Transfusion and Apheresis Science最新文献

Objectives: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated with severe deficiency in ADAMTS13. ADAMTS13 deficiency may be secondary to absent or dysfunctional protein production due to mutations in the ADAMTS13 gene (congenital TTP) or autoantibody-mediated clearance and/or inhibition (immune-mediated TTP). This autoimmunity may, albeit rarely, occur secondary to certain medications (eg, ticlopidine). Recent case reports have implicated alemtuzumab (LETRADA), a monoclonal antibody that selectively inhibits CD52, as a cause of secondary TTP. We aimed to characterize all reports of TTP potentially associated with alemtuzumab.

Methods: We performed a cross-sectional analysis of the United States Food and Drug Administration's Adverse Event Reporting System (FAERS) database as of 21 November 2024 and systematically reviewed the literature as of 03 September 2024 for all reported cases of secondary TTP potentially associated with alemtuzumab. Patient demographics, therapy indications, associated medications, and outcomes were abstracted.

Results: We identified 49 reports of TTP possibly related to alemtuzumab administration since 01 January 2001 in the FAERS database, 9 of which resulted in death. Most patients (n = 31) were receiving alemtuzumab for multiple sclerosis (MS), while 8 reports were in patients undergoing hematopoietic stem cell transplantation. We identified two additional cases in the literature review in patients receiving alemtuzumab for MS.

Conclusions: In conjunction with studies of the United Kingdom's and European Union's pharmacovigilance databases, these results support the current package insert for alemtuzumab in which TTP is listed as a "warning and precaution". Increased awareness of this possible side effect, and prolonged monitoring, is warranted.

Introduction: The Reveos automated blood processing system is the only system developed till date, which can separate whole blood into components on complete automation. Their proprietary LR and NLR blood collection sets have their own advantages and disadvantages. Using LR sets, leukodepleted components can be prepared but individual platelet units cannot be prepared. Using NLR sets, individual platelet units can be prepared, but components are non-leukodepleted. The newly launched LR EXT with the apparent advantage of both LR and NLR sets have been evaluated in this pilot study.

Methodology: Blood components prepared from 31 LR EXT sets were evaluated in this study in comparison with the National regulations and with components routinely prepared from LR and NLR sets.

Results: Excluding under-collected and sero-reactive units, components prepared from 27 LR EXT sets were evaluated for their quality. QC of PRBC units prepared had a mean volume of 296.86 ml, Hct of 59.2 % and WBC count as low as 0.06 × 106 per bag. FFP units had a mean volume of 221.89 ml, mean Fibrinogen of 432.47 mg and FVIII levels of 98.13 IU per bag. PLT units had a mean volume of 73.07 ml, PLT count of 5.74 × 1010 and WBC count of 3.21 × 108 per bag.

Conclusion: The use LR EXT blood collection sets help in achieving adequate inventory of both LD-PRBCs and RDPs especially in blood centres with a lower daily collection rate.

This case report presents first case of RHD*weak D type 9 in a 38-year-old Indian patient with severe osteoarthritis of the left hip joint scheduled for total hip replacement surgery. During routine blood grouping, an unexpected weak reaction with anti-D was observed. Serological characterization using an extended partial D typing kit characterized the variant as DV. The patient was successfully transfused with a O RhD negative compatible unit as per transfusion guidelines after surgery, without any observed transfusion reactions. The blood sample was sent to the ICMR-National Institute of Immunohematology, Mumbai, where molecular characterization revealed the presence of an RHD* 01 W.9 allele, as the underlying cause of weak expression of the RhD antigen. Serology can at best lead to the detection of the presence of a variant but characterization requires the help of molecular techniques.

Background and objectives: This study aimed to assess the seroprevalence of syphilis among blood donors, evaluate the response rate of seroreactive donors, and investigate high-risk behaviors.

Material and methods: The study presents a retrospective analysis of syphilis seroreactivity among blood donors over a 17 year period. Blood donations were screened for syphilis using the Rapid Plasma Reagin (RPR) card test. RPR-reactive blood units were discarded, and donors were notified for follow-up. A detailed history was obtained, including prior syphilis diagnoses, sexual practices, and potential high-risk activities. The donors who were repeatedly RPR reactive were referred for further confirmatory management.

Results: A total of 569 (0.20 %) donors were found RPR reactive. A non-linear trend was observed between RPR reactivity and study period, donor age and donation status. RPR reactivity was significantly higher in repeat blood donors (p < 0.001). The mean donor return rate was significantly higher following introduction of phone calls along with letters for donor notification compared to notification via letters only (p < 0.001). Forty-five percent (n = 241) donors returned for repeat sample testing. Of these, 19 % (n = 45) tested negative on repeat RPR testing while 81.3 % (n = 196) remained RPR reactive and were referred for further management. On follow up of the latter group, 43.9 % (n = 86) donors were negative on confirmatory testing indicating biological false positives while 56.1 % (n = 110) donors were positive on confirmatory testing and were true positives. Of true positive donors, 90.9 % of donors reported engaging in high risk sexual behaviours.

Conclusion: There is a need to update syphilis screening guidelines in developing countries like India, incorporating combination testing to improve the accuracy of syphilis detection in blood donors.

Background: Neuroblastoma (NB) is the most common extracranial solid tumor in pediatric. In highrisk NB patients, the 5-year overall survival rate (OS) remains a stark < 50 % with conventional therapies. Autologous hematopoietic stem cell transplantation with high dose chemotherapies was used in poor prognosis and high-risk patients.Today, Plerixafor is used to increase stem cells mobilization in patients who are candidates for autologous transplantation.

Objective: This study examined safety and efficacy Plerixafor is administered as a subcutaneous injection in pediatric NB patients for stem cell mobilization STUDY DESIGN: A cohort of 19 pediatric neuroblastoma (NB) patients underwent autologous hematopoietic stem cell transplantation (HSCT) between February 2017 and April 2019, receiving G-CSF mobilization only. Subsequently, 37 NB patients underwent HSCT between December 2019 and October 2023, receiving both G-CSF and plerixafor for mobilization (auto-HSCT).

Results: The final product CD34 cell dose /kg was evidently higher in combination group at 5.363 ± 4.243 vs. G-CSF group at 2.827 ± 3.586 × 106(P value= 0.001). Neutrophils and platelet engraftment were occurred sooner in combination group compared with G-CSF group. The 1-year overall survival (OS) rate for the G-CSF and G-CSF-and-plerixafor combination group was 70.8 % and 63.3 %, respectively (P = 0.874). No statistically significant difference in OS or disease-free survival (DFS) was observed between the two treatment groups.

Conclusion: The results show that plerixafor may be safe and effective in NB pediatric patients in routine clinical practice. It was well tolerated in NB patients and no specific side effects were observed. It was not associated with improved survival.

Background: Splenectomy is frequently performed in transfusion-dependent thalassemia (TDT) patients to lower blood transfusion needs but is associated with significant long-term complications, including sepsis, thrombosis, and pulmonary hypertension. This study examines the long-term complications, survival rates, and causes of mortality among adult patients with TDT who have undergone splenectomy in a low and middle-income country (LMIC).

Methods: A retrospective analysis was conducted on 103 adult TDT patients (≥18 years) who underwent splenectomy between July 2013 and March 2024. Data collected included demographic and clinical characteristics, haematological parameters, transfusion requirements before splenectomy and at the last follow-up, survival rates, complications, and mortality causes.

Results: The median age at splenectomy was 12 years (range 5-34). The majority (98 %) underwent open splenectomy. The yearly transfusion volume decreased from 276.7 ml/kg/year pre-splenectomy (range 207-433) to 146.2 (range 0-252.9) post-splenectomy at the last follow-up, p < 0.0001. Three patients were completely transfusion-free at the last follow-up. Complications included pulmonary hypertension in 10 (9.7 %), thrombosis in 5 (4.8 %), and overwhelming post-splenectomy infection (OPSI) in 4 (3.9 %). The iron-overload-related complications included cardiomyopathy in 17 (16.5 %), endocrinopathy in 56 (54.3 %), chronic liver disease in 15 (14.5 %) and hepatocellular carcinoma in 2 (0.9 %). The 15-year post-splenectomy overall survival (OS) was 84.7 % (95 % CI- 77.3 % - 92.8 %), with 17 deaths (16.5 %) recorded. Iron-overload-related cardiomyopathy was the leading cause of death in 8 (53.3 %).

Conclusion: Splenectomy significantly reduces transfusion requirements in TDT patients but is associated with risks such as thrombosis, pulmonary hypertension, and OPSI. Long-term mortality is primarily driven by iron-overload-related cardiomyopathy.

Background: Hemophilia B, or Christmas disease, is a hemorrhagic inherited disorder. Previous studies have reported measurement discrepancies in factor VIII activity between clot-based and chromogenic assays in approximately one-third of patients with non-severe hemophilia A. However, similar discrepancies in hemophilia B have been less extensively studied. This research compares clot-based and chromogenic assays in 33 patients with non-severe hemophilia B and investigates the mutations associated with these discrepancies.

Methods: Citrate and EDTA samples were collected from 33 patients with non-severe hemophilia B at Iran's hemophilia comprehensive care center. Clinical information was also gathered. Both clot-based and chromogenic assays were performed on these patients. DNA was extracted from the EDTA samples for those with discrepancies in the test results, and PCR was conducted to sequence their genes to find mutations.

Results: Among 33 plasma samples from patients with non-severe hemophilia B, 7 showed a measurement discrepancy according to the definition of ISTH (<0.5, >2.0, or an absolute difference >10), which includes both reverse and classic types of discrepancies. In this study, mutations that previously did not show contradictory results now exhibit discrepancies. A difference in classification was observed in 21 % of the patients.

Conclusions: The findings indicate that the impact of specific mutations varies depending on the assay conditions. In addition to mutations, other factors also play a role in this discrepancy. Both types of assays are essential for the accurate diagnosis and classification of hemophilia B.

The pregnancy of a patient with homozygous familial hypercholesterolemia (HoFH) represents a challenge in the clinical setting due to the high cardiovascular risk of the mother and maternal-fetal morbidity. The lipid lowering drugs are generally contraindicated and lipoprotein apheresis (LA) is the only accepted treatment in HoFH pregnant woman. Liposorber D, an LA technique on whole blood, has good efficacy, safety, and short operative time. We present a 31-year-old Caucasian pregnant woman with HoFH clinical phenotype on lipid-lowering treatment with Lomitapide and Evolocumab, discontinued during pregnancy. In multidisciplinary team it was decided to submit the patient to LA throughout the pregnancy. Liposorber D sessions (a whole blood technique) were performed on a strict weekly frequency. The treatment resulted in massive decrease of total cholesterol (TC) and LDL cholesterol (LDL-c), with no significant side effects. The pregnancy presented a normal course and a cesarean section was performed on clinical indications unrelated to the use of LA. She gave birth to a healthy male child at 37 weeks of gestation. LA is considered the only effective, safe and accepted therapy during HoFH pregnancy. This is the first reported case of successful pregnancy treated with Liposorber D, a whole blood LA technique. LA with dextran sulfate has been an effective and safe choice for the mother and fetus. Furthermore it was reasonably well tolerated from the beginning of pregnancy management to its conclusion.