Journal of Pediatric Hematology/Oncology最新文献

Purpose: Central nervous system (CNS) tumors include benign and malignant tumors of the brain and spinal cord. Nutrition risk for children with CNS tumors varies widely based on the diagnosis and treatment plan. Research supports proactive interventions for high-risk patients. However, assessment and intervention strategies vary widely between institutions, and the absence of nutrition management guidelines is a barrier to providing optimal care to these patients. Our group endeavored to survey experienced pediatric oncology dietitians throughout the United States regarding nutrition management practices and then measure agreement with practice recommendations for nutrition care.

Methods: Thirteen dietitians were recruited to participate in a virtual conference concerning nutrition practices in pediatric CNS patients. Two lead dietitians performed thematic analysis of the discussions and developed 16 practice recommendations from the panel. Participants were then invited to provide feedback on the practice recommendations before rating their level of agreement on a final version of the practice recommendations.

Results: Strong agreement (greater than 80% voting agree or strongly agree) was reached on 16/16 of the practice recommendations.

Conclusion: Appropriate nutrition care is gaining attention as an essential element of pediatric cancer therapy. These survey results can guide nutrition care for children with CNS tumors.

Background: Wilms tumor (WT) is the most common pediatric renal tumor. While relapse of WT occurs in up to 15%, its management is not well characterized. We aimed to describe the Canadian experience in relapsed WT and the potential effect of surgical intervention as part of multimodal therapy on survival.

Methods: We identified a cohort of patients with relapsed WT between 2001 and 2020 from the Cancer in Young People in Canada program (CYP-C), a population-based surveillance program. Four-year event-free survival (EFS) and overall survival (OS) were estimated using the Kaplan-Meier method. The effect of surgical intervention on 4-year EFS/OS was evaluated through a Cox proportional hazards model (which included the number of upfront chemotherapy drugs).

Results: Ninety-seven patients were identified. Median age at relapse was 5.2 years (range: 0.4 to 16.0) and median time to relapse from initial diagnosis was 13.0 months (range: 3.0 to 73.0). 28.9% of relapses occurred after initial upfront treatment with 2 chemotherapy drugs versus 62.9% with ≥3 drugs. Four-year EFS and OS from time of first relapse were 54.0% (95% CI: 44.6-65.4) and 61.6% (95% CI: 52.4-72.6), respectively. Seventy-one percent of patients with relapsed WT had surgery as part of multimodal therapy. The hazard ratio associated with surgical intervention in multiple regression was 0.54 for EFS (95% CI: 0.29-1.02; P =.06) and 0.36 for OS (95% CI: 0.19-0.71; P =.003).

Conclusions: Outcomes compare favorably to those reported by international cooperative groups. More favorable survival was observed in patients amenable to surgical intervention at relapse. This association may reflect a true therapeutic benefit of resection or may be confounded by the extent of disease at presentation, response to chemotherapy, the patient's performance status, or other variables. The impact of these on outcomes should be further investigated.

Post-transplant lymphoproliferative disorder is a rare and serious complication of organ and stem cell transplant secondary to immunosuppressive therapies, most commonly of monomorphic B-cell subtype. Here we describe the first reported case of a pediatric heart transplant patient who developed both monomorphic B-cell and nondestructive PTLD with plasmacytic hyperplasia followed by an unrelated case of monomorphic T-cell and nondestructive PTLD with plasmacytic hyperplasia, which later relapsed. We detail the patient's risk factors for development of PTLD and her successful treatment regimens. This case highlights the potential relationship between Epstein-Barr virus and/or cytomegalovirus and PTLD.

Hematologic abnormalities in children with Down Syndrome (DS) have previously been described, but with inconsistent conclusions. A retrospective single-institution cohort study was conducted to evaluate complete blood count (CBC) ranges in children with DS. Mean values for CBC results are presented by age and sex, as a clinical resource for the evaluation of DS children. In comparison to reference values, children with DS had higher mean corpuscular volume (MCV), lower absolute lymphocyte count, and a trend toward higher hemoglobin (Hgb) values. The presence of these findings across the cohort suggests that these are benign hematologic variations in DS.

Objectives: Although thromboembolic events are less common in children compared with adults, complications related to thromboembolism are becoming more frequent due to improved care for children with chronic illnesses. The aim of the study was to evaluate thromboembolic events in pediatric patients hospitalized in palliative care unit (PCU).

Materials and methods: The files of the patients who were hospitalized in PCU of our hospital between January 2020 and January 2023 were retrospectively analyzed. A total of 1002 hospitalizations of 449 patients were examined.

Results: The median age was 6.1 years, F/M: 214/235. We found that 83 (18.4%) patients had thrombosis. In most patients with thrombosis, the primary diseases were malignancy and trauma. During hospitalization, the median time for detecting thrombosis was 26 days (range: 1 to 180 d). We treated 67 out of 83 thrombosis patients with low-molecular-weight heparin (LMWH) for an average of 26 days, ranging from 1 to 188 days. Unfractionated heparin was used in 2 patients, while thrombolytic therapy was used in 2 patients with massive pulmonary embolism and middle cerebral artery thrombosis. None of the thromboembolic events resulted in fatality.

Conclusions: This study revealed that the frequency of thrombosis in pediatric patients hospitalized in PCU was ∼1/5, which is significantly higher than the rates observed in childhood. Catheter-related thrombosis was more frequently observed among patients with thrombosis in the PCU, with malignancy and trauma representing the most common indications for hospitalization. Our findings suggest that certain patient groups should be evaluated for prophylactic anticoagulation.

Levofloxacin is the first line antibiotic used to prevent infection in pediatric oncology patients with prolonged and profound neutropenia. These patients often receive vincristine, a critical component of therapy for leukemia and for many solid tumors. Both levofloxacin and vincristine are known to cause peripheral neuropathy (PN); however, the cumulative effect of both drugs on PN remains unknown. The objective of this study was to evaluate the incidence and extent of PN in pediatric oncology patients receiving vincristine and levofloxacin (VL) compared with vincristine alone (VA). In this retrospective cohort study, manual chart review was performed to extract demographic and clinical data for newly diagnosed patients treated at our institution between 2015 and 2020. Patients who received vincristine alone (VA) were compared with patients who received vincristine and levofloxacin (VL). There was no significant difference in the incidence of peripheral neuropathy between the 2 groups; however, the cohort exposed to vincristine and levofloxacin (VL) experienced significantly more peripheral neuropathy symptoms, had more affected categories of peripheral neuropathy, and received additional treatment for their neuropathy symptoms. Exposure to levofloxacin in addition to vincristine therapy was associated with an increased burden of peripheral neuropathy in this pediatric oncology cohort.

Background: Pediatric cancer survivors remain at risk for numerous late effects of therapy and have shown signs of advanced aging. The specific mechanisms at play are unclear but epigenetic modulation may play a role.

Methods: This cross-sectional study included 20 pediatric cancer survivors who received intensive chemotherapy with or without radiation and were a minimum of 5 years from treatment and 20 healthy biological siblings. DNA methylation patterns were analyzed from peripheral blood samples to determine epigenetic age (the difference between biological age and chronological age). Paired t test analysis or the Wilcoxon signed-rank test was used to compare results between survivors and siblings.

Results: The childhood cancer survivor cohort consisted of 12 males and 8 females; the comparative sibling cohort consisted of 8 males and 12 females. Mean chronological age was 15.2±6.28 years for survivors and 16.4±8.31 years for siblings (mean±SD). Survivors demonstrated increased epigenetic age compared with siblings (1.38±3.71 vs. -0.03±3.12 y (mean±SD), mean difference 1.41, [0.30 to 2.52], P=0.016, n=20 pairs). Patients who additionally received photon radiation demonstrated a more notable increase in epigenetic age compared with siblings (3.33±4.13 vs. 0.78±3.04 y (mean±SD), mean difference 2.55, [0.75 to 4.35], P=0.012, n=8 pairs).

Conclusions: Childhood cancer survivors demonstrate increased epigenetic age compared with their healthy siblings. Exposure to radiation was associated with further increased epigenetic age. Epigenetic modulation through DNA methylation may be a potential mechanism contributing to the aging process in childhood cancer survivors.

Background: L-asparaginase is an essential chemotherapeutic agent for treating pediatric acute lymphoblastic leukemia (ALL), but its use is commonly linked to severe complications, including pancreatitis. Pancreatic pseudocysts and panniculitis are a rare complication of L-asparaginase-induced pancreatitis.

Case presentation: We report the case of a 20-month-old girl diagnosed with pre-B ALL who developed pancreatitis, complicated by pseudocyst formation and panniculitis, following L-asparaginase therapy. The patient had clinical deterioration and the development of a mechanical ventilation requirement; septic shock was initially suspected. However, due to the appearance of cutaneous findings a few days later, radiologic investigations were performed, revealing the formation of intra-abdominal pseudocysts and subcutaneous fat necrosis. Consequently, a delayed diagnosis of pancreatitis was made, which was managed conservatively with a multidisciplinary approach. Eventually, the pseudocyst resolved with close clinical and radiologic monitoring.

Discussion: Pancreatic pseudocyst formation in pediatric oncology patients is uncommon but necessitates prompt recognition and an individualized treatment strategy. Panniculitis secondary to pancreatitis is even rarer and may serve as an early cutaneous clue to underlying pancreatic pathology. Conservative management of pseudocysts, supported by interventional radiology and close follow-up, can be effective in select cases, reducing the risk associated with surgical intervention.

Conclusion: This case underscores the importance of heightened clinical suspicion for pancreatic complications in pediatric patients receiving L-asparaginase. A multidisciplinary team approach is essential for effective diagnosis and treatment. Additional research is warranted to refine treatment guidelines for L-asparaginase-induced pancreatitis and its complications.

As of December 2022, calaspargase-pegol is the standard long-acting asparaginase formulation for patients younger than 22 years of age with acute leukemia. This 3-year retrospective evaluation compared real-world safety and efficacy of calaspargase-pegol versus pegaspargase in pediatric patients. Incidence and severity of hypersensitivity reactions, silent inactivation, and common asparaginase-associated adverse effects were collected. A total of 103 patients were included, of whom 50 received calaspargase-pegol and 58 received pegaspargase. Similar rates of symptomatic hypersensitivity reactions (20% vs. 10.3%; P=0.16) and silent inactivation (16% vs. 5.2%; P=0.18) were seen with calaspargase-pegol versus pegaspargase, respectively. However, 13 patients in the calaspargase-pegol group required conversion to short-acting asparaginase compared with 6 patients in the pegaspargase group (26% vs. 10.3%; P=0.03). Most adverse effects were comparable between the 2 groups, except for a lower rate of hyperbilirubinemia with calaspargase-pegol compared with pegaspargase (34% vs. 58.6%; P=0.01). Overall, no significant differences were found between the formulations in terms of rate of hypersensitivity reactions and silent inactivation. However, calaspargase-pegol did require more conversions to short-acting asparaginase therapy. Additional monitoring and preventative measures should be utilized to prevent calaspargase-pegol-associated adverse effects.

Iron deficiency and iron deficiency anemia (IDA) are prevalent during infancy. Timely iron prophylaxis can prevent associated irreversible complications. As part of the "Iron Like Turkey" program initiated in April 2004, iron prophylaxis is administered from the fourth month of life for at least 5 months. This study aims to evaluate the implementation of iron prophylaxis in our country and compare the effectiveness of different iron preparations used in prophylaxis. Between January 2021 and January 2024, the iron prophylaxis status of 248 healthy, full-term, breastfed infants 9 to 15 months old who were referred to the Pediatric Hematology outpatient clinic of Manisa City Hospital for various reasons was assessed. Iron prophylaxis was administered to 204 (82.3%) infants, of whom 92 (44.1%) had irregular usage. Among those receiving iron prophylaxis, 110 (53.9%) used Fe+3, while 94 (46.1%) used Sucrosomial Iron (SI). No infants received Fe+2 for prophylaxis. Iron deficiency anemia (IDA) was present in 142 (57.3%) cases. No significant difference was found between Fe+3and Sucrosomial Iron in terms of adherence and duration of prophylaxis (P>0.05). However, hemoglobin (Hb), mean corpuscular volume (MCV), ferritin, and transferrin saturation levels were significantly lower in the Sucrosomial Iron group compared with the Fe+3 group (P<0.05). Iron prophylaxis is an easy and effective method to prevent IDA. Both mothers during pregnancy and infants should receive prophylaxis on time and for an adequate duration. Further research is needed to evaluate the efficacy of different iron formulations used in prophylaxis.