Acute complications observed during intensive chemotherapy in pediatric patients with acute lymphoblastic leukemia: Single-center experience.

OBJECTIVE: In childhood acute lymphoblastic leukemia (ALL), very promising results were obtained thanks to the developments in treatment strategies in recent years. However, acute complications during treatment continue to be the important causes of mortality and morbidity. In this study, acute complications that develop during the treatment of ALL in childhood were evaluated. METHODS: Medical records of 47 patients treated according to (ALL Intercontinental Berlin-Frankfurt-Münster) 2009 protocol between 2016 and 2021 were evaluated retrospectively. RESULTS: Of 47 patients, 28 (59.6%) were male and 19 (40.4%) were female. The mean age at diagnosis was 5.9±4.2 years. Forty-four patients (93.6%) were pre-B cell ALL, 3 patients (6.4%) were pre-T cell ALL. Of 47 patients, 9 (19.1%) were high risk, 32 (68.1%) were intermediate risk, and 6 (12.8%) were standard risk. Acute complications developed in 38 patients (80.8%). Among these complications, infectious complications are the most common and these were followed by gastrointestinal complications, drug-related reactions, thrombotic, neurological, and endocrine/metabolic complications, respectively. CONCLUSION: In terms of complications that may develop, the threshold of suspicion should be kept low, and patients should be treated with the same medical team in fully equipped centers with a multidisciplinary approach. Inpatient treatment strategies should be applied especially in the early stages of treatment. The importance of inpatient treatment strategy, especially in the early stages of treatment, is emphasized.

Human chorionic gonadotrophin secreting adrenocortical neoplasm presenting with peripheral precocious puberty in an infant.

Adrenocortical tumor (ACT) is a rare malignant tumor which usually present with Cushing syndrome and virilization. Paraneoplastic syndromes (PNS) due to neoplasms can occur with peptides or cytokines secreted by the tumor. Here, we report a 13-month-old-male presented with severe masculinization. He had signs of precocious puberty with enlarged testicles, very high testosterone levels but low levels of gonadotrophins, and elevated ß-hCG. He underwent a left nephrectomy. The histopathological evaluation revealed a diagnosis of adrenocortical neoplasm. The levels of androgens and ß-hCG normalized after the resection of tumor, and the clinical findings improved within few months. We report the first pediatric patient with peripheral precocious puberty due to an ACT that secretes ß-hCG as PNS. A ß-hCG secreting ACT can cause severe virilization due to increased gonadal androgens stimulated by ß-hCG as well as due to increased adrenal androgens from the tumor.

Neurofibromatosis Type I and Hodgkin Lymphoma: Case Report and Review of the Literature.

OBJECTIVE: Neurofibromatosis 1 is an autosomal dominant inherited tumor susceptibility syndrome. Individuals with neurofibromatosis 1 have a 4-5 times increased risk of malignancy compared to the general population. Central nervous system and soft tissue tumors are common non-hematological malignancies in individuals with neurofibromatosis 1. Although the association of leukemia and non-Hodgkin lymphoma as hematologic malignancies in neurofibromatosis 1 has been reported frequently in the literature in these individuals, association with Hodgkin lymphoma has been reported very rarely. MATERIALS AND METHODS: We presented a patient with neurofibromatosis 1 who further developed Hodgkin lymphoma and reviewed the literature. CONCLUSION: Although rare, Hodgkin lymphoma can develop in individuals with neurofibromatosis 1. Hodgkin lymphoma should be kept in mind in cervical/supraclavicular lymphadenomegalies when evaluating patients with neurofibromatosis 1.

Desires vs. conditions: A qualitative study exploring the factors affecting the place of death of child with cancer in Turkey.

OBJECTIVE: The purpose of this study was to describe factors affecting the place of death of children with cancer at the end of life. METHODS: The descriptive phenomenological approach was used. Eighteen mothers who lost their children to cancer participated in in-depth interviews. Data were analysed using MAXQDA software version. Codes and categories were developed inductively from participants' narratives. RESULTS: The factors affecting the place of death of children were categorised into two main themes: (1) desires and (2) conditions. Most of the mothers reported that their deceased children wanted to be with their families at the end of life and they wanted to go home. The conditions related to health services were defined as the barriers to the death of their children in the places of death preferred by the mothers. CONCLUSION: The desire to be close to the child was the main factor affecting the parents' decisions. The findings revealed the prevailing circumstances in the death place decision beyond parental desires. These were the child's health conditions, physical conditions of hospitals, and the lack of home care and paediatric palliative care services, which were factors related to the system, and the lack of other options for parents.

How do the threshold values used in the decision on red blood cell and platelet transfusions comply with the guidelines?

OBJECTIVE: Blood transfusion is life-saving medical practice with significant risks. National and international guidelines have been established for indications related to blood use and threshold values for transfusions. In this study, we aimed to determine the erythrocyte and thrombocyte transfusion rates in surgical, internal, and emergency clinics in our hospital and to compare the threshold values before erythrocyte and platelet (PLT) transfusion among the clinics. METHODS: Red blood cell (RBC) and PLT transfusions in our hospital between January 2019 and June 2019 were retrospectively analyzed. Clinics were divided into three groups: surgical clinics, internal clinics, and emergency clinic. Demographic characteristics, pre-transfusion hemoglobin (Hb), and PLT values of patients were recorded. Data were analyzed statistically. RESULTS: During the 6-month study period, 5179 patients were transfused in 24,924 patients and the transfusion rate was 21%. In this period, a total of 14,518 units of blood products including 8369 units RBC suspension and 1390 units PLT suspension were transfused. The mean age of the patient was 50.32±28.88 years and the female/male ratio was 1.11. The most RBC transfusions were performed in the general internal medicine service in internal clinics and gynecology in surgical clinics. The most PLT transfusions were performed in the general medicine service in internal clinics and pediatric cardiovascular surgery in surgical clinics. ES transfusions were performed in the emergency medicine clinic with the lowest mean Hb value (Hb: 8.07±1.84 g/dl) and in the surgical clinics with the highest mean Hb value (Hb: 9.29±1.46 g/dl). TS transfusions were performed in internal clinics with the lowest mean PLT value (PLT: 44030±44075/mm3), while the highest mean PLT value (PLT: 97140±75782/mm3) was performed in surgical clinics. CONCLUSION: It was observed that threshold values in particular for PLT transfusions in our hospital were above the guideline recommendations. Our results suggest that the knowledge level of physicians about transfusion limits and practices should be increased.

Use of granulocyte transfusion in early period in life-threatening infections of pediatric hematology and oncology patients: A single-center experience.

Despite all the developments in medicine, infections continue to be one of the most important causes of mortality in pediatric hematology and oncology patients. The more severe the degree of neutropenia develops after intensive chemotherapy in cancer patients, and the longer the neutropenia duration, the higher the risk of infection. Granulocyte transfusion (GT) is used as supportive therapy in cases where the bone marrow needs time to recover in invasive bacterial or fungal infections along with severe neutropenia. The patients who had granulocyte transfusions in our clinic between June 2019 and June 2020 were reviewed retrospectively. A total of 15 units of granulocyte concentrate were used in 11 febrile neutropenia attacks of 9 patients. The demographic characteristics of the patients and features belonging to the period of GT were recorded. In our study, the clinical response rate after GT was 90.9 %, while the hematological response rate was 40 %. Most of the patients were treated succesfully, the mortality rate was 9%. We think that the most critical factor for success with GTs is determining the neutropenic patient in particular with a combination of high-risk malignancy and acute life-threatening infection for using GT. Also, early use of GT in those patients who do not recover despite appropriate antimicrobial and supportive treatment may contribute to improvement of the clinical conditon in a shorter period of time and reduction of repeated GTs.