The Vitamin D Status in Children With Newly Diagnosed Acute Lymphoblastic Leukemia and Its Potential Impact on the Primary Symptoms of Leukemia and Course of Induction Treatment.

BACKGROUND: Vitamin D deficiency is ubiquitous within the population of children. A similar problem is recognized among pediatric patients with acute lymphoblastic leukemia. The purpose of this study was to analyze the prevalence of vitamin D deficiency and to investigate the connection between vitamin D status and the course of induction treatment of ALL. MATERIALS AND METHODS: A cross-sectional study including 59 patients with newly diagnosed ALL from May 2017 until November 2020. RESULTS: Vitamin D deficiency was found in 39% of the patients. There were no seasonal differences in vitamin D status. Patients with optimal 25(OH)D concentration presented more profound thrombocytopenia ( P =0.015) and required more frequent platelet transfusions ( P =0.018). Good prognosis factors such as B phenotype and hyperdiploidy were also more frequent among children with higher 25(OH)D concentration ( P =0.01 and 0.014, respectively). CONCLUSIONS: The study showed that patients with a higher serum concentration of 25(OH)D presented deeper thrombocytopenia and needed more frequent transfusions. Moreover, those patients showed higher rates of B-cell leukemia and hyperdiploid karyotype. We did not find any influence of the possible exposure to sunlight (defined as the season of the year on admission) on serum 25(OH)D concentration, which supports the argument for supplementing vitamin D all year round. Moreover, the supplementing of vitamin D seems to be safe and does not cause any renal complications connected to calcium and phosphorus imbalance as no correlation between their levels and 25(OH)D concentration was found.

Hematopoietic stem cell transplantation in a patient with activated phosphoinositide 3-kinase δ syndrome: A case report and literature review.

Activated phosphoinositide 3-kinase δ syndrome (APDS) is a recently described disease characterized by recurrent infections, lymphoproliferation with a high risk of malignancy, early-onset cytopenia, and a propensity for autoimmune diseases. Hematopoietic stem cell transplantation (HSCT) has proven to be an effective treatment method; however, the recovery process after HSCT is prolonged and accompanied by complications. In this study, we present the case of a patient with APDS type 1. Despite showing signs of immunodeficiency at the age of 6 months, it took almost 6 years to reach a definitive diagnosis. The patient experienced recurrent infections, often accompanied by anemia requiring transfusions, and multifocal nonmalignant lymphoproliferation. Only after receiving the appropriate diagnosis was it possible to implement proper and accurate treatment. HSCT was performed when the patient was 6 years old, leading to significant improvement in his condition. At the 17-month post-HSCT follow-up, the boy is asymptomatic and in good general health, although close monitoring continues due to mixed chimerism and delayed humoral immune recovery. Applying HSCT before the patient develops malignancy contributes to expanding the use of HSCT as a treatment option for APDS type 1.

Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group.

Although isolated central nervous system (CNS) relapses are rare, they may become a serious clinical problem in intensively treated patients with high-risk neuroblastoma (NBL). The aim of this study is the presentation and assessment of the incidence and clinical course of isolated CNS relapses. Retrospective analysis involved 848 NBL patients treated from 2001 to 2019 at 8 centres of the Polish Paediatric Solid Tumours Study Group (PPSTSG). Group characteristics at diagnosis, treatment and patterns of relapse were analysed. Observation was completed in December 2020. We analysed 286 high risk patients, including 16 infants. Isolated CNS relapse, defined as the presence of a tumour in brain parenchyma or leptomeningeal involvement, was found in 13 patients (4.5%; 8.4% of all relapses), all of whom were stage 4 at diagnosis. Isolated CNS relapses seem to be more common in young patients with stage 4 MYCN amplified NBL, and in this group they may occur early during first line therapy. The only or the first symptom may be bleeding into the CNS, especially in younger children, even without a clear relapse picture on imaging, or the relapse may be clinically asymptomatic and found during routine screening. Although the incidence of isolated CNS relapses is not statistically significantly higher in patients after immunotherapy, their occurrence should be carefully monitored, especially in intensively treated infants, with potential disruption of the brain-blood barrier.

Acute Lymphoblastic Leukemia in Children: Better Transplant Outcomes After Total Body Irradiation-based Conditioning.

BACKGROUND/AIM: Comparison of transplant outcomes in long-term follow-up of children after total body irradiation (TBI)- or chemotherapy-based conditioning allogeneic hematopoietic cell transplantation (allo-HCT). PATIENTS AND METHODS: Patients undergoing allo-HCT for Acute lymphoblastic leukemia (ALL) conditioned either with TBI (n=55) or chemotherapy (n=84) were compared. The following transplant outcomes were analyzed: overall survival (OS), event-free survival (EFS), relapse incidence (RI), and graft-versus-host-disease (GVHD)-free-relapse-free survival (GRFS). RESULTS: All analyzed long-term transplant outcomes were significantly better for patients conditioned with TBI at 2 years after transplant. OS at 2 years was 84% after TBI and 60.5% after chemotherapy-conditioning (p=0.005). Risk factor analysis showed that two factors, TBI-based conditioning and transplant in first remission of ALL, significantly improved OS, EFS, GRFS, and decreased RI. CONCLUSION: TBI-based conditioning before allogeneic HCT in children with acute lymphoblastic leukemia provides significantly better transplant outcomes, when compared to chemotherapy-based conditioning.

[Analysis of factors influencing treatment results in children with soft tissue head and neck sarcomas - in the material of the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk]. / Analiza czynników wplywajacych na wyniki leczenia miesakow tkanek miekkich okolicy glowy i szyi u dzieci w materiale kliniki pediatrii, hematologii, onkologii i endokrynologii akademii medycznej w gdansku.

UNLABELLED: The aim of the study was to analyze the prognostic factors for the outcome in childhood head/neck soft tissue sarcomas (STS) treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk between 1992 and 2007. MATERIAL AND METHODS: Among 23 children with STS in ten it was located in non-parameningeal region, in eight it was in the parameningeal region, and in five in the orbit. Patients were qualified to particular disease stages according to the current diagnostic and therapeutic protocols (Cooperative Weichtel-sarkom Studie-CWS). The qualifications were based on the results of radiologic examinations (ultrasonography, computed tomography and magnetic resonance of the head and neck region, computed tomography of the chest and abdomen and bone scintigraphy) and also on myelogram smears and cerebrospinal fluid investigation. RESULTS: The longest history concerned the parameningeal (8 months), the shortest the orbital soft tissue sarcomas. Six out of ten patients with non-parameningeal STS were referred to the oncologist with a delay of 6.5 months. This was due to the initially false histopathological diagnosis which excluded the neoplastic process. 82.6% of tumours were diagnosed in advanced, inoperable stages. In 16 children rhabdomyosarcomas (including embryonal subtype - RME in 10, alveolar - RMA in five and undifferentiated in one); in seven non-rhabdomyosarcomas (non-RMS) were found. Good response to chemo- and radiotherapy was observed in 60% of children, mainly with RME. Nine children (mainly with non-parameningeal STS) relapsed. 15 patients are alive (including all with orbital, 6/10 with non-parameningeal and 4/8 with parameningeal STS). Eight children died of disease progression. CONCLUSIONS: 1. Poor outcome in our patients with non-parameningeal head/neck STS results from false initial diagnosis cansing a delay in referring them to the oncologist. It is essential to give more training to the histopathologists about neoplasms in children. 2. Because complete tumour resection in parameningeal STS is rarely feasible, the prognosis in this group is uncertain despite intense chemo- and radiotherapy. 3. The prognosis in orbital STS is usually good; however, they need mutilating surgery in selected cases not responding to therapy.

[Second neoplasms in children with solid tumours in the years 1992-2007. Experiences of Gdansk medical academy]. / Drugie nowoteory u dzieci z guzami litymi w latach 1992-2007. Doswiadczenia osrodka gdanskiego

UNLABELLED: The occurrence of a second tumour is a severe complication of neoplastic disease and its treatment, and it reduces the patient's chances to survive. The aim of the study was to assess the frequency of a second neoplasm and its clinical course in children treated in Gdansk in the years 1992-2007. PATIENTS AND METHODS: There were 420 children and young adults included in the study. They were treated for malignant tumours in this period in the Department of Paediatrics, Haematology, Oncology and Endocrinology Medical Academy of Gdansk. The medical records of these patients were analysed. RESULTS: The second neoplasm was diagnosed in 9 patients, aged 9 to 23 years. They were treated for nephroblastoma - 3 cases, soft tissue sarcoma - 2, Ewing's sarcoma - 1, medulloblastoma - 1, retinoblastoma - 1 and neuroblastoma - 1 case. The second neoplasms were: acute non lymphoblastic leukaemia - 2, soft tissue sarcoma - 2, osteosarcoma - 2, chondrosarcoma - 1, renal cell carcinoma - 1 and glioblastoma multiforme - 1 case. Time between the first and second diagnosis was from 3 and 11/12 to 19 years. Treatment failed in 5 out of 9 children treated for osteosarcoma (2/2), chondrosarcoma (1/1), soft tissue sarcoma (1/2) and acute non lymphoblastic leukaemia (1/2). These patients died of progression of neoplastic disease during 2 to 20 months after the diagnosis of the second tumour. CONCLUSIONS: The diagnosis of the second tumour worsens the prognosis. It is difficult to define the factors that predispose to the second neoplasm. In 5 cases the second neoplasm occurred in the region which was previously irradiated.

[Clinical characteristics and therapy outcome in children with stage IV Hodgkin's lymphoma--the experience of two oncological centres]. / Charakterystyka kliniczna i wyniki leczenia dzieci z choroba Hodgkina w IV stadium zaawansowania--doswiadczenia dwóch osrodków onkologicznych.

UNLABELLED: The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease. MATERIAL AND METHODS: The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh). RESULTS: Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopathological type of HD predominated. Poor response to standard treatment was observed in five children. One patient received additional cycles of chemotherapy MVPP/B-DOPA, four children were administered the 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd therapy lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation. Radiotherapy was given to 13 children. 13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years. In two of them late complications affecting hormonal status, cardio-pulmonary disorders and chronic B and C hepatitis were observed. Two children died including one admitted in a very severe condition, after long-lasting medical history who died of neutropenia-related sepsis. The second boy died 12 months after stem cell transplantation because of a second neoplasm--acute myeloblastic leukaemia. CONCLUSION: Chemo- and radiotherapy implemented according to protocols approved by the PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis made at earlier stages would result in giving less aggressive therapy, connected with a lower risk of durable late complications.

[Complications in children cured from Hodgkin's disease]. / Powiklania plucne u dzieci wyleczonych z choroby Hodgkina.

UNLABELLED: THE AIM of the study was to evaluate the incidence of pulmonary complications in children cured from Hodgkin's disease (HD). MATERIAL AND METHODS: 42 children with HD were treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, between 1994 and 2004. Stages of HD: II--26 children, III--10, IV--6; general symptoms (group B) were present in 50% of patients. Mediastinal involvement was found in 33 children, lung parenchyma infiltration in seven and bronchi involvement in one. In 1/3 of these patients the localization of HD within the chest was massive and symptomatic with signs of the superior caval vein symptoms, cough, dyspnea and cardiac tamponade. The treatment was conducted according to the schemes of the Polish Paediatric Leukaemia /Lymphoma Study Group. Eleven patients required therapy modification including six, in whom the intense line II chemotherapy Salvage 95 was introduced. 29 patients received chest irradiation with doses between J 75 and 36.5 Gy. Pulmonary function was evaluated from the results of clinical examination, 1 chest radiography (CXR), computed tomography, spirometry and lung scintigraphy. RESULTS: Pulmonary complications occurring as fatigue and diminished physical effort tolerance was observed in only two children. Some of the remaining 40 patients demonstrated asymptomatic abnormalities in the analysed tests. Abnormalities in CXR (upper mediastinal fibrosis, postoperational changes within the diaphragm and pneumonitis) were found in six children, minor ventilation problems in spirometry--in 12 and decreased lung perfusion in five. The scintigraphic signs of lung embolisation were not observed in our material. Most of the pulmonary complications occurred in children with enlarged lymph notes located within the chest, especially these with bulky disease presenting with cardio-pulmonary symptoms. In this group of patients the chest irradiation was performed in all except four children, three patients were also administered aggressive salvage chemotherapy. CONCLUSION: The pulmonary complications in children after completed therapy of HD are not common and mainly asymptomatic and occur predominantly in patients with massive mediastinal and/or lung involvement at diagnosis. The issue needs further evaluation of a more numerous group of HD survivors and a longer follow-up.