Autoimmune cytopenias (AIC) following allogeneic hematopoietic stem cell transplantation (HSCT) may cause significant morbidity and mortality and are often challenging to treat. We present a case of a pediatric patient with primary myelofibrosis of infancy caused by VPS45 protein deficiency, who developed severe refractory hemolytic anemia and immune‐mediated thrombocytopenia 3.5 months following HSCT. After the failure of several treatments, he received daratumumab, an anti‐CD38 specific antibody, and demonstrated fast and sustained response. The only side effect was delayed recovery of humoral immunity. Daratumumab, by targeting antibody‐producing plasma cells, may be a valid treatment option for refractory post‐HSCT AIC.
BACKGROUND: Peripheral stem cell collections can be challenging in the pediatric population and respective experience is limited. Since February 2015 our institution is utilizing the new Spectra Optia (Optia) apheresis device, which has replaced the former COBE Spectra (COBE) device. As a quality initiative we collected and compared collection efficiency (CE2) and other collection variables between the two devices. STUDY DESIGN AND METHODS:In this retrospective study we collected and compared clinical, laboratory, and technical collection data from stem cell collection procedures done with the Optia and COBE devices. The collected data included patient demographics, precollection peripheral CD341 cell counts, total CD341 cells collected, complete blood count, electrolytes before and after collection, side effects attributed to the collection, total blood volumes processed (TBVs), collection times, and calculated CE2 and collection ratios.RESULTS: Forty-one collection procedures performed on 29 pediatric patients with the Optia device were compared to 41 collections performed on 27 patients with the COBE device. The TBVs through the Optia device were significantly smaller than the COBE (3.9 6 0.2 3 TBV vs. 5.5 6 0.1 3 TBV, respectively; p < 0.001), requiring significantly less anticoagulant and providing similar amounts of stem cells while collection times were significantly shorter (mean, 238 6 9 min vs. 264 6 9 min, respectively; p < 0.05). Collections on the Optia caused significantly smaller reductions of plasma calcium and magnesium. No significant side effects attributed to the procedure were noted.CONCLUSION: Stem cell apheresis with the Optia device in children is safe and feasible with smaller blood volumes with shorter collection times.A utologous stem cell transplantation (ASCT) is an integral part of the treatment of various pediatric cancers including high-risk neuroblastoma, various brain tumors, retinoblastoma, bone and soft tissue sarcomas, and lymphomas.1-5 The initial part of the ASCT process requires collection of stem cells, which can be challenging in the pediatric population, especially for low-weight patients such as babies and toddlers, who are more prone to hemodynamic influences of fluid shifts due to the circulating blood volumes and to the exposure to anticoagulants and who may have more difficulty with the prolonged duration of the collection procedure.6-8Our medical center performs approximately 50 ASCTs per year for pediatric patients. In February 2015 our institution introduced the novel Spectra Optia MNC (Optia) apheresis device for the collection of mobilized peripheral stem cells, which has eventually replaced the former COBE Spectra (COBE) device (both from TerumoBCT).Both devices use centrifugation for blood separation, but have some major mechanical differences. The Optia ABBREVIATIONS: ASCT(s) 5 autologous stem cell transplantation(s); BW 5 body weight; CR(s) 5 collection ratio(s); iCa 5 ionized calcium; TBV(s) 5 total blood volume(s) processed. device has a more advanced au...
BACKGROUND:The manufacturing of cellular products for immunotherapy, such as chimeric antigen receptor T cells, requires successful collection of mononuclear cells. Collections from children with high-risk leukemia present a challenge, especially because the established COBE Spectra apheresis device is being replaced by the novel Spectra Optia device (Optia) in many institutions. Published experience for mononuclear cell collections in children with Optia is lacking. Our aim was to compare the two collection devices and describe modified settings on the Optia to optimize mononuclear cell collections. STUDY DESIGN AND METHODS:As a quality initiative, we retrospectively collected and compared data from mononuclear cell collections on both devices. Collected data included patient's clinical characteristics; collection parameters, including precollection lymphocyte/ CD3 counts, total blood volumes processed, runtimes, and side effects (including complete blood count and electrolyte changes); and product characteristics, including volumes and cell counts. Collection efficiencies and collection ratios were calculated. RESULTS:Twenty-six mononuclear cell collections were performed on 20 pediatric patients: 11 with COBE and 15 with Optia. Adequate mononuclear cell products were successfully collected with a single procedure from all patients except one, with mean calculated mononuclear cell collection efficiency that was significantly higher from Optia collections compared with COBE collections (57.9 6 4.6% vs 40.3 6 6.2%, respectively; p 5 0.04). CD3-positive yields were comparable on both machines (p 5 0.34) with significantly smaller blood volumes processed on Optia. Collected products had larger volumes on Optia. No significant side effects attributed to the procedure were noted. CONCLUSION:Mononuclear cell apheresis using the Optia device in children is more efficient and is as safe as that with the COBE device.T reating patients who have therapy-resistant or high-risk, relapsed acute lymphoblastic leukemia (ALL) is challenging.1,2 Cellular therapies using chimeric antigen receptor (CAR)-transfected T cells is currently being investigated as a promising new immunotherapeutic treatment modality.3 To manufacture CAR T cells, mononuclear cells (MNCs) from the patient need to be collected beforehand. 4 Recently, manufacturer support for the commonly used COBE Spectra (COBE) apheresis device has been discontinued, and many centers are replacing the COBE device with the new Spectra Optia (Optia) device (both from Terumo BCT). Both devices use centrifugation for blood separation, but they have different operating systems and collection techniques. The COBE device operates on manual color coordination, which requires intermittent manual interface control and repositioning by the operator based on the product color and aspect of the product aspired through the collect port. The Optia device, on the other hand, automates this process through an advanced automated interface management system, which provides constant feedback ...
Hypnosis involves the manipulation of conscious attentional discrimination. The prepulse inhibition (PPI) paradigm assesses primary unconscious information processing. We investigated the correlation between hypnotizability and PPI of the startle reflex. Forty-eight healthy subjects were evaluated with the Stanford Hypnotic Susceptibility Scale, Form C (SHSS:C) and acoustic PPI. Subjects were divided into low, medium, and high hypnotizable groups. The low-hypnotizable group showed a significantly higher inhibition of the startle response, at lead intervals 60 ms and 120 ms, than did the medium-and highhypnotizable groups. We conclude that hypnotizability and PPI may be negatively correlated. These findings lend further support for the role of dopaminergic neurotransmission mechanisms in the determination of hypnotizability levels.
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