Journal of Applied Hematology最新文献

{"title":"Burden of acquired thrombotic thrombocytopenic purpura: KSA and UAE expert consensus for improved disease management","authors":"Hasan AAL-Yaseen, Amna Al Mehairi, Mohammed Aldarweesh, M. Damlaj, K. El Tayeb, S. Hussain, H. Osman, Abdulkareem M Almomen, M. Marashi","doi":"10.4103/joah.joah_149_21","DOIUrl":"https://doi.org/10.4103/joah.joah_149_21","url":null,"abstract":"BACKGROUND: In Saudi Arabia (KSA) and the United Arab Emirates (UAE), only limited epidemiological data and treatment guidance exist for acquired thrombotic thrombocytopenic purpura (aTTP), a rare, life-threatening blood disorder. AIMS: Expert insights from KSA and UAE were used to obtain local epidemiological data, to characterize current disease management and unmet needs, and to formulate recommendations for the improvement of the diagnosis and treatment of aTTP. Costs and socioeconomic burden were a secondary focus. METHODS: Hematologists from KSA and UAE with clinical experience in the diagnosis and management of aTTP individually answered questions on the burden and management of aTTP via an online survey. Based on these insights, a draft consensus was discussed and refined jointly by the hematologists in a live session for each country. Payers provided information on the economic burden and cost of aTTP management. RESULTS: The experts estimate the incidence of aTTP to 5–6 (KSA) and 1–2 (UAE) per 1,000,000 person-years and anticipate it increasing. Most of the presenting patients are aTTP-naive. Recurrent disease is rare. Diagnosis of aTTP should involve ADAMTS13 activity testing. Plasma exchange and immunosuppression are the current standard of care. Key unmet needs include a lack of awareness of aTTP, access to rapid testing and novel treatments to improve outcomes. CONCLUSION: The expert consensus to address the unmet needs and improve aTTP outcomes include increasing aTTP awareness and access to ADAMTS13 testing; the development of national guidelines; and, additionally, strategies to improve patients' long-term quality of life.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"145 - 153"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49428632","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":"Approaches to acquired thrombotic thrombocytopenic purpura management in Saudi Arabia","authors":"A. Alhejazi, A. Albeihany, Hani Alhashmi, Hazza A Alzahrani, I. Motabi, I. El-Hemaidi, K. Alsaleh, K. El Tayeb, Magdy Rabea, Mohamed M. Khallaf, M. Qari","doi":"10.4103/joah.joah_46_21","DOIUrl":"https://doi.org/10.4103/joah.joah_46_21","url":null,"abstract":"Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening microangiopathy usually characterized by microangiopathic hemolytic anemic, thrombocytopenia, and end-organ ischemia associated with disseminated microvascular platelet-rich thrombi and severe deficiency (activity <10%) of A Disintegrin-like And Metalloprotease with ThromboSpondin Type 1 Motif No. 13 (ADAMTS13). It is a medical emergency, and if left untreated, acute mortality is as high as 90%. This review article is a narrative review based on available literature. In addition, the key discussions of the Kingdom of Saudi Arabia experts and members of “Approaches to aTTP Management” Advisory Board meeting held on October 16, 2020, have been incorporated as expert opinions. It was agreed that treatment should be started based on the presumptive diagnosis and continued until remission or an alternate diagnosis is established. Use of caplacizumab in addition to therapeutic plasma exchange and immunosuppression is recommended in confirmed aTTP episodes.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"111 - 117"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42808316","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 sensitivity and specificity of high fluorescence lymphocyte count percentage of sysmex XN analyzer in diagnosis of dengue","authors":"Aparna Ningombam, S. Acharya, Abhirup Sarkar, Kundan Kumar, M. Brijwal, Srimantinee Routray, J. Sachdev, D. Srigyan, Aashish Choudhary, A. Subramanian, L. Dar","doi":"10.4103/joah.joah_204_20","DOIUrl":"https://doi.org/10.4103/joah.joah_204_20","url":null,"abstract":"BACKGROUND: The Sysmex XN-series hematology analyzers provide newer parameters including high fluorescence lymphocyte cell percentage (HFLC%) which correlates with the presence of atypical lymphocytes in peripheral blood. We aimed to analyze the sensitivity and specificity of HFLC% as a diagnostic tool and its association with serological status in diagnosed dengue patients and thereby establish a cutoff of HFLC% based on serology. Besides, we also wish to correlate HFLC% with thrombocytopenia in these patients. MATERIALS AND METHODS: A total of 1500 serum samples were subjected to serological evaluation for dengue. After excluding hematological malignancies and autoimmune disorders, the same day complete blood count parameters including HFLC% and platelet counts were collected retrospectively for 292 serologically positive dengue cases and 76 seronegative controls. RESULTS: Our result shows that in nonstructural 1 antigen-positive cases, a cutoff of >5.2% HFLC can have a sensitivity of 79.5% and specificity of 98.6%. We found a different cut off of HFLC% >3.2% (sensitivity 83.4%, specificity 98.6%) for the cases with only immunoglobulin M positivity and a cut off of HFLC% >2.6% (sensitivity 86.1%, specificity 96%) in the dual positive cases (immunoglobulin M with nonstructural 1 antigen). Besides, high HFLC% also shows a strong correlation with platelet count with a Spearman correlation coefficient of −0.6. CONCLUSIONS: The result of our study shows that a specific cutoff of HFLC% can not only help us to suspect dengue fever but also predict the risk of thrombocytopenia in already diagnosed dengue patients. The sensitivity and specificity of HFLC% varied with the serological status of the patients which depend on the days of fever on presentation.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"132 - 138"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48143059","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":"Cold agglutinin disease in COVID-19 causing severe intravascular hemolysis","authors":"S. Priyadarshini, Sreelakshmi Pasupulati","doi":"10.4103/joah.joah_75_21","DOIUrl":"https://doi.org/10.4103/joah.joah_75_21","url":null,"abstract":"Cold agglutinin disease (CAD) is a distinct type of acquired immune hemolytic anemia. It can be idiopathic (primary) or secondary to infections, neoplasms and autoimmune diseases. Mycoplasma pneumonia and EBV are the infections commonly associated with secondary CAD. In the current COVID-19 pandemic, there are very few case reports showing an association between CAD and COVID-19.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"154 - 156"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49127010","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":"A neglected tropical disease (Microfilariasis) in an immunocompromised adult","authors":"Malvika Gaur, Tushar Sehgal, A. Subramanian","doi":"10.4103/joah.joah_104_21","DOIUrl":"https://doi.org/10.4103/joah.joah_104_21","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"162 - 162"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43600506","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 experience from a single institute in the United Arab Emirates","authors":"Najam Ahmed Awan, Layla Mohamed Alreyami, Asia Al Mulla, Majed M. Alremeithi, M. Khanani","doi":"10.4103/joah.joah_24_22","DOIUrl":"https://doi.org/10.4103/joah.joah_24_22","url":null,"abstract":"BACKGROUND: Immune tolerance induction (ITI) is the gold standard approach for eradicating inhibitors and increasing patient tolerance to factor VIII. The success rate of ITI may vary depending on patient variables and factors relating to the pattern of treatment for the induction of immune tolerance. Children with recently diagnosed inhibitors are the best candidates for ITI, and those with favourable expected results should be offered ITI as soon as inhibitors are identified. Recombinant factor VIII Fc fusion protein has proved a therapeutic advantage in patients with high factor VIII inhibitor titers AIMS AND OBJECTIVES: To evaluate the clinical characteristics and outcomes of ten hemophilic pediatric patients who underwent ITI therapies to eliminate FVIII inhibitors at Tawam Hospital, UAE MATERIALS AND METHODS: The data of ten hemophilia A children aged 2–7 years with high inhibitor titers who underwent ITI therapy at Tawam Hospital, UAE, were retrospectively collected for this case series. A comparison of bleeds before and after the ITI therapy was also made. Patients with either failed or partially successful primary ITI therapy underwent rescue ITI therapy. Data analysis was performed using SPSS version 26. RESULTS: Full success was achieved in 60% (6/10) of the patients, 10% (1/10) achieved partial success, whereas 30% (3/10) failed the primary ITI therapy. The rescue ITI therapy was successful in 50% (2/4) of the patients and the remaining 50% (2/4) achieved partial success [Table 2]. The rescue ITI was successful in 66% (2/3) of those patients who received Elocta and partially successful in 33% (1/3) CONCLUSION: ITI therapy is the gold standard for the eradication of antibodies against FVIII. The patients with good expected outcomes should be offered ITI as soon as the inhibitors are confirmed. The use of extended half life rFVIIIFc demonstrated therapeutic benefit, particularly in challenging ITI patients with high inhibitor titers","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"91 - 94"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43234406","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":"Imatinib-induced cardiotoxicity: A study to evaluate cardiac functions in patients on imatinib therapy for chronic myeloid leukemia","authors":"P. Chakravarthy, P. Kumari, D. Gupta, M. Bhowmick, M. Naik, Gudimetla Priyanka","doi":"10.4103/joah.joah_9_21","DOIUrl":"https://doi.org/10.4103/joah.joah_9_21","url":null,"abstract":"INTRODUCTION: The success of tyrosine kinase inhibitors in the treatment of chronic myeloid leukemia (CML) has increased the focus on survivorship and late toxicity in oncological care. Cardiovascular (CV) health has emerged as an important consideration in patients of CML. Some studies have shown an increased incidence of CHF with Imatinib therapy. This study was conducted to assess the cardiac functions in patients of CML on Imatinib therapy. METHODS: Seventy newly diagnosed CML patients started on Imatinib therapy having normal baseline cardiac functions as assessed by two-dimensional echocardiography and multigated acquisition (MUGA) scan were included in the study. Further assessment of cardiac functions at 3, 6, and 12 months was done by MUGA scan. RESULTS: At 3, 6, and 12 months of follow-up, 7.14%, 10%, and 11.43% of patients, respectively, showed evidence of cardiotoxicity in the form of reduced ejection fraction and wall abnormalities on MUGA scan (P = 0.58, 0.013, and 0.006, respectively). CONCLUSION: Cardiac dysfunction was seen in 11.43% of patients on Imatinib therapy for CML, though none of them were symptomatic. CML patients on Imatinib therapy must be monitored for cardiac functions, especially if they have other CV risk factors.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"71 - 75"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41416206","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":"Platelet morphology masquerading as trypanosomiasis","authors":"Abhirup Sarkar, S. Acharya, Aparna Ningombam, Kundan Kumar","doi":"10.4103/joah.joah_212_20","DOIUrl":"https://doi.org/10.4103/joah.joah_212_20","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"107 - 108"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49084210","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":"Role of interleukin-10 (1082G/A) and splicing factor 3B subunit 1 (2098A/G) gene polymorphisms in chronic lymphocytic leukemia","authors":"M. Gamaleldin, Maya Moussa, Salma Alaa Eldin Imbaby","doi":"10.4103/joah.joah_93_21","DOIUrl":"https://doi.org/10.4103/joah.joah_93_21","url":null,"abstract":"OBJECTIVE: Interleukin-10 (IL-10) gene polymorphisms might play a part in the development of some malignant tumors. It has been linked with high bcl-2 expression in some B-lymphocyte malignancies. Its relationship with chronic lymphocytic leukemia (CLL) development is still under investigation. Other studies have linked Splicing Factor 3B Subunit 1 (SF3B1) mutations to a poorer prognosis of CLL. From this context, we have great interest to investigate the effect of both IL-10 (1082G/A) and SF3B1 (2098A/G) gene polymorphisms on CLL in this study. MATERIALS AND METHODS: Peripheral blood mononuclear cells were analyzed for IL-10 (1082G/A) and SF3B1 (2098A/G) gene polymorphisms by real-time quantitative polymerase chain reaction in 80 newly diagnosed CLL patients and 80 controls. RESULTS: Our results showed that the IL-10 (G/A) genotype, IL-10 (A/A) genotype and IL-10 A allele and SF3B1 (A/G) genotype and SF3B1 G allele were increased significantly in the patients group compared with the control group. CONCLUSION: IL-10 gene polymorphisms (1082 G/A and A/A) and A alleles might be associated with increased risk of CLL development compared with G/G genotypes and G alleles and are a probable risk factor for the disease. Also, our study demonstrated that SF3B1 (2098A/G) polymorphisms and G allele are related to and might be a causative factor for CLL.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"76 - 83"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44678001","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":"A retrospective study of risk factors of stroke or pathological transcranial doppler ultrasonography in Children with Sickle Cell Disease in Jeddah, Saudi Arabia","authors":"I. al-harbi, A. Algiraigri, S. Khaldi, Rahf Albogmi, Salha Asiri, Wed M. Alogaibi","doi":"10.4103/joah.joah_72_21","DOIUrl":"https://doi.org/10.4103/joah.joah_72_21","url":null,"abstract":"INTRODUCTION: Sickle cell disease (SCD) is an autosomal recessive disorder characterized by hemolytic anemia with abnormal red blood cells. Stroke is a devastating complication of SCD. In Saudi Arabia, there is a high incidence of children with SCD who have had stroke. No studies have been conducted to look at the risk factors. In this review, we aim to explore these risk factors. METHODOLOGY: In this retrospective cohort, individuals between 0 and 18 years old, diagnosed with SCD and had either a stroke, or pathological transcranial Doppler ultrasonography (TCD) result (cerebral blood velocity ≥200 cm/second or repeated borderline result >170 cm/second) were included. 38 cases were assessed. The parents were interviewed regarding patients personal, social, and family history. Medical and laboratory data were obtained from medical records. RESULTS: Females were more affected (20/38). Most children are of an average socioeconomic status (20/38 compared to only 12/38 with poor socioeconomic status). Parental education inversely correlated with the risk of stroke/pathological TCD. The most common presentation of stroke was weakness (9/38), followed by headache (5/38). History of a prior stroke was found to be significant at 15.8%. The Cooperative Study of SCD found that leukocytosis is associated with an increased risk of stroke. In contrary, our review showed an average white blood cell count of 13.01 (+/‒5.36), HbS concentration at the onset of stroke, and/or pathological TCD of 66.23% (+/‒20.11). The most common artery involved was the middle cerebral artery. CONCLUSION: Stroke is very prevalent in children with SCD in Saudi Arabia. A striking finding was that parental education inversely correlated with the risk; therefore, improving the awareness level of SCD among parents is crucial. Wide implementation of TCD as a screening tool and improving compliance with TCD may lead to reduce the risk. We encourage Saudi researchers to focus on central nervous system complications in children with SCD.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"84 - 90"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45093742","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}