform schema of KS treatment in renal transplant recipients. Immunosuppression must be reduced to the lowest levels which preserve allograft function. CsA should be converted to mofetil mycophenolate or mTOR-inhibitors. After conversion to MMF regression of KS was observed, although low therapeutic MMF doses seem to be appropriate. Sirolimus seems to inhibit the growth of established vascularized tumors and this effect is best realized with relatively low immunosuppressive doses of drug.

[Serum apolipoproteins A1, B and Apo A1/Apo B index in children with advanced chronic renal failure and on dialysis]. / Apolipoproteiny A1 i B i wskaznik ApoA1/ApoB u dzieci z zaawansowana przewlekla niewydolnoscia nerek i leczonych nerkozastepczo.

UNLABELLED: There is an increased risk of atherogenesis in adult patients with chronic renal failure (CRF). The aim of study was estimation of the risk of atherogenesis in children with advanced CRF and in children receiving renal replacement therapy. To evaluate the risk ApoA1 and ApoB concentrations were measured and ApoA1/ApoB index was calculated. RESULTS: Increased ApoA1 concentration was found in CADO group without any marked change in other groups. The relative capacity of protein part (ApoA1) of HDL seems to increase in both HD and CAPD children. But in case of ApoB (LDL) the same increase was observed only in HD children. The available data suggest that increased protein capacities of LDL and HDL are connected with lower diameter and modified properties (atherogenic potential) of those molecules. Normal ratio of ApoB/LDL in CAPD group is counterbalanced with increased serum concentration of LDL. A positive correlation between ApoA1/ApoB index value and total protein concentration that has been found in HD group suggests a role of several factors connected with dialysis regimen that affect nutrition status of HD patients. ApoA1/ApoB shows only medium level correlation with other classic indices based on total cholesterol, HDL and LDL concentrations. CONCLUSION: The change of HDL and LDL concentration in children receiving renal replacement therapy failed to significantly affect ApoA1/ApoB index. This fact excluded the possibility to apply ApoA1/ApoB as a tool that could point out the better type of treatment. Taking together the available data describing the relation between protein capacity and size of lipoprotein molecules and our calculations, we suppose that CAPD is connected with slightly lower risk of atherogenesis than HD.