Journal of Applied Hematology最新文献

{"title":"Anticoagulant status under COVID-19: The potential pathophysiological mechanism","authors":"B. Marzoog","doi":"10.4103/joah.joah_154_21","DOIUrl":"https://doi.org/10.4103/joah.joah_154_21","url":null,"abstract":"Coronavirus disease 19 (COVID-19) is a systematic multitropic disease. Sustaining blood homeostasis is a mission of multiple factors includes procoagulant and anticoagulant systems. Subsequently, maintaining a precise equilibrium between these antagonists' systems is crucial to prevent hemostasis. Frequently, during severe forms of COVID-19 patients, studies reported a discrepancy between the procoagulant and anticoagulant that usually results in fatal outcomes through multiorgan ischemia by thrombotic events. The proper interpreting of the anticoagulant status changes in COVID-19 patients is sufficient for effective and safe anticoagulant treatment. Our postulation is the first to address the pathophysiology of anticoagulant status under COVID-19.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"167 - 171"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48569869","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survival outcomes of pediatric hematopoietic stem cell transplant patients admitted to the intensive care unit: A case–control study from a tertiary care center in Saudi Arabia","authors":"Lujain Aljudaibi, M. Bayoumy, H. Altrabolsi, A. Alzaydi, Nawaf Aldajani, Nadia Hammad, Ismail Alzahrani, Marwa Elhadidy, I. Abosoudah","doi":"10.4103/joah.joah_66_22","DOIUrl":"https://doi.org/10.4103/joah.joah_66_22","url":null,"abstract":"BACKGROUND: Hematopoietic Stem Cell Transplantation (HSCT) increases demand on Paediatric Intensive Care Units (PICU) and survival outcomes has emerged as an area of interest in HSCT research. AIMS AND OBJECTIVES: The aim of this retrospective case-control study is to estimate the incidence and survival outcomes, and identify variables associated with survival amongst HSCT patients: MATERIALS AND METHODS: Patients aged ≤15 years transplanted during January 2016 to December 2019 and requiring PICU admission(s). Statistical analysis involved calculating incidence of PICU admission, 2-year overall survival using Kaplan-Meier method; univariate and multivariate logistic regression analysis performed to identify factors associated with outcomes and P-value of <0.05 considered significant. RESULTS: Total of 130 patients underwent 150 HSCT, 31 (24%) of patients required 50 PICU admissions, discharge following PICU admission was observed in 36 (72%) admission episodes, while 14 (28%) associated with patient demise. Two- year Overall Survival (OS) amongst HSCT patients requiring PICU admission was 58% compared to 96.6% amongst patients not requiring PICU (P<0.001). subgroup analysis of cases with malignant disorders and non-malignant disorders showed similar results (P<0.001). Univariate analysis revealed reduced intensity conditioning, no engraftment at the time of PICU admission, and sepsis associated with decreased survival, however multivariate analysis revealed sepsis as the single independent prognostic factor for decreased survival. Our study reports 24% incidence of PICU admission amongst patients undergoing HSCT with a 28% mortality rate following PICU admission. CONCLUSION: Sepsis identified as the single prognostic factor associated with decreased survival. Therefore, efforts to optimize early identification and management of sepsis in the high-acuity setting of pediatric HSCT is warranted","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"192 - 200"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48505226","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Drug therapy in patients with severe forms of sickle cell anemia: A nonrandomized clinical trial of combining l-carnitine with hydroxycarbamide therapy","authors":"Safaa A. Khaled, Israa M. Ashry","doi":"10.4103/joah.joah_23_22","DOIUrl":"https://doi.org/10.4103/joah.joah_23_22","url":null,"abstract":"BACKGROUND AND AIM: Combining Hydroxycarbamide (hydroxyurea) therapy and L-Carnitine (HU+LC) was proved effective and tolerable in patients with thalassemia intermedia; however, its role in sickle cell anemia (SCA) was not investigated. This study aimed to assess various aspects of treatment with HU + LC in adult patients with severe forms of SCA. PATIENTS AND METHODS: Ninety-one adult SCA patients were recruited, then distributed into four treatment groups: Group 1 – 37 patients treated with HU + LC; Group 2 – 16 patients treated with HU; Group 3 – 18 patients treated with LC; and Group 4 – 20 patients received supportive treatment only. Their baseline data were recorded then reassessed 8–12 weeks later. Their compliance was assessed subjectively. RESULTS: Patients were nearly matched as regards gender, age, baseline hematologic, and biochemical data. Compared to baseline, HU + LC treatment significantly increased hemoglobin and reduced reticulocytes, white blood cells, mean corpuscular hemoglobin concentration, and lactate dehydrogenase (LDH). Compared with other lines, HU + LC significantly reduced number of painful episodes, blood transfusions, and in hospital days per year. Adding LC to HU improved patient compliance, with mild side effects. Residence, side effects, baseline hemoglobin (Hb), and Hb F% affected patients' compliance. Both gender and age did not show any influence on response to treatment while side effects, compliance, baseline Hb, and LDH displayed a significant effect. CONCLUSION: Combining LC with HU for the treatment of adult SCA patients showed superior hematologic and clinical effectiveness. The response was more obvious in severe cases. However, health education about the combination is needed to improve patients' compliance and therapeutic responses. Trial registration: Clinical Trials.gov, NCT05081349. https://clinicaltrials.gov/ct2/show/NCT05081349.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"237 - 248"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42423926","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patient activation measure among transfusion-Dependent patients with hemoglobinopathies","authors":"M. Badawi, Sultan Alkhamesi, Dana Alsulaimani, Aseel Khodary, Khulud Almutairi, Roaa Alsolaimani, Salwa Al-Najjar","doi":"10.4103/joah.joah_12_22","DOIUrl":"https://doi.org/10.4103/joah.joah_12_22","url":null,"abstract":"CONTEXT: Patient's sense of control over their disease management (patient activation) is reported to be associated with better disease outcomes in many chronic conditions. AIMS: The aim of this study is to assess patient activation levels among adult transfusion-dependent patients with hemoglobinopathies. The secondary aims were to determine whether the level of patient activation is associated with different disease outcomes and whether an association exists between higher levels of activation and specific patient characteristics. SETTINGS AND DESIGN: Participants were recruited from the day-care unit in the period between November 2019 and April 2021. Patients who agreed to participate were asked to provide demographic and socioeconomic data and complete the patient activation measure (PAM-10) in Arabic. Hospital records were reviewed for the retrieval of clinical data. RESULTS: The total number of participants was 84. The mean PAM score was 52.90 ± 10. Of all the participants, 21 (25%) had very low levels of activation (Level 1), 38 (45.2%) were categorized as Level 2, 21 (25%) were categorized as Level 3, while 4 (4.8%) were categorized as Level 4 (highest activation level). Disease outcomes were not different among patients by level of patient activation. CONCLUSIONS: Our results show that transfusion-dependent patients with hemoglobinopathies have low levels of activation. No association was found between patient activation and clinical disease outcomes.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"263 - 267"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47354922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immune tolerance induction in a severe hemophilia B child with low titer inhibitors","authors":"A. AlMulla, Najam Awan, Faisl Khanani","doi":"10.4103/joah.joah_165_21","DOIUrl":"https://doi.org/10.4103/joah.joah_165_21","url":null,"abstract":"Hemophilia B is an X-linked inherited bleeding disorder caused by either the absence or reduced biosynthesis of clotting factor IX (FIX). This disorder affects approximately 1 in 30,000 male individuals worldwide. Patients with severe form (FIX <1%) account for 30%–45% of hemophilia B cases and usually report of spontaneous bleeds. Treatment includes FIX replacement prophylactically to prevent complications. However, the patient may develop inhibitors against FIX, which is rare and a serious complication, reported to occur in 1.5%–3% of hemophilia B patients. Immune tolerance induction is a therapeutic strategy to eliminate inhibitors. We report a 14-month-old-male child with severe hemophilia B on primary prophylaxis, presented with right knee swelling. He developed anaphylactic reaction while receiving recombinant FIX concentrate. Investigation revealed that FIX inhibitor titers were 1.0 Bethesda Units. He was managed with immune tolerance induction successfully.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"285 - 287"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43147239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"GNE – related severe congenital macrothrombocytopenia: A case report and literature review","authors":"Muhammad Alam, Abdulrhman Alathaibi, M. Kashif, M. Zakaria, Ruwayd A. Attar, H. Al-Ghamdi, Abdullah Al Harbi","doi":"10.4103/joah.joah_44_22","DOIUrl":"https://doi.org/10.4103/joah.joah_44_22","url":null,"abstract":"Congenital thrombocytopenia results from genetic mutations in genes implicated in megakaryocyte differentiation and/or platelet formation and clearance. We report the case of an 11-month-old girl who presented with severe macrothrombocytopenia since birth and subsequently developed an intracranial bleed. She was diagnosed to have GNE gene mutation. GNE gene encodes the key enzyme in sialic acid biosynthesis, glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE/MNK). Its mutation is responsible for the reduction in sialic acid biosynthesis and consequently leads to severe congenital thrombocytopenia and/or myopathy. Although no sign of myopathy is observed in our patient; it is possible myopathy can be developed later, thus long-term follow-up with neurology is highly advisable. We recommend the genetic counseling and a segregation analysis of this variant in other affected individuals in the family.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"280 - 284"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49208704","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unilateral vision loss unmasking underlying acute leukemia","authors":"Pradeep K Panigrahi","doi":"10.4103/joah.joah_161_21","DOIUrl":"https://doi.org/10.4103/joah.joah_161_21","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"288 - 288"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49138377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tumor lysis syndrome in pediatric patients with hematological malignancies","authors":"Lamis Elkhatib, M. Bayoumy, A. Ahmed, Muhammad Alam, I. Abosoudah, H. Altrabolsi","doi":"10.4103/joah.joah_243_20","DOIUrl":"https://doi.org/10.4103/joah.joah_243_20","url":null,"abstract":"BACKGROUND: Tumor lysis syndrome (TLS) is a common complication of hematological malignancies and consists of either hyperkalemia, hyperphosphatemia, hyperuricemia, or hypocalcemia. These metabolic derangements may result in clinical tumor lysis syndrome in the form of acute kidney injury (AKI), arrhythmias, seizures, and sudden death. OBJECTIVES: This study was conducted to determine the incidence and outcome of TLS and to identify local risk factors in children with hematological malignancies. PATIENTS AND METHODS: This was a retrospective chart review of children ≤18 years diagnosed with acute lymphoblastic leukemia (ALL), acute myeloid leukemia, or non-Hodgkin lymphoma between 2014 and 2018. TLS was diagnosed and stratified according to the risk of developing tumor lysis using the Cairo and Bishop definition and Cairo stratification. RESULTS: Among 180 patients, only 11 patients (6%) developed TLS. Four patients had laboratory TLS (LTLS) (36.3%) and six had CLTS (54.5%). The male-to-female ratio was high (2.4:1 in the TLS group). Hyperphosphatemia and hypocalcemia were the most frequently occurring criteria for LTLS (81.8%). The strongest predictors for TLS were hyperuricemia and hypocalcemia at presentation (P < 0.001) followed by diagnosis of T-cell ALL, preceding AKI splenomegaly, high initial white blood cell, and lactate dehydrogenase, with P < 0.05. AKI secondary to tumor lysis occurred in six patients (54.5%), of which five needed dialysis. One patient had seizures secondary to tumor lysis (9.1%) and no patient died from TLS. CONCLUSION: There is a wide variation in reported incidence of TLS from 6% to 45%, likely due to different TLS definitions applied, diverse cohorts and duration. A universal definition and risk-stratified approach to prevent tumor lysis in patients with hematologic malignancies is needed to help in proper comparison between studies.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"118 - 125"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44224651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diverse atypical lymphocytes in the peripheral blood smear of dengue patients: Crystalline rods, mott cells, and downey cells","authors":"S. Acharya, Aparna Ningombam, Abhirup Sarkar, Kundan Kumar","doi":"10.4103/joah.joah_33_21","DOIUrl":"https://doi.org/10.4103/joah.joah_33_21","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"163 - 165"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47390200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of tyrosine kinase inhibitor discontinuation initiative in patients with chronic myeloid leukemia at princess noorah oncology center","authors":"A. Noor, M. Khan, Muteb Al-Thomali, A. Alnatsheh, A. Absi, F. Hakami, Majed A Alshamrani","doi":"10.4103/joah.joah_144_21","DOIUrl":"https://doi.org/10.4103/joah.joah_144_21","url":null,"abstract":"CONTEXT: Tyrosine kinase inhibitors (TKIs) are the standard of care therapy for chronic myeloid leukemia (CML). The current disadvantages of lifelong treatment include adverse effects and financial burden. AIMS: This study aimed to evaluate the outcomes of treatment-free remission (TFR) in CML patients. SETTINGS AND DESIGN: A retrospective cohort study was conducted in our oncology center. SUBJECTS AND METHODS: Patients ≥18 years old diagnosed with CML, received TKI and had a TFR trial to achieve TFR, with at least 6 months follow-up, and who received TKIs were included. Patients with a previous hematopoietic stem cell transplantation were excluded. The primary outcome was the proportion of patients with sustainable TFR at 6 months. The secondary outcomes were the proportion of patients with sustainable TFR at 12 months, the rate of regaining a major molecular response (MMR) after relapse, and the cost-saving impact. Descriptive statistics were used for the demographics and outcomes. RESULTS: A total of 250 patients were screened for eligibility, 25 patients were enrolled. The median age was 54 years (interquartile range: 45.5–59), 60% were female. TFR was 92% at 6 months and 72% at 12 months. 100% of the patients regained MMR. The total number of TFR days were 17,024 which resulted in a direct cost saving of SR 7,205,601.9, based on Saudi FDA pricing. CONCLUSIONS: The study indicated that the majority of the CML patients achieved 6 months TFR. This initiative has resulted in a significant cost saving. Future studies should characterize potential candidates for TKI discontinuation.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"126 - 131"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45083090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}