{"title":"Spontaneous resolution of hemophagocytic lymphohistiocytosis in a child infected with epstein–Barr virus","authors":"Rita Alfattal, Hussain Sadeq, Abdullah Ali","doi":"10.4103/joah.joah_109_22","DOIUrl":"https://doi.org/10.4103/joah.joah_109_22","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70811072","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 rare form of acute myeloid leukemia","authors":"Nicolas Athanase","doi":"10.4103/joah.joah_90_22","DOIUrl":"https://doi.org/10.4103/joah.joah_90_22","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70812218","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":"Mono-dysplasia score based on automated cell counter (Sysmex) – A novel parameter for differentiating reactive monocytosis from hematological malignancies","authors":"Sabina Langer, Priyanka Moule, Nitin Gupta, Jyoti Kotwal","doi":"10.4103/joah.joah_54_23","DOIUrl":"https://doi.org/10.4103/joah.joah_54_23","url":null,"abstract":"INTRODUCTION: In India, there is a high burden of infections such as tuberculosis, dengue, and malaria which are common causes of monocytosis. This increases the workload of smear examination by the pathologist. “Mono-dysplasia score” is obtained with a simple complete blood count on an automated cell counter and includes the parameters neutrophil (Ne) and monocyte (Mo) count and Ne-WX which is a Ne dispersion parameter. It is operator independent, objective, and does not require a high level of expertise.AIMS: The aims of the study were to assess the utility of Monoscore/mono-dysplasia score calculated using research parameters of Sysmex XN automated cell counter, as a screening tool for differentiating reactive monocytosis from hematological malignancies associated with monocytosis.MATERIALS AND METHODS: Samples sent in EDTA vacutainer for routine hemogram fulling the criteria for monocytosis (WHO criteria – absolute monocyte count ≥1 × 109/L and accounting for ≥10% of the total white blood cell count) were included in the study. Monoscore was calculated using the formula established by Schillinger et al. Flow cytometry, bone marrow examination, etc., were done as and when needed as standard-of-care tests to establish a final diagnosis.RESULTS: One thousand two hundred and fifty-seven samples were analyzed out of which 41 samples were chronic myelomonocytic leukemia and 126 were other hematological malignancies (HD) including acute leukemias, myelodysplastic syndrome, myeloproliferative neoplasm, etc. Using receiver operating characteristics curves, we established the cutoff 0.212 which showed a sensitivity of 97.6% and specificity of 96.4% to differentiated reactive monocytosis form HD.CONCLUSIONS: A sample showing monocytosis and Monoscore <0.212 and without any other flags can be safely auto-authorized without peripheral blood smears examination, reducing the burden of slides to be reviewed.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"281 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135754371","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 serum cystatin-C and netrin-1 as predictive biomarkers for renal injury in children with beta-thalassemia major","authors":"AmeerJ Shwayel, AbdulkareemM Jewad, MohammedQ Abdulsattar","doi":"10.4103/joah.joah_29_23","DOIUrl":"https://doi.org/10.4103/joah.joah_29_23","url":null,"abstract":"BACKGROUND: Despite optimal transfusion and chelation therapy, beta-thalassemia patients still commonly experience various complications, including renal complications, which may affect their survival. This case–control study involved pediatric patients with beta-thalassemia major (β-TM).MATERIALS AND METHODS: This case–control study involved pediatric patients with β-TM, a total of 60 patient samples were randomly collected from the Genetic Hematology Center at the Dhi-Qar Health Directorate, with an age range of 1–14 years. In addition, 60 samples were collected from healthy children in the same age range as the control group. In this study, the glomerular injury was predicted by measuring the concentration of serum cystatin-C (Cys-C), and the tubular injury was predicted by measuring the concentration of serum netrin-1 (NTN-1). Routine markers, such as hemoglobin (Hb) level, ferritin, serum albumin, and creatinine, were also examined for the patients and the control group.RESULTS: The results showed a statistically significant increase in serum Cys-C in the patient group compared to the control group (P < 0.001) and a significant increase of serum NTN-1 in β-TM patients compared to the control group (P < 0.001). In addition, the study showed a statistically significant decrease in Hb, serum albumin, and creatinine and a significant increase in serum ferritin in the patients group compared to the control group. In conclusion, from the results of this study, we can conclude that the serum Cys-C and NTN-1 measurements can be used as predictive biomarkers for glomerular and tubular injury and increase with age.CONCLUSION: Predicting glomerular and tubular injury using the measurement of serum Cys-C and NTN-1 has improved significantly and advanced with age.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"33 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135755088","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}
RamadanA Mahmoud, HasnaaA Abo-Elwafa, LailaM Youseff, MahmoudI Elbadry, Ahmed Tawfeek, ShereenP Aziz
{"title":"Venous thromboembolism risk assessment among beta-thalassemia patients","authors":"RamadanA Mahmoud, HasnaaA Abo-Elwafa, LailaM Youseff, MahmoudI Elbadry, Ahmed Tawfeek, ShereenP Aziz","doi":"10.4103/joah.joah_31_23","DOIUrl":"https://doi.org/10.4103/joah.joah_31_23","url":null,"abstract":"BACKGROUND: Thromboembolic (TE) disorders are among the most common complications of beta-thalassemia. We designed this cross-sectional study to investigate the state of hypercoagulability and platelet activation in patients with beta-thalassemia.PATIENTS AND METHODS: Seventy-five patients diagnosed with beta-thalassemia by hemoglobin electrophoresis were divided into three groups of 25 patients each: Group I (thalassemia trait), Group II (thalassemia intermedia [TI]), and Group III (thalassemia major). In addition, 50 healthy individuals were included as controls. Both patients and control groups were subjected to clinical and laboratory assessment, which included measurement of protein C, anti-thrombin III, D-dimer, CD41, CD42, CD61, and CD62P, and detection of beta-chain mutations.RESULTS: Levels of the platelet activation marker CD62P were significantly higher in beta-thalassemia patients (26.28 ± 18.01%) than in controls (4.78 ± 2.27%) (P < 0.001). The D-dimer level was significantly higher in beta-thalassemia patients (348.41 ± 571.01 ng/mL) than in controls (71.6 ± 39.61 ng/mL) (P < 0.001). Protein-C and AT-III levels were significantly lower in beta-thalassemia patients (71.45 ± 13.26%, 78.38 ± 15.32%) in comparison with controls (94.9 ± 13.03%, 96.52 ± 11.01%) (P < 0.001 and P < 0.001, respectively). TE disorders were found in 7/25 (9%) beta-thalassemia patients, especially in older and postsplenectomy patients. TE was most commonly found in beta-TI. Beta-chain mutations were found in all patients with TE disorders, especially compound heterozygous intervening sequence (IVS) (IVS1.6 [T > C]/IVS1.110 [G > A]).CONCLUSION: Postsplenectomy teenagers and adults with beta-thalassemia with lower levels of natural anticoagulant in the blood, an increased level of D-dimer, and platelet activator factor had a significantly higher risk for TE than those with childhood beta-thalassemia and the control group. In comparison with other beta-thalassemia patients, TI with beta-chain mutations is more likely to develop TE.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135750305","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":"Bilateral chilaiditi syndrome in a child with sickle cell trait","authors":"Muneer H Albagshi, Fatimah A Alaithan","doi":"10.4103/joah.joah_54_22","DOIUrl":"https://doi.org/10.4103/joah.joah_54_22","url":null,"abstract":"","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"289 - 290"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48810630","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}
R. El-Gamal, M. Ismail, Inas Mohamed, Mervat Alfeky
{"title":"Individualized antigen expression in precursor T-cell acute lymphoblastic leukemia: A gate to minimal residual disease analysis by flow cytometry","authors":"R. El-Gamal, M. Ismail, Inas Mohamed, Mervat Alfeky","doi":"10.4103/joah.joah_128_21","DOIUrl":"https://doi.org/10.4103/joah.joah_128_21","url":null,"abstract":"BACKGROUND: In T-acute lymphoblastic leukemia (T-ALL), multi-parametric flow cytometry can serve to detect minimal residual disease (MRD) by using immature or aberrant antigens expression as well as the altered expression of T-cell antigens. The latter approach has been specifically introduced to overcome the absence of leukemia-associated antigens. However, there is no agreed-upon method for the use of T-cell antigens in T-ALL MRD testing. AIMS AND OBJECTIVES: To compare the expression of classic T-cell antigens on T-lymphoblasts and T-lymphocytes to establish a protocol for their use in MRD analysis. MATERIALS AND METHODS: Flow cytometric data of PB or BM samples from 63 adults with T-ALL were collected. We assessed the frequency and degree of brightness or dimness of each T-cell marker, in addition to studying the uniformity of the events scatter of a total of 287 follow-up BM samples from 50 patients. RESULTS: Significant differences in expression intensity of T-cell markers were found between T-lymphoblasts and T-lymphocytes; they were reasonably stable on blasts in follow up samples. This detailed study has nominated the conjoint use sCD3neg/dim and CD5dim/neg in the identification of residual cells, to be supported by other T-cell markers. CONCLUSION: The suggested gating sequence showed an acceptable level of accuracy in detecting residual leukemia, supporting their use in T-ALL MRD especially when other distinguishing markers might be absent in the diagnosis sample, or susceptible to be lost with induction therapy.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"268 - 276"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44385318","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}
Ibrahim A AlFawaz, A. Alhejaili, K. Siddiqui, M. Ayas
{"title":"Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis","authors":"Ibrahim A AlFawaz, A. Alhejaili, K. Siddiqui, M. Ayas","doi":"10.4103/joah.joah_223_20","DOIUrl":"https://doi.org/10.4103/joah.joah_223_20","url":null,"abstract":"BACKGROUND: Langerhans cell histiocytosis is a rare disorder leading to destruction of tissues as well as causing lesions in the body. LCH can be self-limiting to disseminated life threatening. OBJECTIVES: We wanted to review data on presentation and outcome of our treatment efforts on patients with LCH diagnosed and treated at our institution. MATERIAL AND METHODS: In this retrospective study involving 75 pediatric patients with LCH diagnosed between 1998 and 2018, we evaluated clinicopathological features, laboratory findings, treatment modalities and outcome. RESULTS: In males median age at the time of diagnosis was 2.4 years. The median follow up was 63+/- 7.9 months. The most common presenting symptom was a bone lesion-related symptom. 57.3% were above 2 years of age at presentation while 38.7% below 2 years of age. 53% of cases had multisystem disease at diagnosis. Liver found to be most common organ involved. Treatment consisted of observation, surgery or chemotherapy. Vinblastine with steroid was the most common used chemotherapy regimen. 3 patients with progressive refractory disease underwent stem cell transplantation. CONCLUSION: Overall survival was significantly lower in patients with liver involvement.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"249 - 254"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44074569","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}
M. Marashi, A. Basha, A. Lal, H. Osman, Hasan Ghazal, S. Hashmi, M. Khanani
{"title":"Management of acquired hemophilia: Consensus recommendations for the United Arab Emirates practitioners","authors":"M. Marashi, A. Basha, A. Lal, H. Osman, Hasan Ghazal, S. Hashmi, M. Khanani","doi":"10.4103/joah.joah_25_22","DOIUrl":"https://doi.org/10.4103/joah.joah_25_22","url":null,"abstract":"Acquired hemophilia A (AHA) is a rare autoimmune condition characterized by spontaneous antibodies formation against coagulation factor VIII (FVIII). In the United Arab Emirates (UAE), the treatment paradigm of AHA varies, leading to heterogeneous patient outcomes. Because AHA cases often present to clinical practitioners with insufficient experience in the disease, diagnosis is sometimes delayed. This document aims to provide recommendations to clinical practitioners in the UAE to bridge the gap of heterogeneous practice in the clinical management of AHA. Structured literature searches in PubMed and Google Scholar with a three-stage Delphi method were conducted. Evidence suggests that prolonged activated partial thromboplastin time before surgery or coupled with acute or recent onset of bleeding is a hallmark of AHA. Treatment using bypassing agents is recommended in AHA patients with bleeding. Corticosteroids and rituximab with (out) a cytotoxic agent represent the first-line treatment in patients with FVIII: C <1 IU/dL. Second-line therapy involves agents not utilized in the first round of treatment. Thromboprophylaxis is recommended if FVIII: C has returned to normal levels. These recommendations are intended to improve the clinical practice and awareness of this disorder among hematologists and nonhematologists in the UAE and provide practical diagnosis and treatment advice.","PeriodicalId":36501,"journal":{"name":"Journal of Applied Hematology","volume":"13 1","pages":"176 - 182"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46519782","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}