Streptococcus pneumoniae-associated hemolytic uremic syndrome (pHUS) is an atypical form of HUS associated with microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Although less common than diarrhea-associated HUS, incidence appears to be increasing. We report a case of a child with pHUS who underwent a course of therapeutic plasma exchange (TPE) and had complete recovery. This report adds to the existing literature supporting TPE in cases of pHUS. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Red blood cell exchange is an accepted superior therapy to simple chronic transfusion, due to minimal risk of iron overload, for secondary prevention of cerebrovascular accidents in selected patients with sickle cell anemia. Recently, we described our experience of Isovolemic Hemodilution–Red Blood Cell Exchange (IHD-RBCx), a two-step modification of the conventional RBCx with several advantages, including cost reduction. We are describing our standard operating procedure for IHD-RBCx with COBE Spectra apheresis system to make it widely available to the apheresis centers interested in implementing this procedure. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Background: Factors affecting progenitor cell mobilization in patients with non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma (HL) are incompletely understood. The aim of this retrospective study was to determine which factors are crucial for effective mobilization and collection of autologous peripheral blood stem cells (PBSC) prior to transplantation in Chinese patients. Patients and methods: A total of 239 patients with lymphoma (198 NHL and 41 HL patients) underwent PBSC collection after mobilization with granulocyte-colony-stimulating factor (G-CSF) or G-CSF plus chemotherapy priming. Results: Patient characteristics at diagnosis and transplant, including low Eastern Cooperative Oncology Group score (P = 0.013), lack of extranodal invasion (P = 0.034), previously administered radiotherapy regimens (P = 0.040), treatment with platinum prior to mobilization (P = 0.042), previous chemotherapy regimens (P = 0.001) and cycles (P < 0.001), and chemotherapy regimens (P < 0.001) were statistically significant for successful mobilization in multivariate analysis. Premobilization factors, including previous radiotherapy (P = 0.009), previous chemotherapy regimens (P = 0.043) and cycles (P = 0.039), low platelet count prior to mobilization (P = 0.042), and lower CD34+ cells in peripheral blood (PB) (P = 0.050) or bone marrow (BM) (P = 0.007) were considered possibly predictive of poor mobilization. We found the patients who had chemosensitive lymphoma had worse progress-free survival (PFS) than the patients with initial treatment and high risks (P = 0.017). Conclusion: Our analysis showed that high amounts of chemotherapy, radiotherapy, low platelet count, chemosensitive recurrent patients, combination chemotherapy plus G-CSF and low CD34+ cells in BM prior to mobilization could emerged as important predictive factors for mobilization failure in Chinese patients with NHL and HL. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Plerixafor enhances CD34⁺ cell mobilization, however, its optimal use is unknown. We hypothesized that plerixafor could “rescue” patients in the midst of mobilization when factors indicated a poor CD34⁺ yield. Of 295 consecutive autologous peripheral blood mobilization attempts at our center, 39 (13%) used plerixafor as rescue strategy due to a CD34⁺ cell concentration <10/μl (median 5.95/μl, n = 30), low CD34⁺ cell yield from prior apheresis day (median 1.06 × 10⁶ CD34⁺ cells/kg, n = 7), or other (n = 2). Patients received a median of one plerixafor dose (range: 1–4). Thirty-four (87%) collected =2 × 10 ⁶ CD34⁺ cells/kg and 26 (67%) collected =4 × 10 ⁶ CD34⁺ cells/kg. Median collections for lymphoma (n = 24) and myeloma (n = 15) patients were 4.1 × 10⁶ and 8.3 × 10⁶ CD34/kg, respectively. A single dose of plerixafor was associated with an increase in the mean peripheral blood CD34⁺ concentration of 17.2 cells/μl (P < 0.001) and mean increased CD34⁺ cell yield following a single apheresis of 5.11 × 10⁶/kg (P < 0.03). A real-time rescue use of plerixafor is feasible and may allow targeted use of this agent. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

The storage of platelets in additive solution (PAS) had lagged behind red cell concentrates, especially in North America. The partial or complete removal of anticoagulated plasma and storage of platelet concentrates in AS presents many advantages. The PAS can be formulated to optimize aerobic metabolism or decrease platelet activation, thus abrogating the platelet storage lesion and potentially improving in vivo viability. Plasma removal has been shown to reduce allergic reactions and the plasma harvested could contribute to the available plasma pool for transfusion or fractionation. PAS coupled to pathogen reduction technology results in a platelet product of equivalent hemostatic efficacy to conventionally stored platelets. Given the above, the likely future direction of platelet storage will be in new generation designer PAS with an extended shelf life and a superior safety profile to plasma stored platelets. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Background: ABO-incompatible (ABOi) kidney transplantation is an established procedure relying on the removal of donor-specific isoagglutinine antibodies as part of the recipient preconditioning. At present, current protocols using immunoadsorption apply a single-use selective carbohydrate isoagglutinine adsorber. A regenerative and selective immunoglobulin immunoadsorption could be an alternative but has not been reported for ABOi transplantation. Methods: Eight patients were treated with the commonly used isoagglutinine carbohydrate epitope adsorber and seven with a regenerative polyclonal sheep anti-immunoglobulin adsorber as part of the preconditioning for ABOi kidney transplantation. An IgG-isoagglutinine titer of less or equal 1:4 qualified for transplantation. Treatment safety, efficiency, length of desensitization, number of postoperative immunoadsorptions, and allograft outcome were retrospectively compared. Results: With the use of the immunoglobulin adsorber the median initial isoagglutinine IgG titers of 1:64 (range 1:32–1:256) were lowered to the target of 1:4 preoperatively with a mean of 6.2 immunoadsorptions (range 5–11). Mean IgG/IgM titer step reduction per IA was 1.98/1.21 for (range 0–4/0–4) and mean titer step rebound 1.31/0.82 (range 0–4/0–3), respectively. The number of immunoadsorptions and length of desensitization was not different from the use of the specific isoagglutinine adsorbers. After transplantation, no rejection occurred and only one postoperative immunoadsorption was necessary. No adverse events in relation to immunoadsorption were observed. Graft function was comparable to the isoagglutinine adsorber group. Conclusion: These data suggest that ABOi kidney transplantation can be performed safely and effectively with a selective regenerative immunoglobulin immunoadsorber. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Refsum's disease is a rare autosomal recessive disorder of fatty acid metabolism. Poorly metabolized phytanic acid accumulates in fatty tissues, including myelin sheaths and internal organs, leading to retinitis pigmentosa, peripheral polyneuropathy, cerebellar ataxia, and renal, cardiac or liver impairment. Dietary restriction of phytanic acid in some cases is not sufficient to prevent acute attacks and stabilize the progressive course. Phytanic acid bound to large low density lipoproteins (LDL) and very low density lipoproteins (VLDL) molecules offers the possibility of extracorporeal elimination by lipid apheresis. We report on the long-term lipid apheresis treatment of four patients with severe Refsum's disease. Retinitis pigmentosa, peripheral polyneuropathy, cerebellar ataxia, anosmia, and sensorineural hearing loss were major symptoms exhibiting a progressive course. Lipid apheresis was performed for 5–13 years without severe complications. Maximum levels of phytanic acid before commencing chronic lipid apheresis were >300 mg/l. During steady state with lipid apheresis, mean phytanic acid before treatments was 87 mg/l and was reduced to 36 mg/l. Mean reduction rate was 59% per treatment. In all patients, abnormal motor nerve conduction velocity with signs of chronic denervation improved, morphological and functional stabilization of eye involvement was observed. Lipid apheresis prevented the extension of the disease to previously unaffected organs in three patients. Extracorporeal elimination of lipoprotein-phytanic acid complexes by lipid apheresis represents a pathophysiologically guided therapeutic approach, resulting in long-term improvement or stabilization of overall rehabilitation in patients with progressive Refsum's disease. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Leukocyte apheresis primarily used for treatment of inflammatory diseases such as inflammatory bowel disease (IBD). Beside an effect of the apheresis column, the plastic lines in the apheresis system might also have an effect due to interaction between the plastic surfaces and circulating leukocytes and plasma proteins. We recently reported generation of LL-37 in the plastic lines during leukocyte adsorbing apheresis. This generation might have a positive impact on the immunologic tolerance and therefore be one operational mechanism by which the apheresis treatment executes its effect. In the present study, we report a significant generation of sIL-1RI in the apheresis lines that is initially absorbed by the LCAP device. This finding, together with our previous data on IL-1Ra indicate that important members of the IL-1 family are significantly altered during the LCAP treatment of patients with IBD. Since IL-1 and its antagonists are important for regulation of inflammatory processes in IBD, we speculate that the LCAP related changes in sIL-1RI and IL-1Ra might impact the clinical outcome. These findings have to be taken into consideration when designing new apheresis techniques as well as sham-controlled studies. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.

Our goal was to measure the quality of care provided in the Pediatric Intensive Care Unit (PICU) during Therapeutic Apheresis (TA). We described the care as a step by step process. We designed a flow chart to carefully document each step of the process. We then defined each step with a unique clinical indictor (CI) that represented the exact task we felt provided quality care. These CIs were studied and modified for 1 year. We measured our performance in this process by the number of times we accomplished the CI vs. the total number of CIs that were to be performed. The degree of compliance, with these clinical indicators, was analyzed and used as a metric for quality by calculating how close the process is running exactly as planned or “in control.” The Apheresis Process was in control (compliance) for 47% of the indicators, as measured in the aggregate for the first observational year. We then applied the theory of Total Quality Management (TQM) through our Design, Measure, Analyze, Improve, and Control (DMAIC) model. We were able to improve the process and bring it into control by increasing the compliance to > 99.74%, in the aggregate, for the third and fourth quarter of the second year. We have implemented TQM to increase compliance, thus control, of a highly complex and multidisciplinary Pediatric Intensive Care therapy. We have shown a reproducible and scalable measure of quality for a complex clinical process in the PICU, without additional capital expenditure. J. Clin. Apheresis, 2011. © 2011 Wiley Periodicals, Inc.