Hand Castings of Children With Terminal Illnesses From Cancer Can Provide Comfort to Grieving Families.

Registered nurses have voluntarily created hand casts for families, providing comfort during challenging moments. Hand casting moves the patient's family and nurses. As requested by parents, staff apply a quick-drying gel to sick children's hands and feet. After preparing the gel mold, alginate molding powder is poured in and hardened for many days. Parents mourn their children with great sensitivity. Every mold and hospital bedside we go to offers closure to the lost child's dying moments. A compelling benefit of a three-dimensional hand-cast is preserving a passing moment.

Vitiligo and Alopecia Areata After Donor Lymphocyte Infusions in a Child With Relapsed Acute Myeloid Leukemia.

Rarely do patients with chronic graft-versus-host disease (cGVHD) experience vitiligo and alopecia areata. Nevertheless, the exact cause of vitiligo and alopecia areata is still not fully understood. The patient experienced a relapse of acute myeloid leukemia (AML) following a second complete remission after undergoing HLA-6/8 mismatched unrelated donor hematopoietic cell transplantation (HCT). Achieving full donor chimerism was successful during the initial stages of the transplant. Nevertheless, the molecular evidence of measurable residual disease remained, prompting the administration of donor lymphocyte infusions (DLI) following a dose-escalation protocol. After three cycles of DLI given at two-month intervals, the circulating blasts eventually vanished. After the third DLI dose, vitiligo developed despite achieving molecular remission. The dermatologist confirmed the presence of vitiligo and alopecia areata, along with cutaneous cGVHD. The outcome was the complete elimination of the molecular presence, and the patient experienced both clinical and molecular remission for a period of five years following DLI. Based on our observations, it was found that DLI could effectively eradicate molecular leukemia in cases of AML relapse after HCT. The development of vitiligo and alopecia areata was influenced by the destruction of melanocytes due to autoimmune reactions caused by cGVHD.

Early Allogeneic Transplantation Favorably Influences the Outcome of Pediatric Acute Myeloid Leukemia-A Retrospective Study in a Single Center Over 2 Decades.

BACKGROUND: Transplantation advancements offer the potential for improving the prognosis of patients with acute myeloid leukemia (AML). Controversies surrounding indications and timing persist. We focused on identifying prognostic factors and exploring the advantages of early transplantation. PATIENTS AND METHODS: We studied 102 pediatric patients with AML (February 1999-August 2022), using Cox regression to analyze survival and hematopoietic cell transplantation (HCT) outcomes and Kaplan-Meier curves to assess HCT timing's impact on prognosis. RESULTS: "Treatment in First Complete Remission [CR1]: Chemotherapy" showed increased risk in multivariate and univariate Cox regression analyses, whereas "HCT during the study period" displayed divergent outcomes. Focusing on transplanted patients, "Treatment in CR1: Chemotherapy" still correlated with higher mortality risk. These findings emphasize the pivotal role of the treatment strategy adopted in CR1 on overall survival rather than HCT alone. Donor cytomegalovirus (CMV) positivity is also related to reduced mortality risk. Kaplan-Meier analysis supported superior 5-year survival rates with "HCT" compared with "chemotherapy" in CR1. In the 3-arm analysis, "HCT in CR1" demonstrated better 5-year overall survival (OS) and 5-year disease-free survival (DFS) compared with "Never HCT," whereas "HCT in CR2" had the least favorable prognosis (5-year OS: 79.2% vs 57.1% vs 50%, P = .056; 5-year DFS: 73.6% vs 55.2% vs 0%, P = .000). CONCLUSION: Our study highlights the benefits of transplantation during CR1 on prognosis. However, when contemplating CR1 transplantation recommendations, evaluation of various factors, such as the patient's clinical state, relapse risk, transplant-related mortality, CMV status, and other pertinent considerations, is vital. Comprehensive case discussions with patients and families are demanded in optimizing treatment.

Aloe vera gel for prevention of chemotherapy-induced hyperpigmentation: Four case reports.

RATIONALE: This study aimed to evaluate the efficacy of topical application of Aloe vera gel in preventing chemotherapy-induced hyperpigmentation (CIH). CIH is a common side effect of chemotherapy that causes skin irritation, redness, and itching. Aloe vera has been studied for its potential use in treating radiation-induced dermatitis, which may help alleviate some of the symptoms associated with this condition. PATIENT CONCERNS: In this study, 4 children requiring curative chemotherapy were prospectively enrolled and treated with Aloe vera gel. DIAGNOSIS: Acute skin reactions were monitored and classified according to the Common Terminology Criteria for Adverse Events Grading Scale. INTERVENTIONS: Patients were asked to use the gel on one-half of the body field twice daily from the beginning of treatment until 4 weeks after the completion of chemotherapy, with no medication to be used on the other half. OUTCOMES: The results indicate that applying Aloe vera gel may reduce the visibility of hyperpigmentation at subsequent time points. The most important observation was that the continued application of Aloe vera gel 4 weeks after the completion of chemotherapy was effective in reducing the grading of CIH. LESSONS: These effects highlight the potential of Aloe vera gel as a topical onconutraceutical treatment for CIH.

Association of CD34+ Cell Dose with Progression-free Survival after Allogeneic Peripheral Blood Hematopoietic Cell Transplantation in Children with Hematologic Malignancies.

BACKGROUND: A higher CD34+ cell dose is associated with improved engraftment but may also be associated with an increased risk of complications after allogeneic hematopoietic stem cell transplantation, including graft-versus-host disease (GVHD). METHODS: We retrospectively analyze the impact of CD34+ cell dose on OS, PFS, neutrophil engraftment, platelet engraftment, treatment-related mortality, and GVHD grading. RESULTS: For analyses, CD34+ cell dose was stratified into low (< 8.5 × 106/kg) and high (> 8.5 × 106/kg). A subgroup analysis of higher CD34+ cell dose leads to prolonged OS and PFS, but statistical significance was achieved only for PFS (OR 0.36; 95%CI 0.14-0.95; P = 0.04). CONCLUSIONS: This study reinforced that CD34+ cell dose at the time of allo-HSCT retained a positive impact on PFS.

Immersive Virtual Reality to Distract From Pain in Children Treated With L-asparaginase by Intramuscular Injection.

Background Treatment-related pain and discomfort are two of the most common manifestations in children with acute lymphoblastic leukemia (ALL). Patients with ALL are usually treated with L-asparaginase (L-ASP) by intramuscular injection. Children receiving L-ASP chemotherapy must bear adverse reactions such as pain caused by intramuscular injections. The use of virtual reality (VR) distraction technology could be a non-pharmacological intervention to bolster patients' comfort and decrease anxiety and procedure-related pain within hospital settings. Methodology The study explored the potential benefits of VR as a psychological intervention to induce positive emotions and reduce pain levels in participants receiving L-ASP injections. Participants in the study had the opportunity to select a nature theme of their choosing during their treatment session. The study provided a noninvasive solution that promoted relaxation to reduce anxiety by shifting an individual's mood positively during treatment. The objective was met by measuring participants' mood and pain levels before and after the VR experience and participant satisfaction with the use of the technology. This mixed-methods study of children aged six to 18 received L-ASP between April 2021 and March 2022, using a Numerical Rating Scale (NRS) with sheer numbers ranging from 0 (no pain) to 10 (extreme or most pain possible). Semi-structured interviews were conducted to collect new data and explore participants' thoughts and beliefs about a particular topic. A total of 14 patients participated. Descriptive statistics and content analysis are used to describe the data analyzed. VR is an enjoyable distraction intervention for managing treatment-related pain in ALL with intramuscular chemotherapy. Results Eight of 14 patients found a reduction in perceived pain after wearing VR. During the intervention implementation, the primary caregivers felt that the patient's pain perception was more positive when using the virtual reality device, and there was less resistance and less crying. Conclusions This study describes changes and experiences associated with pain and physical discomfort in children with ALL receiving intramuscular chemotherapy. This teaching model is applied to developing medical personnel, providing information about the disease and daily care, and educating the participants' family members. This study may expand the usage of VR applications so that more patients can benefit from them.

Allogeneic stem cell transplantation without preconditioning in a child with therapy-related myelodysplastic syndrome: A case report.

RATIONALE: Infants with mixed-lineage leukemia (MLL)-rearranged leukemia are usually refractory to standard induction therapy and are not immediate candidates for allogeneic hematopoietic stem cell transplantation (allo-HSCT). Chromosome 11q23 translocations, resulting in MLL rearrangement, have been well characterized in infant acute lymphoblastic leukemia (ALL). While t(4;11) ALL continues to have carry a bleak prognosis, patients with therapy-related myelodysplastic syndrome (t-MDS) have a shorter median overall survival than those compared with de novo MDS. PATIENT CONCERNS: We describe a child with t-MDS who evolved from MLL-rearranged ALL and was successfully treated with HSCT without toxic preconditioning. DIAGNOSES: MDS diagnosis was based on morphological characteristics of bone marrow dysplasia in patients with clinical manifestations evidence of hematopoiesis impairments by different combinations of anemia, leukopenia, neutropenia, and thrombocytopenia. INTERVENTIONS: Although the best donor for allo-HSCT is generally considered an human leukocyte antigen-matched sibling, only ~ 30% of patients have a suitable sibling. HSCT from an unrelated donor is a suitable option for patients with t-MDS who do not have matched sibling donors. OUTCOMES: Allo-HSCT without recipient preconditioning could be a promising treatment option for t-MDS, especially for patients with recurrent or persistent infections. LESSONS: Cytogenetics, prognosis, and treatment of t-MDS are briefly discussed. Preconditioning before allo-HSCT seriously damages immune function. This work reviews our experience with a patient with t-MDS following ALL complicated by recurrent infections, and highlights our choice to omit preconditioning from allo-HSCT.

Malignant testicular tumors in children: A single institution's 12-year experience.

Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management. This study aimed to create a holistic picture of the interprofessional team in the management of malignant testicular tumors. Seventeen patients had mixed germ cell tumors, 15 had pure yolk sac tumors, 2 had immature teratomas, 2 had teratocarcinomas, and 1 had a sex cord stromal tumor. Five lesions were diagnosed as nongerm cell tumors: 2 embryonal rhabdomyosarcomas, 2 lymphomas, and 1 acute myeloid leukemia. At initial presentation, retroperitoneal (n = 2), bone marrow (n =1), and mediastinal (n = 1) metastases were identified in 4 (10%) patients. The operative interventions performed included radical inguinal orchiectomy (n = 5), scrotal orchiectomy (n = 31), and testicular biopsy or testis-sparing enucleation of the tumor (n = 6). Postoperatively, 18 patients received either adjuvant chemotherapy (n = 14) or chemoradiation (n = 5). Five patients with mixed germ cell tumors (n = 2), group IV paratesticular rhabdomyosarcoma (n = 2), and acute myeloid leukemia with myeloid sarcoma (n =1) died of disease progression. Thirty-six patients remained alive and disease-free at the last visit. Malignant testicular tumors in children deserve proper diagnostic support from a therapeutic perspective. Any concern or suspicion of a testicular tumor warrants an inguinal approach to avoid scrotal violation.

Molecular genetics of ß-thalassemia: A narrative review.

ABSTRACT: ß-thalassemia is a hereditary hematological disease caused by over 350 mutations in the ß-globin gene (HBB). Identifying the genetic variants affecting fetal hemoglobin (HbF) production combined with the α-globin genotype provides some prediction of disease severity for ß-thalassemia. However, the generation of an additive composite genetic risk score predicts prognosis, and guide management requires a larger panel of genetic modifiers yet to be discovered.Presently, using data from prior clinical trials guides the design of further research and academic studies based on gene augmentation, while fundamental insights into globin switching and new technology developments have inspired the investigation of novel gene therapy approaches.Genetic studies have successfully characterized the causal variants and pathways involved in HbF regulation, providing novel therapeutic targets for HbF reactivation. In addition to these HBB mutation-independent strategies involving HbF synthesis de-repression, the expanding genome editing toolkit provides increased accuracy to HBB mutation-specific strategies encompassing adult hemoglobin restoration for personalized treatment of hemoglobinopathies. Allogeneic hematopoietic stem cell transplantation was, until very recently, the curative option available for patients with transfusion-dependent ß-thalassemia. Gene therapy currently represents a novel therapeutic promise after many years of extensive preclinical research to optimize gene transfer protocols.We summarize the current state of developments in the molecular genetics of ß-thalassemia over the last decade, including the mechanisms associated with ineffective erythropoiesis, which have also provided valid therapeutic targets, some of which have been shown as a proof-of-concept.

Comparable Outcomes Between Adolescent/Young Adults and Children With Acute Myeloid Leukemia Following Allogeneic Hematopoietic Cell Transplantation: A Single-Center Experience.

BACKGROUND: The cytogenetics of acute myeloid leukemia (AML) increases exponentially with age. Adolescent and young adult (AYA) patients have specific psychosocial and other challenges, influencing their ability to access appropriate treatment. Therefore, in allogeneic hematopoietic stem cell transplantation (allo-HSCT) for AML, inferior outcomes would be observed in AYA patients compared to children. METHODS: We defined the age range of AYA patients as 15 to 29 years. Sixty-three patients who underwent allo-HSCT from 1998 to 2020 at Chang Gung Children Hospital were enrolled in this study. Overall survival was the time duration from HSCT to death from any cause. Disease-free survival was the time duration from HSCT to the last follow-up or first event (failure to achieve complete remission, relapse, secondary malignancy, or death from any cause). RESULTS: Thirty-seven (59%) patients were <15 years of age during allo-HSCT, and 26 (41%) were 15 to 29 years of age. The median age during allo-HSCT was 6.3 years for those <15 years of age compared with 15.7 years for AYA patients. The median follow-up period was 2.2 years after hematopoietic stem cell transplantation for patients <15 years old and 3.8 years after hematopoietic stem cell transplantation for AYA patients. Univariate analysis revealed no significant difference in the 5-year overall survival or disease-free survival among all patients. CONCLUSIONS: Several distinct AML subtypes could be amenable to treatment deintensification and targeted therapies. Furthermore, we found that children and AYA patients who underwent allo-HSCT for AML had similar survival.

Health-related quality of life predicts length of hospital stay and survival rates for pediatric patients receiving allogeneic hematopoietic cell transplantation.

PURPOSE: We examined the association between health-related quality of life (HRQoL) of pediatric patients during hospitalization for allogeneic hematopoietic cell transplantation (HCT) and length of hospital stay, and 1-year survival. METHODS: Primary family caregivers were proxy-assessors for the Pediatric Quality of Life (PedsQL) Stem Cell Transplant Module at three time points: 5-days pre-HCT (T0); 14-days post-HCT (engraftment, T1); and 1-week before hospital discharge (T2). Cox regression analyses determined predictors of the overall 1-year survival after allogeneic HCT. RESULTS: Thirty-nine eligible caregivers completed all assessments. The mean age of the pediatric patients was 9.07 years (SD = 5.2). PedsQL Stem Cell Transplant Module scores decreased from 71.33 (SD = 13.26) at T0 to 55.41(SD = 13.05) at T1 (p < 0.001) and increased to 68.46 (SD = 13.97) at T2 (p < 0.001). There was no significant difference between scores at T0 and T2. Longer length of hospital stay was associated with children who were younger and had greater relative changes in scores on the caregiver-proxy PedsQL Stem Cell Transplant Module from T0 to T1. PedsQL Stem Cell Transplant Module scores ≥ 58.07 at T2 were associated with higher 1-year survival rates (Hazard Ratio = 0.12, 95% Confidence Interval = 0.02-0.78; p = 0.03). CONCLUSION: Our findings suggest that assessment of HRQoL during early HCT can add prognostic value beyond demographic and HCT factors. Understanding the HRQoL status during hospitalization for HCT could help identify pediatric patients with low prospects of 1-year survival in order to provide support interventions to improve HRQoL and survival rates.

The effect of tetrastarch solution for capillary leak syndrome following allogeneic hematopoietic stem cell transplantation: A report of 2 cases.

Capillary leak syndrome (CLS) is a severe complication of allogeneic hematopoietic stem cell transplantation (HSCT) characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia. The primary pathogenesis is injury of the capillary endothelium resulting in a loss of intravascular fluid into the interstitial space. Treatment is limited to vascular endothelial growth factor withdrawal and systemic corticosteroids. We report two cases with CLS where weight gain, ascites, and hypotension developed after neutrophil engraftment following allogeneic HSCT. We obtained serial electrolytes, blood urea nitrogen, creatinine, and albumin from these patients. Ultrasound with Doppler tracing performed on both patients showed no reversal of portal venous flow. Issues addressed were the restoration of regular hydration by hydroxyethyl starch (HES) solutions, together with systemic corticosteroids and forced diuresis. Tetrastarch was administered 10 and 20 days, respectively. Both patients recovered without sequelae. CLS is a frequent complication after allogeneic HSCT. The effects of HES on CLS merit further consideration and prospective study.

Cytogenetic aberration in mixed-phenotype acute leukemia in children: A single-center retrospective review.

BACKGROUND: Mixed-phenotype acute leukemia (MPAL) poses a diagnostic and therapeutic dilemma. No consensus exists on the strategy to assign patients with MPAL to either lymphoid- or myeloid-directed treatment. Thus, a better understanding of the characteristics of MPAL is a crucial unmet need. This study aims to provide information on a population-based cohort of children who received treatment based on standard, simple immunophenotypic criteria. METHODS: Single-center, retrospective clinical and laboratory reviews of patients with MPAL were provided by morphology, immunophenotyping, cytogenetics, and molecular methods. We identified 242 flow cytometry samples. Of all consecutive pediatric patients with acute leukemia, we identified 8 (3.3%) patients with MPAL fulfilling WHO 2016 criteria; these were classified as follows: B-lymphoid + myeloid (n = 4), T-lymphoid + myeloid (n = 2), and B + T-lymphoid (n = 2). RESULTS: Of 8 MPAL cases, 4 were boys and 4 girls [median age at diagnosis: 10.8 (range 1.1-17) years]. The b3a2 (p210) and e1a2 (p190) BCR/ABL fusion transcripts were detected in 1 patient with B/myeloid MPAL. Regarding the morphology, all patients were initially diagnosed as acute lymphoblastic leukemia, but no morphological characteristics or cytogenetic aberration was particularly predictive of an MPAL. Furthermore, 4 of 8 patients (50%) with MPAL were associated with chromosome 21 monosomy or partial trisomy. CONCLUSION: Despite no single recurrent chromosomal abnormality that could serve as a hallmark lesion in MPAL, cytogenetic alterations are frequent and predominantly associated with complex karyotype involving chromosome 21 abnormalities.

Exploring the concerns and experiences of parents of children scheduled to receive haematopoietic stem cell transplant.

AIMS: To explore the concerns and experiences of parents of children scheduled to receive allogeneic haematopoietic stem cell transplantation. DESIGN: A qualitative secondary analysis of interview data from an intervention study. METHODS: A total of 28 parents (4 fathers and 24 mothers) were recruited from a children's hospital, which performs approximately one-third of all paediatric haematopoietic stem cell transplantation cases in Taiwan. Data were collected between September 2015-August 2018 by one researcher with face-to-face interviews, which were tape-recorded and transcribed verbatim. The data were analysed using inductive content analysis to extract the main themes. RESULTS: Five themes describing parental concerns prior to paediatric haematopoietic stem cell transplantation were identified: the child became their first priority, seeking solutions, an interweaving of hope and uncertainty, grateful for the chance of a cure and the long road to recovery. CONCLUSION: Understanding the concerns and experiences of our participants as they navigated the pre-transplantation period could reduce parents' uncertainty and improve the care of their child. IMPACT: These experiences provide information that could be used by healthcare professionals to develop support interventions and strategies tailored to the individual needs of each parent, which could prepare parents for their child's haematopoietic stem cell transplantation.

Effectiveness of a Three-Stage Intervention in Reducing Caregiver Distress During Pediatric Hematopoietic Stem Cell Transplantation: A Randomized Controlled Trial.

More than one fourth of primary caregivers report clinically significant distress during the hematopoietic stem cell transplantation (HSCT) process. Providing early support to primary caregivers could reduce caregiver distress and improve the quality of life. This study examined the effects of a three-stage caregiver support intervention designed to reduce caregiver distress and improve quality of life during pediatric HSCT hospitalization. A two-group comparative study was conducted with repeated measures. Participants were randomly assigned to an intervention group or a control group. The intervention group received the support intervention 5 days before the transplant, 14 days after transplant, and 1 week before hospital discharge. The control group received standard support provided in the hospital ward. Measures were obtained at all three time points from self-report questionnaires, which were related to anxiety, depression, perceived stress, and quality of life. Findings indicated that primary caregivers in the intervention group (n = 22) reported significantly lower levels of perceived stress and higher levels of quality of life than the control group (n = 23) at 14 days after transplant. In the intervention group, caregiver distress significantly decreased from pretransplant through 14 days after transplant, while over the same period caregiver quality of life significantly increased. The intervention effectively changed the trend of distress and quality of life for caregivers of children during the process of HSCT and hospitalization. The findings of this study suggest that it is important to provide early targeted interventions at critical junctures for caregivers at risk of adverse outcomes.

Diagnostic Value of C-reactive Protein and Interleukin-8 in Risk Stratification of Febrile Neutropenic Children with Allogeneic Hematopoietic Stem Cell Transplantation.

In this analysis, the levels of CRP and IL-8 were employed as a guide for designing the duration of antibiotics administration in the condition of febrile neutropenia. The importance of laboratory biomarkers is in the early diagnosis of critical illness and adjustment of further management. IL-8 is a useful biomarker for the early identification of critically ill patients, compared to CRP in FN.

Factors associated with cytomegalovirus infection in children undergoing allogeneic hematopoietic stem-cell transplantation.

While preemptive therapy with ganciclovir (GCV) for cytomegalovirus (CMV) infection is used following allogeneic hematopoietic stem-cell transplantation (HSCT), risk factors for CMV infection in children undergoing HSCT are poorly understood.We studied CMV reactivation following allogeneic HSCT by retrospectively analyzing pediatric patients who received allogeneic HSCT and preemptive GCV therapy between 1998 and 2016. The level of viremia requiring preemptive GCV therapy was >1 CMV antigen-positive cells per 5 × 10 leukocytes during the antigenemia assay era and >1000 copies/mL in the polymerase chain reaction era. Among 290 at-risk patients, 54 (18.6%) patients had primary CMV infection or CMV reactivation occurring at a median of 76 days (range, 7-234) following HSCT. CMV reactivation occurred in 28.2% (44/156) of CMV-seropositive transplant recipients at a median of 26 days posttransplant.Univariate and multivariate analyses revealed statistically significant relationships between CMV infection and grade III-IV acute graft-vs-host disease, seronegative donor/seropositive recipient combination, and unrelated/mismatched donors. The remaining demographic factors were not predictive of CMV infection.The seronegative donor/seropositive recipient combination for HSCT was associated with an incomplete response to antiviral therapy. Human leukocyte antigen identical donors were the best choice for patients undergoing allogeneic HSCT to reduce the incidence of CMV disease and mortality.

Small cell variant of ALK-positive anaplastic large cell lymphoma with primary subcutaneous presentation: A case report.

RATIONALE: The rare morphological variant of anaplastic large cell lymphoma (ALCL) may pose a challenge in diagnosis, especially when presentation primarily involves skin lesions. PATIENT CONCERNS: Here we describe a rare case of small cell variant of ALCL in an 11-year-old girl. DIAGNOSIS: We performed clinical, morphological, and immunohistochemical analyses of developed cutaneous nodules. INTERVENTIONS: Pathologists should consider this small cell variant in ALCL differential diagnosis, as early and correct diagnosis has important clinical implications. OUTCOMES: Allogeneic hematopoietic stem cell transplantation appears to be a promising treatment option for small cell variant of ALCL. LESSONS: Histological diagnosis of small cell variant of ALCL is challenging among pediatricians because of its low incidence and atypical presentation. We provide a short review of the small cell variant of ALCL to facilitate the diagnosis of this difficult-to-recognize entity.