Effect of platelet count and platelet transfusion on fever duration in patients with febrile neutropenia.

Platelets play a role in hemostasis, thrombosis, and vascular integrity. They also play a major role in the development of inflammation and the activation of immune responses. They have phagocytic activity, stimulate the secretion of immune modulators, and activate other immune cells, which results in platelet-neutrophil aggregation, platelet-induced neutrophil degranulation, and the formation of neutrophil extracellular traps. Data on 124 febrile neutropenia attacks were retrospectively examined. Patients' age, sex, diagnosis, and relapse history were obtained. The complete blood count levels on the first and last febrile day of the febrile neutropenia attacks, duration of fever, and number, type, and timing of thrombocyte suspension transfusions were recorded. The patients were divided into three groups according to the day of fever when the platelet suspension was administered (1 day, 2-3 days, and >3 days); the duration of fever was compared between the three groups. The fever duration of those who were transfused with platelet suspension on the first day of fever was found to be significantly shorter (p = 0.03 and p < 0.001, respectively). When treating a patient with febrile neutropenia, if thrombocyte suspension transfusion is indicated, transfusing thrombocytes in the first days of fever shortens the fever duration and improves the prognosis of febrile neutropenia attack, supporting the hypothesis that not only neutrophils but also platelets may play a role in fighting against microorganisms.

Neurofibromatosis Type 1 in Children: A Single-Center Experience.

OBJECTIVE: Neurofibromatosis (NF) is the most common autosomal dominantly inherited neurocutaneous syndrome. The characteristic features of NF type 1 (NF-1) are café au lait spots, axillary and inguinal freckling, peripheral neurofibromas, optic pathway glioma, and Lisch nodules. The present study aimed to analyze the clinical features of children with NF-1. MATERIALS AND METHODS: In this study, the children with NF-1 diagnosed and followed-up in our center between 2000 and 2020 were retrospectively evaluated. Demographic and clinical features of patients were defined. RESULTS: The study group consisted of 52 patients. Of those, 25 were boys and 27 were girls. The children's median age at diagnosis was 5.9 years (1-15.8). Café au lait (CAL) spots and axillary/inguinal freckling were observed in 50 and 24 patients, respectively. Neurofibroma was present in 22 cases. Ten of the cohort had optic gliomas, and 39 of them had cranial hamartomas. Orthopedic complications such as scoliosis, tibial pseudoarthrosis, and osteoporosis were observed in 13 patients. Eleven children had neurocognitive disorders. CONCLUSIONS: Early diagnosis is important in neurofibromatosis to prevent the complications of the disease. Also, neurological development and secondary malignancy follow-up should be done carefully in this group of patients.

Outcomes of Eltrombopag Treatment and Development of Iron Deficiency in Children with Immune Thrombocytopenia in Turkey

Objective: Immune thrombocytopenia (ITP) is a rare autoimmune disease and hematologic disorder characterized by reduced platelet counts that can result in significant symptoms, such as bleeding, bruising, epistaxis, or petechiae. The thrombopoietin receptor agonist eltrombopag (EPAG) is a second-line agent used to treat chronic ITP purpura in adults and children. Materials and Methods: The present retrospective study evaluated the efficacy, safety, and side effects of EPAG treatment in pediatric patients with acute refractory and chronic immune thrombocytopenia, particularly focusing on iron-deficiency anemia. Results: The diagnosis was chronic ITP in 89 patients and acute refractory ITP in 16 patients. The mean age of patients was 9.5±4.5 years (minimum-maximum: 1.2-18 years) at the beginning of EPAG treatment. The overall response rate was 74.3% (n=78). The mean time for platelet count of ≥50x109/L was 11.6±8 weeks (range: 1-34 weeks). The treatment was stopped for 27 patients (25.7%) at an average of 6.8±9 months (range: 1-38 months). The reason for discontinuation was lack of response in 18 patients, nonadherence in 4 patients, and hepatotoxicity in 2 patients. Response to treatment continued for an average of 4 months after cessation of EPAG in 3 patients. Conclusion: Results of the current study imply that EPAG is an effective therapeutic option in pediatric patients with acute refractory and chronic ITP. However, patients must be closely monitored for response and side effects during treatment, and especially for iron deficiency.