Fatal outcome in a patient with autoimmune hemolytic anemia associated with an IgM bithermic anti-ITP

BACKGROUND: Several cold autoantibodies (usually IgG) with IT specificity have been reported previously, as have autoantibodies with joint I and P blood group specificities (IP1, ITP1, iP1, IP). A fatal outcome associated with an IgM cold autoantibody of ITP specificity is reported. CASE REPORT: A 54-year-old man suffered from progressively severe cold autoimmune hemolytic anemia for 9 months. Hemoglobin concentration ranged from 6 to 7 g per dL (60-70 g/L) and reticulocytes from 3 to 5 percent (0.030-0.050). The direct antiglobulin test was weakly positive for IgM and strongly positive for C3d. The serum contained a cold agglutinin that reacted strongest with cord i red cells (RBCs) > adult I RBCs > adult i RBCs, which is consistent with IT specificity. The Donath-Landsteiner test was positive; the reaction was neutralized by globoside. The serum reacted weakly or was negative with RBCs from five group p blood donors, which suggests anti-ITP specificity. Dithiothreitol treatment of the serum abolished the cold agglutinin reactivity, which suggests that the anti-IT was IgM. The patient received > 40 RBC transfusions and failed to respond to oral steroids, oral cytoxan, high-dose pulse intravenous steroids, and plasma exchange at room temperature and at 35 degrees C. He died of sepsis following an unsuccessful trial of chlorambucil. Autopsy revealed unsuspected disseminated non-Hodgkin's lymphoma. CONCLUSION: Serologic studies are consistent with our patient's having a single IgM cold autoantibody with IT and P specificities (anti-ITP) and requiring both specificities on the same RBC to permit maximal antibody expression.     

Invalidation of antiglobulin tests by a high thermal amplitude cryoglobulin

A multiply transfused patient was referred for evaluation of a transfusion reaction. The direct and indirect antiglobulin tests (DAT, IAT) for alloantibody were negative. However, IgG-coated control cells failed to agglutinate in the negative reactions, casting doubt on their validity. At 4 degrees C, the patient's serum exhibited a large cryoprecipitate (2.9 mg/mL), made up predominantly of an IgG kappa paraprotein and having trace amounts of IgM and C3. Clear serum separated at 37 degrees C became cloudy within 10 minutes at room temperature (RT); within 4 hours, approximately 60 percent of the total precipitable cryoprotein had precipitated. Red cells (RBCs) incubated in fresh serum that had cooled to RT or RBCs obtained from RT or refrigerated samples contained cryoprecipitate that sedimented with the RBCs during washing with RT saline. On resuspension, enough IgG cryoglobulin redissolved to neutralize completely the commercial anti-IgG reagents. If the patient's samples were maintained at 37 degrees C, cryoprecipitate did not form, and RBCs washed four times at 37 degrees C gave valid DAT and IAT reactions. The removal of all cryoprecipitate from the patient's serum by centrifugation after overnight incubation at 4 degrees C also made possible valid antibody screening and compatibility tests.     

保存血小板的体外评估:是否有希望预见输入后血小板的生存和功能

输入病人体内的血小板的循环能力和功能与体外保存损害和受者的体内环境有关。患有血小板减少症的住院病人,体内影响血小板存活和功能的因素比体外保存损害的影响更重要。然而人们长久以来一直认为,预测血小板成分在体内的作用可以为现行的输血措施提供有价值的参考,也可能进一步提高临床疗效,同时更有效的管理血小板贮存。确实,新的病原菌灭活方法推动了临床应用,同时可能使浓缩血小板保存更安全、保存时间更长,精确评定临床有意义的浓缩血小板质量。 保存血小板在输血后的存活和功能差异较大。如果血小板处理小心,也没有长时间保存(采集后24—48小时内)便很快     

Efficacy of cannabidiol in convulsive and nonconvulsive seizure types associated with treatment-resistant epilepsies in the Expanded Access Program

The cannabidiol (CBD) Expanded Access Program (EAP), initiated in 2014, provided CBD (Epidiolex) to patients with treatment-resistant epilepsy (TRE). In the final pooled analysis of 892 patients treated through January 2019 (median exposure = 694 days), CBD treatment was associated with a 46%–66% reduction in median monthly total (convulsive plus nonconvulsive) seizure frequency. CBD was well tolerated, and adverse events were consistent with previous findings. We used pooled EAP data to investigate the effectiveness of add-on CBD therapy for individual convulsive seizure types (clonic, tonic, tonic–clonic, atonic, focal to bilateral tonic–clonic), nonconvulsive seizure types (focal with and without impaired consciousness, absence [typical and atypical], myoclonic, myoclonic absence), and epileptic spasms. CBD treatment was associated with a reduction in the frequency of convulsive seizure types (median percentage reduction = 47%–100%), and nonconvulsive seizure types and epileptic spasms (median percentage reduction = 50%–100%) across visit intervals through 144 weeks of treatment. Approximately 50% of patients had ≥50% reduction in convulsive and nonconvulsive seizure types and epileptic spasms at nearly all intervals. These results show a favorable effect of long-term CBD use in patients with TRE, who may experience various convulsive and nonconvulsive seizure types. Future controlled trials are needed to confirm these findings.     

Interdisciplinary interactions,social systems and technical infrastructure required for successful implementation of mobile stroke units: A qualitative process evaluation

Rationale

Mobile stroke units (MSUs) are increasingly being implemented to provide acute stroke care in the prehospital environment, but a comprehensive implementation evaluation has not been undertaken.

Aim

To identify successes and challenges in the pre- and initial operations of the first Australian MSU service from an interdisciplinary perspective.

Methods

Process evaluation of the Melbourne MSU with a mixed-methods design. Purposive sampling targeted key stakeholder groups. Online surveys (administered June–September 2019) and semistructured interviews (October–November 2019) explored experiences. Directed content analysis (raters' agreement 85%) and thematic analysis results are presented using the Interactive Sociotechnical Analysis framework.

Results

Participants representing executive/program operations, MSU clinicians and hospital-based clinicians completed 135 surveys and 38 interviews. Results converged, with major themes addressing successes and challenges: stakeholders, vehicle, knowledge, training/education, communication, work processes and working relationships.

Conclusions

Successes and challenges of establishing a new MSU service extend beyond technical, to include operational and social aspects across prehospital and hospital environments.