FROM EDITION
OUR COMMUNITY – ACTIVITIES OF THE NATIONAL SOCIETY OF PEDIATRIC HEMATOLOGISTS AND ONCOLOGISTS
Прогресс в детской гематологии-онкологии в XXI веке
Purpose of review. Reduction of child mortality is one of the Millennium Development Goals; as low-income and middle-income countries (LMICs) advance toward the achievement of this goal, initiatives aimed at reducing the burden of noncommunicable diseases, including childhood cancer, need to be developed.
Recent findings. Approximately 200 000 children and adolescents are diagnosed with cancer every year worldwide; of those, 80 % live in LMICs, which account for 90 % of the deaths. Lack of quality population-based cancer registries in LMICs limits our knowledge of the epidemiology of pediatric cancer; however, available information showing variations in incidence may indicate unique interactions between environmental and genetic factors that could provide clues to cause. Outcome of children with cancer in LMICs is dictated by late presentation and underdiagnosis, high abandonment rates, high prevalence of malnutrition and other comorbidities, suboptimal supportive and palliative care, and limited access to curative therapies. Initiatives integrating program building with education of healthcare providers and research have proven to be successful in the development of regional capacity. Intensity-graduated treatments adjusted to the local capacity have been developed.
Summary. Childhood cancer burden is shifted toward LMICs; global initiatives directed at pediatric cancer care and control are urgently needed. International partnerships facilitating stepwise processes that build capacity while incorporating epidemiology and health services research and implementing intensity-graduated treatments have been shown to be effective.
Оригинальные исследования
According to a number of trials in the USA, Europe, and Asia, Clostridium difficule is the most common cause of nosocomial diarrheas. It is significant that Cl. difficule-associated colitis may occasionally result in death in patients with severe comorbidities. Children with hematological, oncological and other severe diseases are no exception. This patient group may develop Cl. difficule-associated enterocolitides that lead to serious consequences. These complications bring a much greater threat to patients after hematopoietic stem cell transplantation. Long-term persistent Cl. difficule infection causes an increase in hospitalization length, drug costs, and a significant bowel lesion that may induce various complications and, as an example, graft-versus-host disease. There has been recently a decrease in the detection rates of Cl. difficule-induced enterocolitides. However, this problem has held a high position in the list of chemotherapy complications so far.
This paper deals with the epidemiology, diagnosis, and therapy of different types of Cl. difficule-associated enterocolitides, as well the authors’ experience with rifaximin used to prevent this complication in children in the context of hematopoietic stem cell transplantation.
The children with new-onset cancer admitted to hospital are observed to have varying degrees (10 to 50 % or more) of malnutrition depending on the nosology of a tumor just prior to starting therapy. Malnutrition is diagnosed in 75–80 % of cases at the stage of chemoradiation therapy and in 100 % after hematopoietic stem cell transplantation. Based on the experience with clinical nutrition at the Federal Research Center of Pediatric Hematology, Oncology and Immunology, the authors discuss the organizational and methodical problems of dietary accompaniment and nutritional support of children with cancer.
Clinical recommendations
Effective chemotherapy leading to rapid neoplastic cell disintegration is commonly accompanied by the too dangerous complication – tumor lysis syndrome (TLS). The latter is characterized by a number of metabolic disorders caused by the massive release of cell components into the bloodstream due to rapid lysis. This complication is more frequently encountered in the medical treatment of drug-susceptible tumors – hemoblastoses, such as acute lymphoblastic leukemia or Burkitt’s lymphoma. TLS may also spontaneously develop in massive tumors of other types distinguished by rapid proliferation even in the absence of chemotherapy. The basis for TLS pathogenesis is the overload and decompensation of release systems for intracellular metabolites including nucleic acids, proteins, phosphorus, and potassium, which may give rise to hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, and uremia. Crystallization of uric acid or calcium phosphate in the renal tubules may impair renal function and lead to acute renal failure and even death.
The current knowledge about the pathophysiology, predisposing factors, and predictors of TLS allow high-risk patients to be identified. The development of effective prevention methods in high-risk groups, such as hydration; monitoring of electrolyte and uric acid levels, use of drugs that delay the release of uric acid (allopurinol) or promote its rapid disintegration (rasburicase) allow a considerable decline in the number of patients with this menacing complication.
These guidelines summarize the current standards for TLS prevention in patients at high risk for this complication and outline approaches to treating evolving TLS.
PROBLEMS OF PRACTICAL HEMATOLOGY/ONCOLOGY
Iron overload is one of the major problems in patients with hereditary and acquired anemias whose treatment uses regular packed red blood cell transfusions. The paper analyzes in detail all currently used iron store-estimating methods, such as a calculation method; determination of serum ferritin levels; measurement of liver iron concentration by both determining directly iron content in a biopsy specimen and indirectly by T2*-weighted magnetic resonance imaging (MRI (T2*)), estimation of myocardial iron by MRI (T2*). According to the current concept, the patients receiving regular packed red blood cell replacement transfusions need adequate chelation therapy, the goal of which is to reduce toxic iron in the cell and the extracellular space and total body iron stores, which can prevent irreversible organ and tissue damage, increase survival, and improve quality of life in patients. The paper gives an algorithm for chelation therapy (to choose a chelating agent for its primary use, to modify therapy in relation to a treatment goal, and to discontinue chelation therapy). Intensive chelation therapy as continuous intravenous deferoxamine infusion or combined therapy (simultaneous or successive use of 2 different iron chelating agents) is recommended in case of severe iron overload. This literature review analyzes in detail the dosing regimen and possible combinations of different iron chelating agents, as well as criteria for evaluating their efficiency and safety.
Обзоры литературы
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder. Chronic neurological symptoms in patients, improperly examined and treated, and diagnostic errors during their first visit to a doctor suggest that most physicians are unaware of this syndrome and criteria for its diagnosis. The pediatrician who follows up an infant with cerebellar ataxia must know that this condition may be a manifestation of OMS. 50 % of childhood-onset OMS cases are associated with neuroblastoma (NB). However, to date difficulties have emerged in the early diagnosis of this condition and in the determination of initial examination scope and therapy policy. This paper reviews the literature on different aspects of the pathogenesis, diagnosis, clinical presentation, treatment, and prognosis of OMS. The issues of NB-associated OMS are considered separately.
The incidence of malignancies in children is steadily increasing and the improvement of antitumor treatment quality is of priority in the work of a pediatric oncologist. However, the basic diagnosis of these diseases is at times made at the level of primary care and its quality plays an important role in the further course of the disease and its prognosis. This paper familiarizes with the principles and rules of biopsy in pediatric oncology and with the current trends and technologies of surgical manipulations.
CLINICAL CASES
Haemolytic anaemia is a serious life-threatening complication that follows haematopoietic stem cell transplantation (HSCT) as a result of allo- or autoimmune derived anti-erythrocyte antibodies production. Unlike alloimmune haemolytic anaemia, mechanisms of autoimmune haemolysis in posttransplant period are poorly understood that explains the lack of standardized protocols for treatment of refractory or recurrent cases. We present here a report of a case of refractory autoimmune haemolysis in a patient with juvenile myelomonocytic leukemia after re-HSCT, as well as a review of current approaches to classification, diagnosis and therapy of immune haemolysis in posttransplant patients.
Regional centers and divisions of clinical hematology and oncology
The paper gives information on the young and dynamic team of the pediatric oncology unit, Nizhnevartovsk District Children’s Clinical Hospital (DCCH). The 27-bed unit, headed by Guzel Rafailovna Sharapova, annually receives as many as 37 primary patients with hematological diseases and cancer. It takes an active part in the activities of DCCH and the National Society of Pediatric Hematologists and Oncologists.
Мероприятия в области гематологии-онкологии в 2014 г.
ISSN 2413-5496 (Online)