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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to conventional therapy can lead to marked disability and represents a therapeutic challenge.
To report five cases of treatment-refractory disabling CIDP treated with autologous hematopoietic stem cell transplantation (AHSCT).
This was a retrospective cohort study from a tertiary care referral center for both neuromuscular disease and AHSCT. Patients with CIDP treated with AHSCT between 2008 and 2020 were included. All patients had major persistent and disabling neuropathic deficits despite combinations of intensive immunosuppressive therapy. The primary outcome measures were: Medical Research Council sum score, Overall Neuropathy Limitations Scale and requirement for ongoing CIDP immunotherapy after transplantation. We also analyzed safety outcomes by documenting all severe AHSCT-related complications.
Five patients with refractory CIDP underwent AHSCT. Three were classified as manifesting a typical syndrome, two were classified as the multifocal Lewis Sumner variant. The mean age at time of CIDP diagnosis was 33.4 years (range 24–46 years), with a median delay of 46 months (range 21–135 months) between diagnosis and AHSCT. The median follow-up period was 41 months. All five patients were able to wean off CIDP-related immunotherapy. Marked improvements in Medical Research Council scale and overall Neuropathy Limitations Scale were noted in 4/5 patients. One patient with longstanding neurogenic atrophy showed no improvement in disability scales. There were no treatment-related deaths or critical illnesses.
AHSCT can achieve marked sustained clinical improvement of refractory CIDP and may allow for weaning off long-term complex immunotherapies.
Peripherally inserted central catheters (PICCs) are a mainstay of nonpermanent vascular access devices. In this study, we assessed patients displaying anaphylaxis or anaphylactoid reactions to the PowerPICC SOLO and Groshong PICC (Bard Access Systems) using the Sherlock tip locating system (TLS).
Patients from 2 tertiary-care hospitals were systematically monitored over 4 years for adverse events following the insertion of a PICC using the Sherlock TLS. Insertion data were also collected using the BioFlo PICC (Angiodynamics)from a third hospital site and from The Ottawa Hospital over 4 years as an additional comparator. Three definitions of anaphylaxis and anaphylactoid reactions were utilized, and the Cohen κ was used to assess interrater agreement. Analysis of reactions among the patient cohorts was performed using the χ2 test with Yates correction or the Fisher exact test as appropriate.
Among 8,257 insertions using the TLS PICCs, 37 potential reactions (0.45%) were recorded. Using specific definitions for anaphylaxis or anaphylactoid reactions, 54.1%–91.9% met criteria. Comparator populations using data from Calgary (n = 491) and Ottawa (n = 7,889) using the BioFlo PICC insertion found no reactions. Anaphylactic or anaphylactoid reactions were significantly associated with the PowerPICC SOLO and Groshong PICC with the TLS compared to the BioFlo PICC (P < .0001) across all definitions. The largest subset of patients experiencing adverse reactions had cystic fibrosis (CF) (n = 4, 10.8%).
Our study results demonstrate significant adverse events associated with the PowerPICC SOLO and Groshong PICC using the Sherlock TLS inserted across a range of patient populations. The incidence rate of anaphylaxis or anaphylactoid reactions in the CF population at our center is significantly higher than in non-CF patients (P < .001).
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