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Background@#Transfusions in pediatrics need to be performed carefully because of various variables, such as the blood volume and immature immune system. As a result, adverse transfusion reactions may appear differently from adults. This study examined the frequency and types of adverse transfusion reactions in pediatric patients. @*Methods@#From January 2018 to December 2021, this study was conducted on 58 children who requested red blood cells, platelets, and plasma blood components from Chungbuk National University Hospital. The frequency and types of adverse transfusion reactions were analyzed retrospectively by reviewing blood transfusion-related medical records and compared with previous studies. @*Results@#Approximately 0.9% of total blood components were transfused into pediatric patients; 1,179 units of blood components were transfused. The number of transfusions for red blood cells, platelets, and plasma was 383, 712, and 84 units, respectively. Among 58 patients, 23 adverse transfusion reactions were observed in 15 (25.9%) patients. Of these, 18 were febrile nonhemolytic transfusion reactions, and five were allergic transfusion reactions. Febrile nonhemolytic transfusion reactions occurred in 66.7% of cases with red blood cells, and allergic transfusion reactions occurred with platelets in 60% of cases. @*Conclusion@#This paper reported the incidence and types of adverse transfusion reactions in pediatric patients. This is expected to be more frequent in pediatric patients than adults, but most of them were relieved by supportive treatment because the symptoms were mild. As the awareness of hemovigilance is still low, it is essential to recognize and deal with adverse transfusion reactions through continuous education.
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Although the prevalence of hereditary hemolytic anemia (HHA) is relatively low in Korea, it has been gradually increasing in recent decades due to increment in the proportions of hemoglobinopathies from immigrants of South East Asia, raising awareness of the disease among clinicians, and advances in diagnostic technology. As such, the red blood cell (RBC) Disorder Working Party (WP), previously called HHA WP, of the Korean Society of Hematology (KSH) developed the Korean Standard Operating Procedures (SOPs) for the diagnosis of HHA in 2007. These SOPs have been continuously revised and updated following advances in diagnostic technology [e.g., flow cytometric osmotic fragility test (FOFT) and eosin-5-maleimide (EMA) binding test], current methods for membrane protein or enzyme analysis [e.g., liquid chromatography-tandem mass spectrometry (LC-MS/MS), ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS), high-performance liquid chromatography (HPLC)], and molecular genetic tests using next-generation sequencing (NGS). However, the diagnosis and treatment of HHA remain challenging as they require considerable experience and understanding of the disease. Therefore, in this new Korean Clinical Practice Guidelines for the Diagnosis of HHA, on behalf of the RBC Disorder WP of KSH, updated guidelines to approach patients suspected of HHA are summarized. NGS is proposed to perform prior to membrane protein or enzyme analysis by LC-MS/MS, UPLC-MS/MS or HPLC techniques due to the availability of gene testing in more laboratories in Korea. We hope that this guideline will be helpful for clinicians in making diagnostic decisions for patients with HHA in Korea.
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Background@#Blood transfusion is frequently performed as a supportive therapy during the diagnosis and chemotherapy of acute myeloid leukemia (AML). This study examined the frequency of blood transfusion and analyzed the correlation with the treatment response during induction therapy in patients with AML. @*Methods@#From January 2018 to December 2020, blood transfusion information was collected from 23 patients diagnosed with AML during induction therapy. The frequency and volumes of blood transfusions according to the treatment response were collected and analyzed with the overall survival retrospectively. @*Results@#The blood transfusion was performed in all patients with AML during induction therapy. The transfusion frequency and volumes were a median of five (1∼13) times and nine (2∼27) units for red blood cells, respectively.In the platelets, the median frequency was seven (2∼21) times, and the transfusion volumes were 42 (12∼128) units. At the time of the treatment response evaluation, the transfusion dependence was 0% in morphological complete remission and 20% in the morphological leukemic-free state for both RBC and platelets, and 78% for RBC and 67% for platelets in treatment failure. Although not statistically significant, transfusion independence for more than eight weeks after induction therapy showed a better overall survival (P=0.312). @*Conclusion@#When the treatment response was good, the dependence on blood transfusion decreased. The transfusion frequency is expected to help predict the patient's treatment response and prognosis along with the peripheral blood counts.
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Purpose@#To summarize the results of chromosomal microarray analysis (CMA) for copy number variants (CNVs) detection and clinical utility in a single tertiary hospital. @*Materials and Methods@#We performed CMA in 46 patients over the course of two years. Detected CNVs were classified into five categories according to the American College of Medical Genetics and Genomics guidelines and correlated with clinical manifestations. @*Results@#A total of 31 CNVs were detected in 19 patients, with a median CNV number per patient of two CNVs. Among these, 16 CNVs were classified as pathogenic (n=3) or likely pathogenic (LP) (n=11) or variant of uncertain significance (n=4). The 16p11.2 deletion and 16p13.11 deletion classified as LP were most often detected in 6.5% (3/46), retrospectively. CMA diagnostic yield was 24.3% (9/37 patients) for symptomatic patients. The CNVs results of the commercial newborn screening test using next generation sequencing platforms showed high concordance with CMA results. @*Conclusion@#CMA seems useful as a first-tier test for developmental delay with or without congenital anomalies. However, the classification and interpretation of CMA still remained a challenge. Further research is needed for evidence-based interpretation.
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Background@#Blood transfusion is frequently performed as a supportive therapy during the diagnosis and chemotherapy of acute myeloid leukemia (AML). This study examined the frequency of blood transfusion and analyzed the correlation with the treatment response during induction therapy in patients with AML. @*Methods@#From January 2018 to December 2020, blood transfusion information was collected from 23 patients diagnosed with AML during induction therapy. The frequency and volumes of blood transfusions according to the treatment response were collected and analyzed with the overall survival retrospectively. @*Results@#The blood transfusion was performed in all patients with AML during induction therapy. The transfusion frequency and volumes were a median of five (1∼13) times and nine (2∼27) units for red blood cells, respectively.In the platelets, the median frequency was seven (2∼21) times, and the transfusion volumes were 42 (12∼128) units. At the time of the treatment response evaluation, the transfusion dependence was 0% in morphological complete remission and 20% in the morphological leukemic-free state for both RBC and platelets, and 78% for RBC and 67% for platelets in treatment failure. Although not statistically significant, transfusion independence for more than eight weeks after induction therapy showed a better overall survival (P=0.312). @*Conclusion@#When the treatment response was good, the dependence on blood transfusion decreased. The transfusion frequency is expected to help predict the patient's treatment response and prognosis along with the peripheral blood counts.
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Purpose@#To summarize the results of chromosomal microarray analysis (CMA) for copy number variants (CNVs) detection and clinical utility in a single tertiary hospital. @*Materials and Methods@#We performed CMA in 46 patients over the course of two years. Detected CNVs were classified into five categories according to the American College of Medical Genetics and Genomics guidelines and correlated with clinical manifestations. @*Results@#A total of 31 CNVs were detected in 19 patients, with a median CNV number per patient of two CNVs. Among these, 16 CNVs were classified as pathogenic (n=3) or likely pathogenic (LP) (n=11) or variant of uncertain significance (n=4). The 16p11.2 deletion and 16p13.11 deletion classified as LP were most often detected in 6.5% (3/46), retrospectively. CMA diagnostic yield was 24.3% (9/37 patients) for symptomatic patients. The CNVs results of the commercial newborn screening test using next generation sequencing platforms showed high concordance with CMA results. @*Conclusion@#CMA seems useful as a first-tier test for developmental delay with or without congenital anomalies. However, the classification and interpretation of CMA still remained a challenge. Further research is needed for evidence-based interpretation.
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Background/Aims@#Myelodysplastic syndrome (MDS) is caused by genetic and epigenetic alteration of hematopoietic precursors and immune dysregulation. Approximately 20% of patients with MDS develop an autoimmune disease (AID). Here, we investigated whether particular genetic mutations are associated with AID in patients with MDS. @*Methods@#Eighty-eight genetic mutations associated with myeloid malignancy were sequenced in 73 MDS patients. The association between these mutations and AID was then analyzed. @*Results@#The median age of the 73 MDS patients was 70 years (interquartile range, 56 to 75), and 49 (67.1%) were male. AID was observed in 16 of 73 patients (21.9%). Mutations were detected in 57 (78.1%) patients. The percentage (68.8% vs. 80.7%, p = 0.32) and the mean number of mutations (1.8 ± 1.6 vs. 2.2 ± 1.8, p = 0.34) in MDS patients with or without AID were similar. However, the ten-eleven translocation- 2 (TET2) mutation rate was significantly higher in patients with AID than in those without (31.3% vs. 5.3%, respectively; p = 0.001). All TET2 mutations were variants of strong clinical significance. @*Conclusions@#Mutation of TET2 in patients with MDS may be associated with increased risk of developing AID.
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BACKGROUND: In platelets transfusion, alloimmunization against the HLA and HPA antigen present in the white blood cells/platelets of the donor blood occurred. In addition, unexpected red blood cell alloantibodies might be produced by the alloimmunization of red blood cells antigens in the transfused platelet component. Therefore, this study examined the incidence of red blood cell alloantibodies after platelet transfusion. METHODS: From January to December 2018, adult patients who requested platelet concentrates or single donor platelets were enrolled. The results of pre/post-transfusion test, including antibody screening test and antibody identification test, were collected the incidence of red blood cell alloantibody formation was then analyzed, retrospectively. RESULTS: A total of 685 patients received 11,894 units of platelet concentrates and 1,402 units of single donor platelets. The median patient age was 64 years and the number of blood transfusions was 4.1. The amount of transfusion per session was 7.3 units, and the total transfused platelet concentrates was 30.9 units. New red blood cell alloantibodies were detected in 0.9% of all patients, and the identification results were observed as unidentified non-specific antibody in 66.7% and anti-E antibodies in 33.3%. The incidence of alloantibody was proportional to the frequency and amount of platelet transfusion. CONCLUSION: This paper reported the incidence of red blood cell alloantibody after platelet transfusion for the first time in Korea. Although matched platelet concentrates supply may be not practical in terms of cost-effectiveness, it may be useful to recognize the possibility of red blood cell alloimmunization and expand the understanding of extended matching transfusion.
Subject(s)
Adult , Humans , Antibodies , Blood Platelets , Blood Transfusion , Erythrocytes , Incidence , Isoantibodies , Korea , Mass Screening , Platelet Transfusion , Retrospective Studies , Tissue DonorsABSTRACT
We describe a cutaneous abscess caused by Nocardia abscessus in a previously healthy woman. A 74-year-old woman presented with recurrent bullae on her left forearm that developed 1 week prior and was initially suspected to be a cutaneous infection with Mycobacteria or Tinea corporis. Histopathologically, the skin lesion formed an abscess. A smear revealed a few branched Gram-positive filamentous microorganisms that formed a creamy white colony on a blood agar plate after incubation for 3 days. The colony tested negative on acid-fast bacilli (AFB) staining, but was positive on modified AFB staining. The isolate was confirmed to be N. abscessus by 16S rRNA sequencing analysis. The isolate was susceptible to trimethoprim-sulfamethoxazole, amikacin, cefotaxime and erythromycin but resistant to penicillin. The patient was treated with clarithromycin but subsequently lost to follow-up. To the best of our knowledge, this is the first report of a human cutaneous infection with N. abscessus in Korea.
Subject(s)
Aged , Female , Humans , Abscess , Agar , Amikacin , Cefotaxime , Clarithromycin , Erythromycin , Forearm , Korea , Lost to Follow-Up , Nocardia , Penicillins , Skin , Tinea , Trimethoprim, Sulfamethoxazole Drug CombinationABSTRACT
BACKGROUND: Telomere shortening is thought to be involved in the pathophysiology of myeloid malignancies, but telomere lengths (TL) during interphase and metaphase in hematopoietic malignancies have not been analyzed. We aimed to assess the TLs of interphase and metaphase cells of MDS and telomerase activity (TA) and to find out prognostic significances of TL and TA. METHODS: The prognostic significance of TA by quantitative PCR and TL by quantitative fluorescence in situ hybridization (QFISH) of interphase nuclei and metaphase chromosome arms of bone marrow cells from patients with MDS were evaluated. RESULTS: MDS patients had shorter interphase TL than normal healthy donors (P<0.001). Average interphase and metaphase TL were inversely correlated (P=0.013, p arm; P=0.029, q arm), but there was no statistically significant correlation between TA and TL (P=0.258). The progression free survival was significantly shorter in patients with high TA, but the overall survival was not different according to average TA or interphase TL groups. Multivariable Cox analysis showed that old age, higher International Prognostic Scoring System (IPSS) subtypes, transformation to AML, no history of hematopoietic stem cell transplantation and short average interphase TL (<433 TL) as independent prognostic factors for poorer survival (P=0.003, 0.001, 0.005, 0.005, and 0.013, respectively). CONCLUSIONS: The lack of correlation between age and TL, TA, and TL, and the inverse relationship between TL and TA in MDS patients reflect the dysregulation of telomere status and proliferation. As a prognostic marker for leukemia progression, TA may be considered, and since interphase TL has the advantage of automated measurement by QFISH, it may be used as a prognostic marker for survival in MDS.