ABSTRACT
BACKGROUND: The novel coronavirus disease 2019 has caused psychological distress such as anxiety and fear among nurses worldwide. Nurses are a creditable source of vaccine-related information and have a primary role in building confidence regarding vaccines. AIM: The present study was designed to investigate the fear and anxiety of nurses regarding novel coronavirus disease 2019 (COVID-19) and their attitudes towards the COVID-19 vaccine. The predictors of fear, anxiety and attitudes towards the vaccine were also investigated. METHODS: A descriptive and correlational design was used. The study was conducted in three public hospitals from different parts of the Turkey, and 405 nurses from the three hospitals were recruited by stratified sampling. RESULTS: Anxiety and negative attitudes towards the vaccine were positively related to fear of COVID-19. Age and fear were also positively associated with anxiety related to COVID-19. Nurses who had received the vaccine and those who were fearful had a positive attitude towards the vaccine. CONCLUSION: Psychosocial support programmes should be implemented to improve psychological resilience and coping strategies in nurses and organized education programmes are needed to increase positive attitudes towards vaccination.
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INTRODUCTION: Invasive fungal infections (IFIs) are significant causes of morbidity and mortality in leukemia patients. This study investigated antifungal treatment and prophylaxis features according to leukemia risk groups and treatment phases in pediatric acute lymphoblastic leukemia (ALL) patients who received Berlin-Frankfurt-Munster-based protocols. MATERIALS AND METHODS: We retrospectively examined ALL patients' data between the ages of 1 and 18 and treated them with Berlin-Frankfurt-Munster-ALL protocols between June 2013 and December 2016. RESULTS: A total of 446 febrile neutropenic attacks in 85 children were evaluated. Seventy-two patients received antifungals in 151 infection attacks, while 13 patients did not receive any antifungal treatment during chemotherapy. Empirical, preemptive, or proven treatments were given to 74.8%, 21.2%, and 4% of patients, respectively. The frequency of antifungal therapy increased linearly and significantly from the standard-risk group to the intermediate-risk (IR) group, high-risk (HR) group, and relapsed group. IR patients needed more antifungal therapy while receiving induction, whereas HR patients needed more throughout the induction and HR consolidation blocks than other phases. During induction, IR patients received antifungal therapy similar to HR patients' treatment in the induction and HR consolidation blocks. CONCLUSIONS: Antifungal therapy requirements increased as the severity and intensity of chemotherapy increased for all leukemia risk groups. The requirement of antifungal therapy for IR patients receiving induction was similar to that of HR patients; further studies are needed to evaluate the potential advantages of using primary antifungal prophylaxis in IR patients.
Subject(s)
Invasive Fungal Infections , Leukemia, Myeloid, Acute , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Adolescent , Antifungal Agents/therapeutic use , Child , Child, Preschool , Humans , Infant , Invasive Fungal Infections/drug therapy , Invasive Fungal Infections/etiology , Invasive Fungal Infections/prevention & control , Leukemia, Myeloid, Acute/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Retrospective Studies , Risk FactorsABSTRACT
Primary immune deficiencies are a group of heterogenous genetic disorders characterized by frequent infections, autoimmunity and malignancy. In this study, we aimed to evaluate clinical characteristics, outcomes of children with malignancy developed on background of primary immunodeficiency and compare survival rates of patients between malignant lymphoma with primary immunodeficiency and without immunodeficiency from tertiary oncology center in a developing country. A total 23 patients with primary immunodeficiency and malignancy were evaluated retrospectively. A total of 26 malignancies (first or second) in 23 patients were determined. The median age at the time of the first malignancy was 8 years (ranges 2-18 years) with increased male ratio (M/F:14/9). Non-Hodgkin lymphoma (n = 17; 65%) was the most common malignancy, followed by Hodgkin lymphoma (n = 5), anaplastic ependymoma (n = 1), spinal glioblastoma multiforme (n = 1), retinoblastoma (n = 1) and intracranial hemangiopericytoma (n = 1). The median follow-up time of patients was 25 months (ranges between 1 and 189 months). The 5-year overall survival rate of patients with malignant lymphoma associated with primary immunodeficiency (41%) were lower than immunocompetent patients with malignant lymphoma (80%) (p = 0.000). The 5-year overall survival of patients was diagnosed between 2021 and 2013 years (62%) was higher than previous years (22%) (p = 0.03). In conclusion, non-Hodgkin lymphomas were the most common histopathologic type in patients with malignancy associated with primary immunodeficiency in the present study. The survival of patients with malignant lymphoma associated with primary immunodeficiency has improved in recent years, yet it is still lower than immunocompetent patients with lymphoma and new targeted drugs are required for better survival rates.
Subject(s)
Lymphoma, Non-Hodgkin , Lymphoma , Neoplasms , Adolescent , Child , Child, Preschool , Developing Countries , Humans , Lymphoma/epidemiology , Lymphoma/therapy , Lymphoma, Non-Hodgkin/epidemiology , Lymphoma, Non-Hodgkin/pathology , Lymphoma, Non-Hodgkin/therapy , Male , Neoplasms/epidemiology , Neoplasms/therapy , Retrospective StudiesABSTRACT
OBJECTIVE: Traditional children's games can protect the physical, emotional, and mental health of children. This study was conducted to determine the effect of traditional children's games on internet addiction, social skills, and stress level. METHODS: A pre-test/post-test quasi-experimental design with a control group was used. The population of the study consisted of a fifth- and sixth-grade students studying at two secondary schools in a city of Turkey. A total of 42 students - 20 in the intervention group and 22 in the control group - were included in the study. The Family-Child Internet Addiction Scale, the Social Skills Assessment Scale, the Social Skills Scale, and the Perceived Stress Scale in Children (8-11 years) were used to collect the data. The intervention group received eight weeks of traditional children's games. RESULTS: There was no significant difference between the intervention and control groups in terms of internet use, social skills, and stress level in the pre-test (p > 0.05). There was a statistically significant difference between groups in terms of daily and weekly internet use in the post-test (p < 0.05). The mean scores regarding social skills of the intervention group increased after the games compared with the control group (p < 0.05). There was no statistically significant difference between the groups in terms of stress-level mean scores (p > 0.05). CONCLUSIONS: The results showed that traditional children's games may be effective in reducing internet use and increasing social skills, however they do not affect stress level. Childhood is important for physical, cognitive, and psychosocial development. Therefore, in the school settings, traditional children's games can be used for the protection and promotion of children's health.
Subject(s)
Internet Addiction Disorder , Social Skills , Child , Family , Humans , Internet , Schools , Students/psychologyABSTRACT
BACKGROUND: The importance of health-related quality of life (HRQoL) in patients with acute lymphoblastic leukemia (ALL) has increased in recent years. This study aimed to assess HRQoL in children with ALL, affecting factors, and the relationship between parent proxy-report and child self-report HRQoL. MATERIALS AND METHODS: A total of 59 children and their parents (both mother and father) were enrolled in this cross-sectional study. Turkish version of the Pediatric Quality of Life Inventory (PedsQL) 3.0 Cancer Modules were used to determine HRQoL. RESULTS: According to subscales of the self-report form, nausea and operational anxiety scores differed significantly by the treatment status; communication score varied considerably by the hospitalization length of stay; pain and hurt, cognitive problems, and perceived physical appearance scores differed significantly by the maternal chronic disease status (P<0.05). The presence of maternal chronic disease was significantly related to the total score of the parent-proxy report (mother) (P<0.05). There was a moderate correlation between total scores of child and mother (P<0.05, r=0.419) but not with the father. CONCLUSION: Children on-treatment had significant problems in nausea and procedural anxiety subscales; however, children who were hospitalized more had fewer issues in the communication subscale. Also, children whose mother had chronic disease had poorer HRQoL regarding pain and hurt cognitive problems and treatment anxiety. Given the importance of assessment and monitoring HRQoL in children with ALL, health professionals should be aware of how parents' chronic disease affects HRQoL. Psychosocial support should be provided to children and their parents, especially for those whose parents have a chronic illness.
Subject(s)
Health Status , Leukemia/psychology , Parents/psychology , Quality of Life , Self Report , Adolescent , Adult , Child , Child, Preschool , Cross-Sectional Studies , Female , Follow-Up Studies , Humans , Leukemia/rehabilitation , Male , Middle Aged , Prognosis , Surveys and Questionnaires , Young AdultABSTRACT
In patients with hemophilia, the usual treatment for both iliopsoas hematoma and associated femoral nerve compression is conservative. The case presented herein is a moderate hemophilia A patient with significant femoral neuropathy due to iliopsoas hematoma whose symptoms could not be managed with an aggressive factor VIII replacement program for >2 weeks. An unusual treatment option - percutaneous catheter insertion and aspiration of the hematoma - alleviated the examination findings and reversed his abilities.
Subject(s)
Drainage/methods , Hematoma/therapy , Hemophilia A/complications , Adolescent , Factor VIII/genetics , Hematoma/etiology , Hematoma/pathology , Humans , Male , PrognosisABSTRACT
Endocrine system dysfunctions are the significant complications of excessive iron overload in beta thalassemia patients. The aim of this study was to evaluate the long-term effect of chelation with deferasirox on endocrine complications. The study group consisted of children with beta thalassemia who had been evaluated for the growth and pubertal development, bone metabolism, thyroid/parathyroid functions, glucose metabolism dysfunctions in the department of pediatric hematology of Ankara Diskapi Child Health and Diseases Hematology Oncology Training And Research Hospital between 2009-2011 and reevaluated after deferasirox chelation therapy in 2018. Thirty-one transfusion dependent beta-thalassemia patients were enrolled for the study. Seventeen (54.8%) patients were male and the mean age was 16.9 ± 3.8 (9-23) years. Splenectomy was performed in 11 patients (35.5%). In the initial evaluation, 26 patients (84%) received deferoxamine and/or deferiprone and five (17%) patients received deferasirox as a chelator; in the final evaluation all patients were receiving deferasirox. The mean duration of deferasirox treatment was 5.9 ± 2.02 years (1-10 years). Of the 26 patients who had endocrine complications between 2009-2011, 18 were recovered. In the final evaluation, eight patients (25%) developed new endocrinopathies. The frequency of endocrine complications seen before the deferasirox treatment (83%) was higher than the frequency of complications while receiving deferasirox treatment (25.8%) (p < 0,05). In this study, it was determined that both existing endocrine abnormalities were reduced and recent developed problems were less likely with long-term deferasirox treatment in thalassemia patients.
Subject(s)
Deferasirox , Splenectomy , beta-Thalassemia , Adolescent , Adult , Child , Deferasirox/administration & dosage , Deferasirox/adverse effects , Endocrine System Diseases/blood , Endocrine System Diseases/chemically induced , Endocrine System Diseases/epidemiology , Female , Follow-Up Studies , Humans , Male , beta-Thalassemia/blood , beta-Thalassemia/epidemiology , beta-Thalassemia/therapyABSTRACT
Burkitt lymphoma manifesting as an intracardiac mass is a rare entity. This report describes the case of a 10-year-old boy who presented with an intracardiac mass and tumor thrombosis in the anterior mediastinum that proved to be Burkitt lymphoma. The LMB-96 chemotherapy protocol was given and at the end of the treatment there was still residual mass. A biopsy was performed and the pathology revealed thymus tissue. The patient has been in complete remission for 3 months. Burkitt lymphoma has a short doubling time and an intracardiac lesion can become life threatening. Early recognition and prompt treatment are crucial in achieving optimal outcomes.
Subject(s)
Burkitt Lymphoma/diagnosis , Heart Neoplasms/pathology , Mediastinum/pathology , Thrombosis/etiology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Child , Heart Neoplasms/diagnosis , Humans , Male , Neoplasms/complications , Remission Induction , Thymus Gland/pathologyABSTRACT
Acquired aplastic anemia (AAA) is a rare and potentially life threatening disorder. We retrospectively compared the outcomes of 29 children with AAA who received immunosuppressive therapy (IST) or underwent hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 9.0 years (range, 2-18 years) and median follow-up period was 36 months (range, 3-108 months). Viral infection associated/post hepatitis AAA was in 6 patients (20.6%). According to the initial laboratory findings, 8 patients were classified as very severe AA (vSAA), 8 as severe AA (SAA), and 13 patients as transfusion-dependent moderate AA (MAA). Out of 13, 5 transfusion-dependent MAA patients progressed SAA in median one month (range, 1-5 months), another 6 MAA patients developed remission or became transfusion free during follow-up. Eight patients underwent upfront matched family donor (MFD) HSCT at median 6 months (range, 1-9 months) and achieved complete response (100%). Fifteen cycles of IST were given to 10 (34%) patients lacking MFD at median 3 months (range, 2-6 months). Fifty percent of patients had complete/partial response after IST protocol. Three patients who were unresponsive to IST, proceeded to alternative donor HSCT, in 2nd or 3rd year after the diagnosis and only 1 patient was sustained remission. Several drugs such as mycophenolatemofetil, high-dose cyclophosphamide, levamisole and eltrombopag have been investigated in order to improve the outcome of patients with AAA. Early intervention in AAA patients results in significantly better outcomes.
Subject(s)
Anemia, Aplastic/therapy , Benzoates/administration & dosage , Cyclophosphamide/administration & dosage , Hematopoietic Stem Cell Transplantation , Hydrazines/administration & dosage , Immunosuppression Therapy , Levamisole/administration & dosage , Pyrazoles/administration & dosage , Adolescent , Allografts , Anemia, Aplastic/epidemiology , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Male , Retrospective StudiesABSTRACT
Massive splenic infarction and portal vein thrombosis (PVT) due to chronic myeloid leukemia (CML) is extremely rare. We describe 2 children who were presented with massive splenic infarction and PVT in the course of CML. Massive splenic infarction and PVT treated with splenectomy in one and with medical treatment in another in whom PVT resolved by cytoreductive treatment, led to downsizing of spleen or splenectomy. Splenic infarct and PVT should be considered in CML patients with long-lasting severe abdominal pain despite appropriate medical attempts. Splenectomy should be spared for persistent symptoms and complications.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/complications , Portal Vein , Splenic Infarction/etiology , Thrombosis/etiology , Antineoplastic Agents/therapeutic use , Child , Female , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Male , Remission Induction , Severity of Illness Index , Splenectomy , Splenic Infarction/surgery , Thrombosis/surgeryABSTRACT
Introduction: Port catheters facilitate the administration of chemotherapy, antibiotics, blood products, fluid, and parenteral nutrition to pediatric patients with hematological malignancies. However, as its use has become widespread, local and systemic, catheter-related infections have emerged as important causes of morbidity and mortality. In our study, we aimed to evaluate the success of antibiotic lock therapy in port catheter-related infections of pediatric patients followed up with acute leukemia. Methods: Port catheter cultures taken from a total of 182 pediatric patients with acute lymphoblastic/myeloblastic leukemia who were followed up at Ankara City Hospital Pediatric Hematology Clinic between August 2019 and August 2023 were evaluated retrospectively. Results: Bacterial growth was identified in 739 port catheter culture specimens of 182 patients. Closure or removal of the port was required in 91, and removal of the port catheters in 49 patients due to port catheter-related infections. Antibiotic lock therapy was started in 56 patients with bacterial growth in the port catheter. With antibiotic lock therapy, port catheter-related infections of 42 patients were eradicated, and their catheters began to be used again. As a result, the port catheter-related infections of 42 of 56 (75%) patients whose ports were closed and also received systemic antibiotic therapy were eradicated, and no infection recurrence was observed. Conclusion: Adding antibiotic lock therapy to systemic antibiotics in pediatric patients may be beneficial in terms of catheter salvage.
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BACKGROUND: Microbiologic confirmation of respiratory tract infections gained importance during the coronavirus disease 2019 (COVID-19) pandemic. This study retrospectively evaluated seasonal distribution, clinical presentation, and complications of respiratory viral infections (RVIs) other than COVID-19 in children with cancer during and after the pandemic lockdown. METHODS: Two hundred and sixty-five inpatient and outpatient RVI episodes in 219 pediatric cancer patients confirmed by multiplex reverse transcriptase polymerase chain reaction (RT-PCR) panels from 13 centers were enrolled. RESULTS: Eighty-six (32.5%) of the total 265 episodes occurred in 16 months corresponding to the lockdowns in Türkiye, and the remaining 67.5% in 10 months thereafter. Human rhinovirus/enterovirus (hRE) (48.3%) was the most common agent detected during and after lockdown. Parainfluenza virus (PIV) (23.0%), influenza virus (9.8%), and respiratory syncytial virus (RSV) (9.1%) were the other common agents. The 28.7% of episodes were lower respiratory tract infections (LRTIs), and complications and mortality were higher than upper respiratory tract infections (URTIs) (25.0% vs 5.3%). Bacteremia was identified in 11.5% of culture-drawn episodes. Treatment delay in one-third and death within four weeks after RVI in 4.9% of episodes were observed. CONCLUSION: During the pandemic, fewer episodes of RVIs occurred during the lockdown period. Respiratory viruses may cause complications, delays in treatment, and even death in children with cancer. Therefore, increased awareness of RVIs and rapid detection of respiratory viruses will benefit the prevention and, in some cases, abrupt supportive and some antiviral treatment of RVI in children with cancer.
Subject(s)
COVID-19 , Neoplasms , Respiratory Tract Infections , Humans , COVID-19/epidemiology , COVID-19/complications , Respiratory Tract Infections/epidemiology , Respiratory Tract Infections/virology , Child , Male , Female , Retrospective Studies , Child, Preschool , Infant , Turkey/epidemiology , Adolescent , SARS-CoV-2 , Pandemics , SeasonsABSTRACT
OBJECTIVE: Beta thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is a feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle. The aim of our study is to evaluate the association between vitamin D deï¬ciency and deformation of the left ventricular myocardium measured by speckle-tracking echocardiography in children with thalassemia major. METHODS: In this prospective study, 33 thalassemic patients with vitamin D deï¬ciency were enrolled. Cardiac magnetic resonance T2* value, conventional echocardiography, and speckle tracking, and also left ventricular longitudinal and circumferential strain values were measured. Myocardial functions of the patients with vitamin D deï¬ciency or insuï¬ciency were evaluated by speckle-tracking echocardiography before and after vitamin D replacement. RESULTS: The mean age of the patients was 15.4 ± 3.09 years. Vitamin D level was deï¬cient in 30 (90%) and insuï¬cient in 3 (10%) of them. Speckle-tracking analysis showed a signiï¬cantly decreased absolute value of the left ventricular global longitudinal strain before vitamin D replacement. A signiï¬cant improvement in the global longitudinal strain was detected after vitamin D replacement (P < 0.05). A statistically signiï¬cant increase was observed in parameters showing left ventricular systolic and diastolic functions after vitamin D replacement. CONCLUSION: Vitamin D deï¬ciency is frequently observed and causes decreased contractility in thalassemic patients. In our study, we observed that our patients' cardiac functions had improved after vitamin D replacement therapy.
Subject(s)
Ventricular Dysfunction, Left , Vitamin D Deficiency , beta-Thalassemia , Humans , Child , Adolescent , beta-Thalassemia/complications , beta-Thalassemia/pathology , Vitamin D , Prospective Studies , Echocardiography/methods , Myocardium/pathology , Ventricular Function, LeftSubject(s)
Anti-Infective Agents/therapeutic use , Cytoreduction Surgical Procedures , Leukostasis/drug therapy , Lung Diseases/drug therapy , Child , Cytarabine/therapeutic use , Humans , Leukostasis/pathology , Leukostasis/surgery , Lung Diseases/pathology , Lung Diseases/surgery , Male , PrognosisABSTRACT
OBJECTIVE: Anthracyclines are widely used in the treatment of acute lymphoblastic leukemia (ALL). However, cardiotoxicity is the most critical side effect that requires dose limitation. It is thought to occur at first exposure, but the clinical presentation may occur years later. In this study, we aimed to determine the time of initial damage and cardiotoxicity that develops in children with ALL. METHODS: In this prospective study, 13 patients with newly diagnosed intermediate-risk precursor B cell ALL treated with the ALL-IC BFM 2009 protocol were included. Conventional echocardiography, tissue Doppler imaging (TDI), and speckle-tracking echocardiography (STE) were performed in all the patients before chemotherapy, after completing the induction phase, and at the end of the reinduction phase. RESULTS: The mean age of the patients was 7.8±4.6 (3.1-16.3) years. Myocardial velocity during systole (Sm) determined by TDI at the interventricular septum significantly decreased during the induction phase. Despite a decrease in STE parameters, a statistically significant reduction was determined in the global longitudinal strain rate at both left and right ventricles at the end of the induction. Nevertheless, a statistically significant increase was observed among the conventional echocardiographic findings in the left ventricular end-diastolic diameter at the end of the reinduction. CONCLUSION: During the treatment of ALL, subclinical anthracycline-associated cardiotoxicity develops in the early stages of treatment. The findings detected by TDI and STE could be missed by conventional echocardiography. We recommend evaluating patients with these newly developed techniques to detect subclinical cardiotoxicity at an early stage and starting appropriate therapy on time.
Subject(s)
Precursor Cell Lymphoblastic Leukemia-Lymphoma , Ventricular Dysfunction, Left , Anthracyclines/adverse effects , Cardiotoxicity/diagnostic imaging , Child , Child, Preschool , Echocardiography , Humans , Precursor Cell Lymphoblastic Leukemia-Lymphoma/chemically induced , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prospective Studies , Ventricular Dysfunction, Left/chemically inducedABSTRACT
BACKGROUND: Pyruvate kinase (PK) deficiency is the most common enzyme abnormality in the glycolytic pathway. Here, we describe two siblings with PK deficiency that mimicked congenital dyserythropoietic anemia (CDA) type I. CASE: The siblings were referred to our hospital for evaluation of anemia when they were newborns. Their PK enzyme activities were normal. Their bone marrow aspirations and electron microscopies showed CDA-like findings. A CDA panel with next-generation sequencing showed no mutation. Though their PK enzyme levels were normal, a molecular study of the PKLR gene showed a homozygous variant c.1623G > C (p.Lys541Asn) in exon 12 of our patients. CONCLUSIONS: Although the diagnosis of pyruvate kinase deficiency is difficult, it can be confused with many other diagnoses. Bone marrow findings of these cases are similar to congenital dyserythropoietic anemia. In patients with normal pyruvate kinase enzyme levels, the diagnosis cannot be excluded and genetic analysis is required.
Subject(s)
Anemia, Dyserythropoietic, Congenital , Anemia, Hemolytic, Congenital Nonspherocytic , Pyruvate Metabolism, Inborn Errors , Humans , Infant, Newborn , Pyruvate Kinase/genetics , Anemia, Dyserythropoietic, Congenital/diagnosis , Anemia, Dyserythropoietic, Congenital/genetics , Pyruvate Metabolism, Inborn Errors/diagnosis , Pyruvate Metabolism, Inborn Errors/genetics , Anemia, Hemolytic, Congenital Nonspherocytic/diagnosis , Anemia, Hemolytic, Congenital Nonspherocytic/geneticsABSTRACT
There have been several studies that have shown that patients with beta thalassemia major are at a higher risk of thrombosis due to the procoagulant activity of thalassemic erythrocytes, decreased liver synthetic function, increased platelet activity and vascular endothelial activation attributed to chronic oxidative stress, although there are no established tests to predict thrombotic risk in TM patients. In this study, we evaluated whether or not the platelet function analyser (PFA-200) and thrombin generation test (TGT) would be useful tools to identify hypercoagulability and risk of thrombosis in thalassemia major patients. The study included 50 patients with thalassemia major and 104 healthy control group. Pretransfusion and posttransfusion PFA-200 and TGT results were compared with control group. We found that median C/ADP and C/EPI values in the thalassemia major group were greater in both the pre and posttransfusion samples than the C/ADP and C/EPI results from the control group. The TGT results showed there was no difference between control group and the results from the thalassemia major group. The TGT and PFA-200 testing did not identify hypercoagulability nor identify clear testing parameters to predict a thalassemia major patient's risk of thrombosis. There may be other mechanisms/causes yet unidentified that could better explain thalassemia major patient's increased risk from thromboembolic events.
Subject(s)
Thrombophilia , Thrombosis , beta-Thalassemia , Adenosine Diphosphate , Blood Coagulation , Humans , Thrombophilia/complications , Thrombosis/etiology , beta-Thalassemia/complications , beta-Thalassemia/therapyABSTRACT
Langerhans cell histiocytosis is a rare hematologic disorder and patients who fail first-line treatment have a poor prognosis, and require more intensive treatment. We present an infant diagnosed with multisystem Langerhans cell histiocytosis refractory to multimodal therapy who was successfully treated with cyclosporine. Cyclosporine might be an effective alternative drug as nonmyelosuppressive rescue therapy for multiple relapsed-refractory Langerhans cell histiocytosis that has not achieved remission with cladribine and cytarabine therapy.
Subject(s)
Cyclosporine/therapeutic use , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/pathology , Immunosuppressive Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclosporine/administration & dosage , Cyclosporine/adverse effects , Drug Resistance, Neoplasm , Histiocytosis, Langerhans-Cell/etiology , Histocytochemistry , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Infant , Male , Radiography, Thoracic , Recurrence , Retreatment , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
OBJECTIVE: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. MATERIALS AND METHODS: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. RESULTS: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x109/L, 5.4x109/L, and 58.3x109/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30±17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x109/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. CONCLUSION: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.