| 1. |
- Alcorn, Sara, et al.
(author)
-
Practice Patterns of Stereotactic Radiotherapy in Pediatrics : Results From an International Pediatric Research Consortium
- 2018
-
In: Journal of pediatric hematology/oncology (Print). - : Lippincott Williams & Wilkins. - 1077-4114 .- 1536-3678. ; 40:7, s. 522-526
-
Journal article (peer-reviewed)abstract
- Purpose/Objectives: There is little consensus regarding the application of stereotactic radiotherapy (SRT) in pediatrics. We evaluated patterns of pediatric SRT practice through an international research consortium. Materials and Methods: Eight international institutions with pediatric expertise completed a 124-item survey evaluating patterns of SRT use for patients 21 years old and younger. Frequencies of SRT use and median margins applied with and without SRT were evaluated. Results: Across institutions, 75% reported utilizing SRT in pediatrics. SRT was used in 22% of brain, 18% of spine, 16% of other bone, 16% of head and neck, and <1% of abdomen/pelvis, lung, and liver cases across sites. Of the hypofractionated SRT cases, 42% were delivered with definitive intent. Median gross tumor volume to planning target volume margins for SRT versus non-SRT plans were 0.2 versus 1.4 cm for brain, 0.3 versus 1.5 cm for spine/other bone, 0.3 versus 2.0 cm for abdomen/pelvis, 0.7 versus 1.5 cm for head and neck, 0.5 versus 1.7 cm for lung, and 0.5 versus 2.0 cm for liver sites. Conclusions: SRT is commonly utilized in pediatrics across a range of treatment sites. Margins used for SRT were substantially smaller than for non-SRT planning, highlighting the utility of this approach in reducing treatment volumes.
|
|
| 2. |
|
|
| 3. |
- Björklund, Elisabet, et al.
(author)
-
Quality control of flow cytometry data analysis for evaluation of minimal residual disease in bone marrow from acute leukemia patients during treatment.
- 2009
-
In: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - : Lippincott Williams & Wilkins. - 1536-3678 .- 1077-4114. ; 31:6, s. 406-15
-
Journal article (peer-reviewed)abstract
- Low levels of leukemia cells in the bone marrow, minimal residual disease (MRD), are considered to be a powerful indicator of treatment response in acute lymphatic leukemia (ALL). A Nordic quality assurance program, aimed on standardization of the flow cytometry MRD analysis, has been established before implementation of MRD at cutoff level 10 as one of stratifying parameters in next Nordic Society of Pediatric Hematology and Oncology (NOPHO) treatment program for ALL. In 4 quality control (QC) rounds 15 laboratories determined the MRD levels in 48 follow-up samples from 12 ALL patients treated according to NOPHO 2000. Analysis procedures were standardized. For each QC round a compact disc containing data in list-mode files was sent out and results were submitted to a central laboratory. At cutoff level 10, which will be applied for clinical decisions, laboratories obtained a high concordance (91.6%). If cutoff level 10 was applied, the concordance would be lower (85.3%). The continuing standardization resulted in better concordance in QC3 and QC4 compared with QC1 and QC2. The concordance was higher in precursor B as compared with T-cell ALL. We conclude that after standardization, flow cytometry MRD detection can be reliably applied in international, multicenter treatment protocols.
|
|
| 4. |
- Boman, Krister K, et al.
(author)
-
Life after cancer in childhood : social adjustment and educational and vocational status of young-adult survivors
- 2004
-
In: Journal of pediatric hematology/oncology (Print). - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114 .- 1536-3678. ; 26:6, s. 354-62
-
Journal article (peer-reviewed)abstract
- PURPOSE: To evaluate the long-term social effect of illness and its treatment on young-adult survivors of pediatric cancer by addressing a selection of general social adjustment criteria.METHODS: In a cross-sectional case-control study, 30 young-adult survivors of childhood cancer were compared with (1). controls with no history of serious illness, matched by sex, age, and geographic area of residence, and (2). general population norms on the subject of educational and vocational factors, habitation, family/partner relationships, parenthood, and leisure activities.RESULTS: The educational status of survivors was similar to that of controls, although a smaller proportion of the patients expressed concrete plans for future vocational or educational advancement. Survivors had less frequently entered higher education compared with general population norms. A longer duration of treatment was related to a lower estimated socioeconomic level, and poor psychological coping with the illness experience was associated with the fact that they were still living with their parents, a shorter education, and a lower socioeconomic level.CONCLUSIONS: The social, vocational, and educational adjustment of relapse-free survivors from childhood cancer appears as only moderately, if at all, negatively affected by the illness and treatment history. However, the treatment intensity and particularly the survivors' coping with their illness experience may influence their ability to achieve long-term social goals. These findings suggest that special attention should be given to matters concerning education and partner relationships at long-term follow-up of pediatric cancer patients.
|
|
| 5. |
- Bonnesen, Trine G., et al.
(author)
-
Disease-specific Hospitalizations among 5-Year Survivors of Hepatoblastoma : A Nordic Population-based Cohort Study
- 2019
-
In: Journal of Pediatric Hematology/Oncology. - 1077-4114. ; 41:3, s. 181-186
-
Journal article (peer-reviewed)abstract
- Introduction: The long-term risk of somatic disease in hepatoblastoma survivors has not been thoroughly evaluated in previous studies. In this population-based study of 86 five-year HB survivors, we used inpatient registers to evaluate the risk for a range of somatic diseases.Methods: In total, 86 five-year survivors of hepatoblastoma were identified in the Nordic cancer registries from 1964 to 2008 and 152,231 population comparisons were selected. Study subjects were followed in national hospital registries for somatic disease classified into 12 main diagnostic groups. Standardized hospitalization rate ratios (RRs) and absolute excess risks were calculated.Results: After a median follow-up of 11 years, 35 of the 86 five-year hepatoblastoma survivors had been hospitalized with a total of 69 hospitalizations, resulting in an RR of 2.7 (95% confidence interval [CI], 2.2-3.5) and an overall absolute excess risk of 4.2 per 100 person-years. Highest risk was seen for benign neoplasms (RR=16) with 6 hospitalizations for benign neoplasms in the colon and one in rectum.Conclusions: The pattern of hospitalizations found in this first comprehensive follow-up of hepatoblastoma survivors seems reassuring. Less than 50% of the 5-year survivors had been hospitalized and often for diseases that were not severe or life-threatening.
|
|
| 6. |
- Borgstedt-Bendixen, Sofie E., et al.
(author)
-
Abdominal Complications During Treatment for Pediatric Acute Myeloid Leukemia
- 2022
-
In: Journal of Pediatric Hematology/Oncology. - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114 .- 1536-3678. ; 44:5, s. 220-229
-
Journal article (peer-reviewed)abstract
- Acute myeloid leukemia (AML) accounts for 15% to 20% of childhood leukemias. Because of high-intensive therapy, up to 5% of patients suffer from treatment-related mortality (TRM). Abdominal complications are frequent, however, literature on this subject is sparse. We aimed to characterize severe abdominal pain (AP) and hyperbilirubinemia experienced by pediatric AML patients treated according to the Nordic Society of Pediatric Hematology and Oncology (NOPHO)-AML 2004 protocol (n=313). Patients were censored at hematopoietic stem cell transplantation and relapse. Toxicity information was collected prospectively. Additional information was requested retrospectively from the treating centers. Sixteen episodes of hyperbilirubinemia and 107 episodes of AP were reported. The treating centers deemed infection (30%) and typhlitis (18%) as the most frequent causes of AP. Six patients developed appendicitis (2%). Patients experiencing concurrent AP and sepsis had a high risk of TRM (36%, n=4). Eighty percent of episodes with hyperbilirubinemia fulfilled the European Society for Bone and Marrow Transplantation criteria for sinusoidal obstruction syndrome. In conclusion, abdominal complications were frequent with infection considered the predominate cause. Most patients with hyperbilirubinemia fulfilled the criteria for sinusoidal obstruction syndrome. AML treatment might be associated with appendicitis. Patients suffering from concurrent AP and sepsis had a high risk of TRM indicating that high awareness of abdominal complications is essential to reduce mortality, especially during sepsis.
|
|
| 7. |
|
|
| 8. |
|
|
| 9. |
- Diffner, Eva, et al.
(author)
-
Expression of VEGF and VEGF Receptors in Childhood Precursor B-cell Acute Lymphoblastic Leukemia Evaluated by Immunohistochemistry
- 2009
-
In: Journal of pediatric hematology/oncology (Print). - : Wolters Kluwer. - 1077-4114 .- 1536-3678. ; 31:9, s. 696-701
-
Journal article (peer-reviewed)abstract
- Perturbation in the expression and signaling pathways of vascular endothelial growth factor (VEGF) has been linked to pathogenesis of hematologic malignancies. We investigated the expression and clinical importance of VEGF and two of its receptors, VEGFR-1 and VEGFR-2, in childhood precursor B-cell acute lymphoblastic leukemia (pre-B ALL) by using immunohistochemistry. These angiogenic proteins were expressed in the majority of leukemic bone marrow samples. Notably, pre-B ALL patients had significantly increased expression of VEGFR-1 compared with no expression in the nonmalignant group, indicating a link between VEGFR-1 protein expression and pre-B ALL. These novel findings suggest that VEGFR-1 may have clinical importance in childhood pre-B ALL.
|
|
| 10. |
|
|
| 11. |
- Ek, Torben, 1963, et al.
(author)
-
Ara-C fever and infections after high-dose ara-C treatment in pediatric lymphoid malignancies
- 2005
-
In: J Pediatr Hematol Oncol. - 1077-4114. ; 27:7, s. 364-9
-
Journal article (peer-reviewed)abstract
- The objective of this study was to examine the incidence and characteristics of Ara-C-related fever and the frequency and severity of infections after single-drug, high-dose Ara-C treatment (HDAC) in children treated for acute lymphoblastic leukemia (ALL) or non-Hodgkin lymphoma (NHL). A retrospective review was performed of 169 courses of HDAC administered to 57 patients (age 1.8-17.8 years). Procalcitonin values (PCT) were analyzed in a subgroup of 16 patients. Fever during HDAC occurred in 113 of 169 (67%) cases. C-reactive protein (CRP) was elevated in the febrile patients (median 38 mg/L [range 3-150]). PCT was elevated (>0.5 ng/mL) during HDAC in 4 of the 16 evaluated patients. Corticosteroids could inhibit fever (P < 0.001). Myelosuppression after HDAC was prominent: 99% developed neutropenia (<0.5 x 10/L) and 92% thrombocytopenia (<25 x 10/L). An early lymphopenia (median 0.1 x 10/L [range 0.01-0.68]) was seen during the first week. G-CSF was used after 12 of the 169 HDAC courses. A febrile episode occurred after 93 of the 169 (55%) HDAC courses, with no need for intensive care and no deaths. The incidence of viridans streptococcal septicemia was 2 of the 169 cases. Ara-C fever is common, and evaluation with inflammation markers is complicated by the fact that HDAC can induce a moderate release of both CRP and PCT. Profound neutropenia and lymphopenia are causative factors for the high incidence of infections, but the risk of life-threatening complications after HDAC in children in remission of lymphoid malignancies is low, even without prophylactic use of colony-stimulating factors.
|
|
| 12. |
- Ek, Torben, 1963, et al.
(author)
-
Intensive treatment for childhood acute lymphoblastic leukemia reduces immune responses to diphtheria, tetanus, and Haemophilus influenzae type b
- 2004
-
In: J Pediatr Hematol Oncol. - 1077-4114. ; 26:11, s. 727-34
-
Journal article (peer-reviewed)abstract
- OBJECTIVES: Immunity to diphtheria toxoid (D), tetanus toxoid (T), and Haemophilus influenzae type b (Hib) is affected in children with acute lymphoblastic leukemia (ALL). The aims were to examine immunity and to compare the response to immunization at 1 or 6 months after treatment. METHODS: Thirty-one patients were immunized with DT and conjugated Hib vaccine (ActHib) at 1 month or 6 months after treatment of ALL with the NOPHO 92 protocol. Antibody levels were determined before and 3 weeks after vaccination. Specific T and Hib antibody-secreting cells of IgG/IgA/IgM isotypes were analyzed in peripheral blood using an ELISPOT technique. RESULTS: All specific antibody levels decreased during ALL treatment, and protective levels after treatment were noted for 17% against D, 33% against T, and 100% against Hib. No high-risk patient had full D or T protection after treatment. After vaccination all the standard- and intermediate-risk patients achieved full protection against D, T, and Hib. The high-risk group showed insufficient immune response (full protection after vaccination: D 56%, T 22%, Hib 78%). No difference was found between vaccination at 1 month or 6 months after treatment. The poor antibody production in the high-risk group correlated to low numbers of antibody-secreting cells. CONCLUSIONS: Nonprotective antibody levels against D, T, and Hib after childhood ALL are more common than previously thought. Insufficient immune response was restricted to the high-risk group and was related to a low number of memory B cells in this study. Immunizations should be included in follow-up after childhood ALL, and the policy should be adapted to treatment intensity.
|
|
| 13. |
- Forestier, Erik, et al.
(author)
-
The incidence peaks of the childhood acute leukemias reflect specific cytogenetic aberrations.
- 2006
-
In: Journal of pediatric hematology/oncology (Print). - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114 .- 1536-3678. ; 28:8, s. 486-95
-
Journal article (peer-reviewed)abstract
- The correlation between age and karyotype was studied in 1425, 0 to 14.9 years old children who were diagnosed with acute lymphoblastic leukemia (ALL) or acute myeloblastic leukemia. Almost 80% of the non-Down B-cell precursor ALL cases in the 2 to 7 years frequency peak group who had aberrant cytogenetic results had either a high-hyperdiploid clone (51 to 61 chromosomes) or a translocation t(12;21)(p13;q22). Among B-cell precursor ALL cases, high white blood cell counts correlated with earlier age at diagnosis (rS=-0.23; P<0.001) being most evident for 11q23/MLL-aberrations, translocation t(12;21)(p13;q22), and high-hyperdiploidy. Among acute myeloblastic leukemia patients, frequency peaks were found for those with MLL/11q23 rearrangements (peak: first year), Down syndrome (peak: second to third year), or cytogenetic abnormalities other than translocations t(8;21), t(15;17), and inv(16)/t(16;16) (peak: first to third year). The epidemiology of the cytogenetic subsets of acute leukemias questions whether age as a disease-related prognostic parameter has any relevance in childhood leukemia clinical research beyond being a surrogate marker for more important, truly biologic features such as cytogenetic aberrations and white cell count at diagnosis. Further research is needed to explore whether the 2 to 7 years age incidence peak in childhood ALL harbor yet unidentified cytogenetic subsets with the same natural history as the high-hyperdiploid and t(12;21)-positive leukemias.
|
|
| 14. |
- Gustafsson, Britt, et al.
(author)
-
KI, WU, and Merkel Cell Polyomavirus DNA was not Detected in Guthrie Cards of Children who Later Developed Acute Lymphoblastic Leukemia
- 2012
-
In: Journal of pediatric hematology/oncology (Print). - 1077-4114 .- 1536-3678. ; 34:5, s. 364-367
-
Journal article (peer-reviewed)abstract
- Background: Neonatal dried blood spots (Guthrie cards) have been used to demonstrate a prenatal origin of clonal leukemia-specific genetic aberrations in several subgroups of childhood acute lymphoblastic leukemia (ALL). One hypothesis suggests that an infectious agent could initiate genetic transformation already in utero. In search for a possible viral agent, Guthrie cards were analyzed for the presence of 3 newly discovered polyomavirus Karolinska Institutet polymavirus (KIPyV), Washington University polyomavirus (WUPyV), and Merkel cell polyomavirus (MCPyV). Methods: Guthrie cards from 50 children who later developed ALL and 100 matched controls were collected and analyzed by standard or real-time polymerase chain reaction for the presence of the VP1 region of KIPyV, WUPyV, and MCPyV, and the LT region for MCPyV. Results and Conclusions: DNA from KIPyV, WUPyV, and MCPyV was not detected in neonatal blood samples from children with ALL or controls. Prenatal infections with these viruses are not likely to be etiological drivers for childhood leukemogenesis.
|
|
| 15. |
|
|
| 16. |
|
|
| 17. |
- Hedström, Mariann, et al.
(author)
-
Perceptions of distress among adolescents recently diagnosed with cancer
- 2005
-
In: Journal of pediatric hematology/oncology (Print). - 1077-4114 .- 1536-3678. ; 27:1, s. 15-22
-
Journal article (peer-reviewed)abstract
- With the goal of studying perceived distress among adolescents recently diagnosed with cancer, 56 adolescents were interviewed by telephone 4 to 8 weeks after diagnosis. The interviews included a structured interview guide, the Hospital Anxiety and Depression Scale, and the subscales Mental Health and Vitality from SF-36. "Losing hair" and "missing leisure activities" were identified as the most prevalent aspects of distress, whereas "missing leisure activities" and "fatigue" were rated with the highest levels of distress. "Worry about not getting well," "mucositis," "nausea," "pain from procedures and treatments," and "worry about missing school" were rated as the overall worst aspects by most adolescents. Twelve percent reached the cutoff score for potential clinical anxiety and 21% for potential clinical depression. Ratings of Mental Health and Vitality were lower than norm values. Prevalence of pain from procedures/treatments was higher among those who scored in the clinical range of depression, and more adolescents who were treated at a local hospital scored in the clinical range of anxiety. The findings show that ratings of prevalence, levels, and aspects perceived as the worst are not necessarily in accordance, that adolescents scoring in the clinical range of psychological distress are in the minority, and that the adolescents experience reduced physical and mental well-being.
|
|
| 18. |
|
|
| 19. |
- Hero, Barbara, et al.
(author)
-
Genomic Profiles of Neuroblastoma Associated With Opsoclonus Myoclonus Syndrome
- 2018
-
In: Journal of Pediatric Hematology/Oncology. - 1077-4114. ; 40:2, s. 93-98
-
Journal article (peer-reviewed)abstract
- Opsoclonus myoclonus syndrome (OMS), often called "dancing eyed syndrome," is a rare neurological condition associated with neuroblastoma in the majority of all childhood cases. Genomic copy number profiles have shown to be of prognostic significance for neuroblastoma patients. The aim of this retrospective multicenter study was to analyze the genomic copy number profiles of tumors from children with neuroblastoma presenting with OMS at diagnosis. In 44 cases of neuroblastoma associated with OMS, overall genomic profiling by either array-comparative genomic hybridization or single nucleotide polymorphism array proved successful in 91% of the cases, distinguishing tumors harboring segmental chromosome alterations from those with numerical chromosome alterations only. A total of 23/44 (52%) tumors showed an segmental chromosome alterations genomic profile, 16/44 (36%) an numerical chromosome alterations genomic profile, and 1 case displayed an atypical profile (12q amplicon). No recurrently small interstitial copy number alterations were identified. With no tumor relapse nor disease-related deaths, the overall genomic profile was not of prognostic impact with regard to the oncological outcome in this series of patients. Thus, the observation of an excellent oncological outcome, even for those with an unfavorable genomic profile of neuroblastoma, supports the hypothesis that an immune response might be involved in tumor control in these patients with OMS.
|
|
| 20. |
- Hoven, Emma, et al.
(author)
-
The Influence of Pediatric Cancer Diagnosis and Illness Complication Factors on Parental Distress
- 2008
-
In: Journal of pediatric hematology/oncology (Print). - 1077-4114 .- 1536-3678. ; 30:11, s. 807-814
-
Journal article (peer-reviewed)abstract
- Objective: We investigated how primary diagnosis and risk for diagnosis-related complication factors influence parental distress after a childs cancer diagnosisMethods: We used a model in which "complicated childhood cancers" were grouped into 1 category after identifying a set of potentially influential illness complication variables. This category included central nervous system tumors, acute myeloid leukemia, and bone tumors. Parental distress in that category (n = 144) was compared with distress after acute lymphoblastic leukemia (n = 177) in the child. In addition, comparisons were made between parents of the specific diagnosis groups. A multidimensional questionnaire assessing symptoms of distress was used.Results: Parents in the complicated cancer category showed significantly heightened disease-related fear, anxiety, depression, loss of control, late effects-related uncertainty, and poorer self-esteem compared with parents of children with acute lymphoblastic leukemia. Significantly heightened parental distress was associated with the child having been treated with cranial irradiation.Conclusions: Relatively heightened distress in parents of children with complicated cancer is influenced by diagnosis-related factors like an intricate prediagnostic phase, and uncertainty about late effects. Heightened vulnerability to distress signals exceptional needs for support and information among parents of children treated for central nervous system or bone tumors.
|
|
| 21. |
|
|
| 22. |
- Jarvis, Kirsten B., et al.
(author)
-
Asymptomatic Right Atrial Thrombosis After Acute Lymphoblastic Leukemia Treatment
- 2021
-
In: Journal of pediatric hematology/oncology (Print). - : Lippincott Williams & Wilkins. - 1077-4114 .- 1536-3678. ; 43:4, s. E564-E566
-
Journal article (peer-reviewed)abstract
- Right atrial thrombosis is a rare, but potentially serious complication of acute lymphoblastic leukemia treatment. We conducted a retrospective multicenter study to assess the incidence, treatment, and outcome of asymptomatic right atrial thrombosis detected at routine echocardiography of children after acute lymphoblastic leukemia treatment in the Nordic and Baltic countries. Eleven (2.7%, 95% confidence interval, 1.4-4.9) of 406 patients had asymptomatic right atrial thrombosis, ranging from 10 to 25 mm at detection. Three patients were treated with anticoagulation. None of the thromboses affected cardiac function, and they showed neither sign of progress nor spontaneous or treatment-related regress at follow-up.
|
|
| 23. |
- Levinsen, Mette, et al.
(author)
-
Efficacy and Toxicity of Intrathecal Liposomal Cytarabine in First-line Therapy of Childhood Acute Lymphoblastic Leukemia
- 2016
-
In: Journal of Pediatric Hematology/Oncology. - 1077-4114 .- 1536-3678. ; 38:8, s. 602-609
-
Journal article (peer-reviewed)abstract
- We investigated efficacy and toxicity of replacing conventional triple (cytarabine, methotrexate, and hydrocortisone) intrathecal therapy (TIT) with liposomal cytarabine during maintenance therapy among 40 acute lymphoblastic leukemia patients. Twenty-eight of 29 patients in the TIT arm received TIT and 9/11 in the liposomal cytarabine arm received liposomal cytarabine. Arachnoiditis occurred in all initial 5 patients given liposomal cytarabine and intrathecal prednisolone succinate. Subsequently liposomal cytarabine was given with systemic dexamethasone. Neurotoxicity occurred at 6/27 liposomal cytarabine administrations with concomitant dexamethasone (22%). More liposomal cytarabine-treated patients experienced neurotoxicity in relation to intrathecal therapy during at least 1 cycle compared with TIT-treated patients (6/9 [67%] vs. 3/28 [11%], P=0.002). Apart from intermittent lower extremity sensory pain in 1 liposomal cytarabine-treated patient, no permanent adverse neurological sequelae were observed. In intention-to-treat analysis, projected 5-year event-free survival (pEFS-5y) was borderline higher for patients in the liposomal cytarabine arm compared with the TIT arm (1.0 vs. 0.69, P=0.046). However, pEFS-5y and projected 5-year relapse-free survival did not differ signficantly between patients treated with liposomal cytarabine or TIT (1.0 vs. 0.73, P=0.10; 1.0 vs. 0.76, P=0.12). Larger prospective trials are needed to explore whether liposomal cytarabine should be used as first-line prevention of relapse.
|
|
| 24. |
- Marky, Ildiko, 1940, et al.
(author)
-
Intensive chemotherapy without radiotherapy gives more than 85% event-free survival for non-Hodgkin lymphoma without central nervous involvement: a 6-year population-based study from the nordic society of pediatric hematology and oncology.
- 2004
-
In: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114. ; 26:9, s. 555-60
-
Journal article (peer-reviewed)abstract
- BACKGROUND: The prognosis in childhood non-Hodgkin lymphoma (NHL) has improved dramatically during recent decades. The authors report the results from a 6-year population-based study of clinical characteristics and treatment results of NHL from the five Nordic countries. METHODS: All children younger than 15 years of age at diagnosis with NHL diagnosed from 1995 to 2000 were stratified and treated according to immunophenotypic classification and stage of disease. RESULTS: A total of 230 patients were diagnosed with primary NHL, which gives an annual incidence of 0.9/100.000 children, with a median age of 8 years. Seven percent of the children were below 3 years of age at diagnosis. The male/female ratio was 2.3 and was unrelated to age. Patients with pre-B and T-cell NHL constituted 33%, B-cell NHL 53%, and anaplastic large cell lymphoma (ALCL) 14%. According to Murphy's classification, 14% had stage 1, 17% stage 2, 50% stage 3, and 19% stage 4 disease, 12 of whom (28%) had central nervous involvement (CNS) at diagnosis. By January 1, 2003, four children had died during induction, three children died in remission (2, 6, and 26 months from diagnosis), and 24 children experienced a relapse. At 5 years, the probability of event-free survival (p-EFS) was 86+/-2% for all children. The 5-year p-EFS values for stages 1 through 4 were 94%, 97%, 83%, and 79%, respectively. The 5-year p-EFS values were 91% for B-cell, 87% for pre-B, 81% for ALCL, and 79% for T-cell NHL. The 12 patients with CNS involvement at diagnosis had a significantly poorer outcome than stage 4 patients with CNS involvement (p-EFS = 50% vs. 90%, P < 0.01). The 218 patients without CNS disease at diagnosis had a 5-year p-EFS of 88%. CONCLUSIONS: With modern intensive chemotherapy, more than 85% of NHL patients will achieve long-lasting first remission. In the future, preventing death during induction and remission and improving therapy for patients with CNS disease would have a major impact on the overall p-EFS.
|
|
| 25. |
- Mellgren, Karin, 1962, et al.
(author)
-
Plasma Cytokine Profiles at Diagnosis in Pediatric Patients With Non-Hodgkin Lymphoma
- 2012
-
In: Journal of Pediatric Hematology Oncology. - 1077-4114. ; 34:4, s. 271-275
-
Journal article (peer-reviewed)abstract
- Non-Hodgkin lymphoma (NHL) has been associated with elevated levels of inflammatory and immune-regulating cytokines, and polymorphisms in the genes encoding interleukin (IL)-10 and tumor necrosis factor (TNF)-alpha have been associated with increased incidence of certain subtypes of NHL. The aim of the present study was to screen for a broader spectrum of growth factors and inflammatory mediators and to compare the profiles in different subtypes of NHL in pediatric patients. Serum samples were collected at diagnosis from 31 pediatric patients diagnosed with NHL admitted at Rigshospitalet, Copenhagen, between 1995 and 2008. Cytokines and growth factors were measured in serum using the Luminex platform by application of a 30-plex kit. Levels of IL-6, IL-2R, IL-10, TNF-RI, and macrophage inflammatory protein-1 alpha were significantly higher in patients with anaplastic large-cell lymphoma compared with patients diagnosed with B-cell lymphomas and lymphoblastic lymphomas. High levels of IL-4, IL-13, TNF-RI, and epidermal growth factor were associated with a poorer general condition at diagnosis. The present study suggests that NHL subgrouping and the general condition of pediatric patients at diagnosis are associated with plasma levels of growth factors and inflammatory mediators at presentation.
|
|
| 26. |
- Norberg, Annika Lindahl
(author)
-
Parents of children surviving a brain tumor : burnout and the perceived disease-related influence on everyday life.
- 2010
-
In: Journal of pediatric hematology/oncology (Print). - 1077-4114 .- 1536-3678. ; 32:7, s. e285-9
-
Journal article (peer-reviewed)abstract
- Parents of children diagnosed with a brain tumor often report distress, even after successfully completed cancer treatment. The aim of this study was to examine predictors of burnout (ie stress-induced exhaustion) in parents of children who have had a brain tumor. Twenty-four mothers and 20 fathers completed self-report questionnaires on 2 occasions at an interval of 7 months. Controlling for generic stress, parents' perception of the influence of the disease on everyday life-predicted burnout symptoms. Moreover, parents' appraisal of a disease-related influence on everyday life showed stability, implying that parental stress may be chronic. The findings encourage furthermore investigation of chronic stress among parents of children diagnosed with cancer.
|
|
| 27. |
- Pramanik-Jonsson, Lotta, et al.
(author)
-
Severe thrombocytopenia due to bone marrow failure in children with dyskeratosis congenita does not respond to eltrombopag treatment : case series
- 2024
-
In: Journal of Pediatric Hematology/Oncology. - : Wolters Kluwer. - 1077-4114 .- 1536-3678. ; 46:1, s. 57-62
-
Journal article (peer-reviewed)abstract
- Dyskeratosis congenita is a rare inherited disease with classic cutaneous symptoms, sometimes accompanied with more severe extracutaneous manifestations such as bone marrow failure, which can be lethal. Eltrombopag is an orally available thrombopoietin receptor agonist in clinical use for increasing platelet levels in patients with immune thrombocytopenia and aplastic anemia. Here, 3 pediatric patients with dyskeratosis congenita are presented with varying disease severity, in which off-label eltrombopag treatment had no clinical effect on bone marrow failure. This, in addition to the negative results in a previous case report, supports the preclusion of eltrombopag use in dyskeratosis congenita.
|
|
| 28. |
- Ranta, Susanna, et al.
(author)
-
Extracorporeal Membrane Oxygenation Support in Children With Hematologic Malignancies in Sweden
- 2021
-
In: Journal of Pediatric Hematology/Oncology. - : Wolters Kluwer. - 1077-4114 .- 1536-3678. ; 43:2, s. e272-e275
-
Journal article (peer-reviewed)abstract
- Background: Extracorporeal membrane oxygenation (ECMO) is used in severe respiratory and/or circulatory failure when conventional critical care fails. Studies on patients with hematologic malignancies on ECMO have shown contradictory results; immunosuppression and coagulopathy are relative contraindications to ECMO.Observations: This nationwide Swedish retrospective chart review identified 958 children with hematologic malignancies of whom 12 (1.3%) required ECMO support. Eight patients survived ECMO, 7 the total intensive care period, and 6 survived the underlying malignancy.Conclusions: ECMO may be considered in children with hematologic malignancy. Short-term and long-term survival, in this limited group, was similar to that of children on ECMO at large.
|
|
| 29. |
|
|
| 30. |
|
|
| 31. |
- Warrier, Indira, et al.
(author)
-
Factor IX inhibitors and anaphylaxis in hemophilia B
- 1997
-
In: Journal of Pediatric Hematology/Oncology. - : Ovid Technologies (Wolters Kluwer Health). - 1077-4114 .- 0192-8562. ; 19:1, s. 23-27
-
Journal article (peer-reviewed)abstract
- Purpose: We present clinical and laboratory data on 18 children from 12 hemophilia treatment centers in the United States, Canada, and Europe with the purpose of disseminating information regarding a recently recognized, potentially life-threatening complication of treatment in very young children with hemophilia B. Patients and Methods: Twelve hemophilia centers from the United States, Canada, and Europe provided clinical information and laboratory data concerning 18 children who had severe allergic reactions to infused factor (F) IX in close association with the development of an inhibitor to FIX. Laboratory testing for establishment of the diagnosis of hemophilia B and inhibitor to FIX was done locally at the centers treating these patients. FIX gene analysis was performed at one of six molecular genetics institutes. Results: All 18 children had severe hemophilia B, and in each an inhibitor antibody to FIX developed. The median age at the time of anaphylaxis (or anaphylactoid reaction) was 16 months, and the median number of exposure days to FIX was 11. The FIX inhibitor was detected almost simultaneously with the first occurrence of anaphylaxis in 12 of 18 patients. Maximum inhibitor liters were 4.5-600 Bethesda units (BU), with a median titer of 48 BU. FIX gene analysis, performed in 17 of 18 patients, demonstrated complete deletion of the FIX gene in 10 and major derangements in seven. Immune tolerance induction (ITI) regimens have been attempted in 12 patients, with generally poor responses. Two of the 12 experienced nephrotic syndrome while on ITI. Recombinant FVIIa has been successfully used to treat bleeding episodes in 11 of these children. Conclusion: Physicians treating young children with hemophilia B should be aware of the potentially life- threatening complication of anaphylaxis. Children with complete gene deletions or major derangements of the FIX gene appear to be at greater risk. Those identified by genotype as being at greater risk may need to receive their first 10-20 treatments in a medical facility equipped for handling such emergencies. Recombinant FVIIa, although not licensed for use in the United States, appears to be the most suitable treatment option for bleeding episodes in such patients.
|
|
| 32. |
- Österlundh, Gustaf, 1956, et al.
(author)
-
Regional cerebral blood flow and neuron-specific enolase in cerebrospinal fluid in children with acute lymphoblastic leukemia during induction treatment.
- 1999
-
In: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - 1077-4114. ; 21:5, s. 378-83
-
Journal article (peer-reviewed)abstract
- PURPOSE: To investigate possible side effects on the central nervous system from intrathecal methotrexate given during induction treatment for acute lymphoblastic leukemia in childhood. PATIENTS AND METHODS: Twenty-five children with acute lymphoblastic leukemia were examined by cerebral single photon emission computed tomography at the beginning of treatment (16 untreated, 9 during the first week) and after 4 weeks of treatment. Cerebrospinal fluid was sampled for analyses of neuron-specific enolase on four occasions in 54 patients. RESULTS: Regional cerebral blood flow became impaired during treatment in all patients. The single photon emission computed tomography score for nonhomogeneous perfusion increased from 6.4/50 to 16.6/50. Hypoperfusion was global without any clear preference for any lobe. The cerebellum was not affected. Neuron-specific enolase increased significantly during treatment, with a peak after 1 week, followed by a gradual decrease, but it was still significantly elevated after 4 weeks. CONCLUSIONS: Nonhomogeneous cerebral hypoperfusion was found in all patients during induction treatment, including repeated intrathecal administration of methotrexate, but before systemic high-dose methotrexate. Signs of neuronal injury, in the form of a moderate increase in neuron-specific enolase in the cerebrospinal fluid, were found early in the treatment. Follow-up is needed to evaluate the long-term impact of these findings.
|
|
| 33. |
- Österlundh, Gustaf, 1956, et al.
(author)
-
Studies of cerebral blood flow in children with acute lymphoblastic leukemia: case reports of six children treated with methotrexate examined by single photon emission computed tomography.
- 1997
-
In: Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology. - 1077-4114. ; 19:1, s. 28-34
-
Journal article (peer-reviewed)abstract
- PURPOSE: Cranial irradiation has been widely used in order to prevent central nervous system (CNS) relapse of acute lymphoblastic leukemia (ALL) in childhood. Owing to the risk of late side effects, the Nordic Society for Pediatric Hematology and Oncology (NOPHO) replaced CNS irradiation with systemic high-dose methotrexate (HDMTX) in 1992. A prospective study of the effects of HDMTX and intrathecal MTX on CNS function is in progress at our center. PATIENTS AND METHODS: Six ALL patients underwent (99m)Tc-HMPAO single-photon emission computed tomography (SPECT) examination of regional cerebral blood flow (rCBF): three owing to neurological symptoms during treatment for ALL and the other three as part of the study. RESULTS: All the patients had various degrees of disturbed rCBF, which was more pronounced in the patients with neurological symptoms. One patient had severe symptoms and impaired rCBF after three intrathecal injections of MTX but before administration of HDMTX. CONCLUSIONS: Impaired cerebral perfusion was found in patients with and without neurological symptoms during treatment for ALL. The impact of these findings is still unknown, from both the long- and the short-term perspective. The possibility that intrathecal MTX alone or in combination with HDMTX may affect rCBF through vascular damage should be further investigated, in terms of both mechanisms and clinical significance.
|
|
