فهرست مطالب

  • Volume:14 Issue: 4, 2020
  • تاریخ انتشار: 1399/08/03
  • تعداد عناوین: 8
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  • Fayez Abdulrazeq, Monica Matsumoto, Reem Abduljabbar, Amira Al-Hajj, Melad Alayash, Rahaf Ballourah, Sumayya Issak, Zubeida Issak Pages 213-225
    Background

    The expansion of umbilical cord blood (UCB) banking necessitates a greater understanding among obstetricians, who are responsible for informing parents about UCB collection and storage. Gaps in knowledge can compromise public UCB banking efforts and result in missed opportunities and misguidance about UCB banking.

    Methods

    A cross-sectional survey was disseminated among obstetricians in Amman, Jordan. The questionnaire aimed to evaluate obstetricians’ knowledge of and attitude toward UCB storage and applications, as well as current practice patterns.

    Results

    Ninety-six obstetricians responded (55% response rate), most of whom were Jordanian (71%), female (83%), resident physicians (59%), and working in either private (43%) or public (42%) hospitals, with an average of 6.5 years in practice. Only 26% had personal experience in UCB collection, and only 20% had received education on UCB collection. Nearly three-fourths said their hospitals lacked standard operating procedures, guidelines, or infectious disease screening for UCB units. Overall knowledge about UCB was moderate, with the internet as the most common source (54%). Overall attitudes were positive, especially in desire to expand personal knowledge about UCB, integrate information into medical residency curricula, and establish a public UCB bank in Jordan. However, many believed that ethical (61%) and religious (56%) controversies surround UCB donation.

    Conclusions

    This study identifies deficiencies in quality control and experience in UCB collection in Jordan, as well as areas of inadequate knowledge, false beliefs, and negative attitudes among obstetricians. These issues can contribute to public misinformation and should be addressed through improved medical education on this topic.

    Keywords: Umbilical cord blood banking, Obstetricians, Jordan, Knowledge, Attitudes
  • Rony Benson, Sreejith Nair *, Geetha Narayanan Pages 226-231
    Background

    The half-life of free light chain is short and can be used as an early marker for tumor response in patients with multiple myeloma [MM]. This prospective study is aimed at evaluating whether early light chain response can predict response to treatment in patients with MM.

    Materials and Methods

    Thirty six patients with a diagnosis of MM and with an abnormal to normal light chain ratio of > 10 were included in this study.

    Results

    The median age at presentation was 56 years. Fourteen patients had lambda light chain disease, whereas 22 patients had kappa light chain disease. Twenty-four patients [66.6%] had reduction of abnormal to normal light chain ratio to < 10 after 2 cycles, of whom 15 [62.5%] achieved a CR or VGPR after 6 cycles. Among 12 patients who did not have reduction of abnormal to normal light chain ratio to < 10, only 1 patient achieved CR while 11 patients [91.6%] achieved a PR or less[Fishers exact p=0.004]. Median follow-up was 13 months. Median progression-free survival for the entire cohort was 15 months. One-year Progression-Free Survival was 77% vs 57.1%, [p= 0.008], respectively for patients with early normalization and those who did not show early normalization.

    Conclusion

    Early light chain response after 2 cycles of chemotherapy is a good predictor for treatment response in patients with MM treated with bortezomib based chemotherapy. Treatment intensification based on early light chain response merits further evaluation in a prospective trial

    Keywords: Light chain response, Multiple myeloma, Prognostic marker
  • Rafet Eren, Bahar Sevinçoğlu, Esra Doğan, Demet Aydın, Nihan Nizam, Naciye Demirel Pages 232-236
    Background

    We evaluated the frequency of subnormal erythropoietin levels, JAK2V617F positivity and polycythemia vera (PV) in patients who did not meet WHO 2008 criterion for hemoglobin levels but were suggested to be investigated for PV in 2016 revision.

    Materials and Methods

    We assessed the data of 92 patients, who were further evaluated with JAK2V617F mutation and serum erythropoietin (EPO) levels and bone marrow biopsy, if necessary. We also compared this patient group with 20 patients whose Hgb>18.5 g/dL for men and >16.5 g/dL for women.

    Results

    Nine patients (45%) in the higher hemoglobin group were JAK2V617F positive, while 4 patients (4.3%) in the lower hemoglobin group were JAK2V617F positive (p<0.001). The number of patients with serum EPO levels <4.3 mIU/mL was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=7, 7.6%) (p<0.001). Finally, the number of patients who received a diagnosis of PV was significantly higher in the higher hemoglobin group (n=13, 65%) than the lower hemoglobin group (n=9, 9.8%) (p<0.001).

    Conclusion

    We found a substantial increase in patients who were candidates for testing for PV with the introduction of WHO 2016 criteria; these patients were diagnosed with PV with a rate (9.8%) that cannot be underestimated.

    Keywords: Polycythemia vera, JAK2V617F mutation, Serum erythropoietin, WHO 2016 revision
  • Mehrnaz Ahmadi, Mahin Gheibizadeh*, Maryam Rassouli, Abbas Ebadi, Marziyeh Asadizaker, Mojtaba Jahanifar Pages 237-247
    Background

    Uncertainty leads to a stressful situation in patients with thalassemia major that can dramatically affect their psychosocial coping ability, treatment process and disease outcomes, and reduce patients' quality of life. As one of the important factors affecting the health of thalassemia patients, understanding the concept of uncertainty is of major importance to health care providers especially nurses as the first line of exposure to these patients. The present study aimed to explore the experiences of uncertainty in patients with thalassemia major.

    Materials and Methods

    The present qualitative study was conducted through in-depth face-to-face semi-structured interviews held with 18 patients with major thalassemia selected through purposive sampling. Interviews continued until saturation of data. All interviews were recorded, transcribed and analyzed with conventional content analysis method of Landman and Graneheim using MAXQDA10 software.

    Results

    Two main themes, including 'living in the shadow of anxiety' and 'coping with uncertainty' emerged from patients’ experiences of illness uncertainty of thalassemia. 'Living in the shadow of anxiety' included four categories of 'fear of complications', 'contradictory views on treatment', 'unknown future' and 'stigma'. 'Coping with uncertainty' included three categories of 'spiritual coping', 'psychosocial coping' and 'knowledge acquisition'.

    Conclusion

    According to the results of this study, uncertainty is a major psychological stress in patients with thalassemia major. Healthcare providers should therefore consider the challenges and concerns faced by patients and, through utilizing appropriate training and communicational practices, plan interventions and strategies to empower patients for coping with uncertainty.

    Keywords: Patients with thalassemia, Uncertainty, Qualitative study, Iran
  • Hojat Shahraki, Akbar Dorgalaleh, Majid Fathi, Shadi Tabibian, Shahram Teimourian, Hasan Mollanoori, Alireza Khiabani, Farhad Zaker* Pages 248-256

    Congenital factor XIII (FXIII) deficiency is an extremely rare bleeding disorder (RBD) with estimated prevalence of one per 2 million in the general population. The disorder causes different clinical manifestations such as intracranial hemorrhage (ICH), recurrent miscarriage, umbilical cord bleeding, etc. High incidence of the disorder might be due to founder effect. To assess founder effect, haplotype analysis is an important step. For this purpose, suitable and reliable genetic markers such as microsatellites (Hum FXIII01 and HumFXIIIA02) and single nucleotide polymorphisms (SNP) are suggested. In the present study we tried to describe evaluation of founder effect in patients with congenital FXIII deficiency via haplotype analysis using suitable genetic markers.

    Keywords: Factor XIII deficiency, Intracranial hemorrhage, Founder effect, Genetic Markers
  • Aysun Senturk Yikilmaz*, Sema Akinci, Sule Bakanay, İmdat Dilek Pages 257-265
    Background

    Complete response (CR) and very good partial response (VGPR) are targeted with pre-ASCT induction regimens in patients by diagnosed multiple myeloma (MM), who are candidates for ASCT. In this study, it was aimed to compare the response and survival evaluations of cases who underwent induction treatment by vincristine-doxorubicin-dexamethasone (VAD) protocol versus bortezomib containing regimens.

    Materials and Methods

    The data of 96 ASCT eligible patients, retrospectively analyzed. P value> 0.05 was considered statistically significant.

    Results

    While 66 cases had received bortezomib containing regimens as induction regimen, 30 cases had received VAD protocol. The total survival was 91.3 (st.s 6) months and 43 (st.s 7.9) months, respectively, when we compared the cases without ASCT and with ASCT (p = 0.001). The OS of patients who underwent ASCT after reaching at least VGPR was longer than the underwent ASCT without reaching VGPR (p=0.019).  Post-ASCT PFS (p=0.717) and OS (p = 0.126) analyzes were performed in 74 cases undergoing ASCT treatment, there was no significant statistical difference when patients with treated by VAD protochol and treated by bortezomib containing regimens as pre-ASCT induction regimens was compared to each other.

    Conclusion

    Whatever the type of induction regimen is, the level of response achieved before ASCT is important. The survival of the myeloma patients are much more influenced with HDT-ASCT as well as post-transplantation strategies to keep the patients in remission. Even though it is outdated, we think that the VAD protocol may be an option in patients who are not responding with the new generation of agents in the following days.

    Keywords: Multiple myeloma, VAD, Autologous stem cell transplantation, Bortezomib
  • Amirhosein Maali, Elaheh Ferdosi Shahandashti *, Farzin Sadeghi, Ehsan Aali Pages 266-273
    Background

    Adult T-cell leukemia/lymphoma (ATLL) is a poor prognostic Hematopoietic malignancy with various therapeutic challenges, which had been classified as non-Hodgkin lymphoma.  The Drug switching, as a novel, innovative and promising approach, is an opportunity to overcoming on therapeutic challenges of hard-treating disease, e.g. ATLL. Our aim is evaluating the antiproliferative and apoptotic effect of Mebendazole (MBZ) on ATLL cancer cells in in-vitro conditions.

    Materials and Methods

    We used Jurkat cell-line as ATLL cancer cells. After treatment of MBZ in different concentrations on jurkat cells, the cell viabilities were determined by MTT assay. After IC50 value determination, the 24-, 48- and 72-h treatments had been performed in IC50 concentration and control to evaluating the quantitative apoptosis rate by Annexin/PI Flowcytometry and qualitative apoptosis by DAPI Nuclear staining. Also, Glucose spectrophotometry were performed to evaluate the reduced amount of glucose uptake through MBZ treatment.

    Results

    MBZ inhibits proliferation of jurkat cells and IC50 value had been estimated 10 μM (P< 0.01). According to the flowcytometric results, increasing in drug concentration is associated with decrease cell viability and the percentage of full-apoptosis. However, it inversely correlates with percentage of early-apoptosis rate. Also, the microscopic captures of DAPI Nuclear staining confirms the flowcytometry results in qualitative manner. In addition, it was found that inhibition of glucose uptake was inversely correlated with increased MBZ concentration (P< 0.05).

    Conclusion

    MBZ potentially inhibits the proliferation of ATLL cancer cells in in-vitro condition. MBZ inhibits the growth of Jurkat cells by inducing apoptosis. Also, we suggest that indirectly inhibition of Glucose transporting occurs by MBZ, which could induce apoptosis in cancer cells.

    Keywords: Adult T-cell leukemia, lymphoma, Mebendazole, Drug switching, Apoptosis
  • Lucio Henrique Sousa Pinheiro, Louise Trindade, Fernandes De Oliveira Amanda Costa, Nathanielly De Lima Silva, Alex Freire Sandes, Marco Antônio Prado Nunes, Cristiane Bani Correa, Carlos Arthur Cardoso Almeida, Geydson Silveira Da Cruz, Divaldo Pereira De Lyra Júnior, Dulce Marta Shimieguel Pages 274-288
    Background

    The aim of this review was to evaluate the influence of aberrant phenotypes in prognosis and survival in acute myeloid leukemia (AML) patients by multiparametric flow cytometry.

    Materials and Methods

    Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a review of PubMed, Scopus, Science Direct and Web of Science was carried out through 1998 to 2016, conducted by two reviewers independently, evaluating titles, abstracts and full-texts of the selected studies.

    Results

    Ten studies were included on this review, in which the aberrant phenotype expression of 17 markers were detected in AML patients. From these, 11 aberrant phenotypes were associated with prognosis, which eight had shown negative impact on prognosis: CD7, CD56, CD15, CD2, CD3, CD90low, CD123high, CD117high, and three others were associated with good prognosis: CD19, CD98high and CD117+/CD15+. Meta-analysis showed that aberrant expression of CD56 as a poor prognostic marker with unfavorable outcomes is implicated in decreased overall survival in AML patients in 28 months (95% CI: 0.62 to 0.92).

    Conclusion

    This was observed when there was association between CD56 expression and other prognostic factors, influencing on patients’ management care and treatment.

    Keywords: Aberrant phenotype, Acute myeloid leukemia, Immunophenotyping, Prognosis, Survival