Engraftment after hematopoietic stem cell transplantation is the recovery rate of neutrophils and platelets. This study aimed to test the impact of the patient's general characteristics, pre-transplantation factors, and quality parameters of hematopietic stem cell products on hematopietic recovery and to define predictive factors for engraftment in children. This retrospective study included 52 patients aged from 1 to 18 years old treated with autologous transplantation at the Mother and Child Health Care Institute of Serbia "Dr. Vukan Čupić" in Belgrade, from January 2013 until December 2018. Data were collected from medical records and apheresis procedure protocols. SPSS 20.0 software package was used for statistical data processing. The median neutrophil engraftment was 18.0 (16.0-22.5) days, while the median platelet engraftment was 11.0 (10.0-18.0) days. Statistically significant correlations were found between neutrophil engraftment and patient's age (p-value = 0.050), body weight (p-value = 0.021), diagnosis (p-value = 0.023), source of stem cells (p-value = 0.001), and the number of CFU-GM/kg (p-value = 0.018). A statistically significant correlation was found between platelet engraftment and the time from diagnosis to the transplantation (p-value = 0.043), source of stem cells (p-value = 0.009), and the number of CD34+ cells/kg (p-value = 0.014). Predictive factors for hematopoietic recovery in this study were the patient's age, body weight, diagnosis, time from diagnosis to hematopoietic stem cell transplantation, source of hematopietic stem cells, the number of CD34+ cells/kg, and the number of CFU-GM/kg.
1 April 2020 in Transfusion Clinique et Biologique
Paediatric patients with the syndrome of an inappropriate antidiuretic hormone secretion (SIADH), as a manifestation of inflammatory demyelinating disorders of the central nervous system, have been rarely described until now, in only a few cases of neuromyelitis optica spectrum disorders (NMOSDs). We present a case of relapsing SIADH associated with NMOSD, in an anti-aquaporin-4 antibody positive 14-year-old girl, who is, to our best knowledge, the first reported paediatric patient with relapsing SIADH and NMOSD. Additionally, our case further supports the notion that paediatric encephalomyelitis associated with SIADH should suggest the diagnosis of NMOSD.
Introduction. The group of autosomal dominant disorders - Epstein syndrome, Sebastian syndrome, Fechthner syndrome and May-Hegglin anomaly - are characterised by thrombocytopenia with giant platelets, inclusion bodies in granulocytes and variable levels of deafness, disturbances of vision and renal function impairment. A common genetic background of these disorders are mutations in MYH9 gene, coding for the nonmuscle myosin heavy chain IIA. Differential diagnosis is important for the adequate treatment strategy. The aim of this case report was to present a patient with MYH9 disorder in Serbia. Case report. A 16-year-old boy was referred to our hospital with the diagnosis of resistant immune thrombocytopenia for splenectomy. Thrombocytopenia was incidentally discovered at the age of five. The treatment with corticosteroids on several occasions was unsuccessful. Although the platelet count was below 10 ? 109/L, there were no bleeding symptoms. Besides thrombocytopenia with giant platelets, on admission the patient also suffered sensorineuronal hearing loss and proteinuria. The diagnosis was confirmed with immunofluorescence and genetic analyses. Conclusion. Early recognition of MYH9-related diseases is essential to avoid unnecessary and potentially harmful treatments for misdiagnosed immune thrombocytopenia, and also for timely and proper therapy in attempt to delay end-stage renal failure and improve quality of life.