HSCT (hematopoietic stem cell transplantation) is a widely applied method of treatment of pediatric patients with leukemia and other bone marrow-associated disorders. Metabolic disturbances can appear as procedure side effects. This study aimed to report incidence of lipid and thyroid disorders and time of their onset in pediatric patients after HSCT. There were 198 pediatric patients (123 males) aged 0.5–20 years who were subjected to HSCT. Patients were mostly diagnosed with Acute Leukemia (n = 190). The analysis of lipids, thyroid hormones, and thyroid antibodies levels comprised one month before the HSCT to last follow up visit between 2016 and 2019 (median 3.8 ± 1.8 years after HSCT). In males, the triglycerides levels increased over two times in the course of HSCT in both patients with initially low and elevated HDL (high-density lipoprotein) levels. Most of the lipid disorders occurred in six months after HSCT. Patients treated with L-thyroxine exhibited decreased LDL (low-density lipoprotein) levels. HDL remained at a lower level in males. Thyroid hormone abnormalities were evenly distributed in time until 4 years after HSCT. Patients require long term follow up including lipid metabolism and thyroid function analysis. HSCT survivors demand introduction of polyunsaturated fatty acids into the diet to reduce risk of developing the lipid complications.
Background: Apheresis in children with low body weight is technically limited by their tolerance of the extracorporeal blood volume. Study design and methods: This paper presents a single-center experience with 23 procedures in 12 children with weights between 5.2 and 9.5 kg using the Spectra Optia mononuclear cell (MNC) protocol with blood priming. Results: The average procedure duration was 158 minutes, and the median processed blood volume was 316 mL/kg. The white blood cell (WBC), platelet (PLT), and hemoglobin (HGB) values showed a downward trend with increased volume of processed blood. The post-apheresis HGB concentration was increased in all procedures due to initial priming with packed red blood cells (PRBCs), but this effect disappeared at a level of~400 mL of processed blood/kg. The median volume of the cellular product was 36 mL, the WBC count was 153 K/μL, the hematocrit (HCT) was 1.5%, the PLT count was 602 K/μL, the WBC collection efficacy (CE2) was 13.2%, and the PLT CE2 was 9.5%. The median CD34+ CE2 was 28%, and interpolation of the CD34+ CE2 yielded a Y-intercept value of 32%.Higher pre-collection CD34+ counts resulted in higher CD34+ yields. No correlation was found between the pre-collection CD34+ results and CD34+ CE2. CONCLUSION: The analyzed data demonstrated the feasibility and safety of apheresis in very low-weight children. The laboratory abnormalities were asymptomatic and citrate toxicity was mild. Visual control of clogging with manual adjustment of the citrate infusion rate is important to reduce exposure to citrate. K E Y W O R D S apheresis, low-weight, pediatric
Allo‐HSCT is associated with life‐threatening complications. Therefore, a considerable number of patients require admission to a PICU. We evaluated the incidence and outcome of PICU admissions after allo‐HSCT in children, along with the potential factors influencing PICU survival. A retrospective chart review of 668 children who underwent first allo‐HSCT in the Department of Pediatric Hematology/Oncology and BMT in Wrocław during years 2005‐2017, particularly focusing on patients admitted to the PICU within 1‐year post‐HSCT. Fifty‐eight (8.7%) patients required 64 admissions to the PICU. Twenty‐four (41.5%) were discharged, and 34 (58.6%) patients died. Among the discharged patients, 6‐month survival was 66.7%. Compared with survivors, death cases were more likely to have required MV (31/34; 91.2% vs. 16/24; 66.7% P = .049), received more aggressive cardiac support (17/34; 50% vs. 2/24; 8.3% P = .002), and had a lower ANC on the last day of their PICU stay (P = .004). Five patients were successfully treated with NIV and survived longer than 6 months post‐discharge. The intensity of cardiac support and ANC on the last day of PICU treatment was independent factors influencing PICU survival. Children admitted to the PICU after allo‐HSCT have a high mortality rate. Mainly those who needed a more aggressive approach and had a lower ANC on the last day of treatment had a greater risk of death. While requiring MV is associated with decreased PICU survival, early implementation of NIV might be considered.
Ataxia-telangiectasia (A-T) syndrome is an autosomal recessive chromosomal breakage syndrome caused by mutation of the ataxia-telangiectasia mutated gene manifested by progressive neurodegeneration, telangiectasias of sclera and skin, immune deficiency with sinopulmonary infections, and increased incidence of lymphoid malignancies and solid tumors. Three children with AT underwent allogeneic stem cell transplantation (SCT) using protocols for Fanconi anemia. All 3 patients were engrafted with a mixed donorÀrecipient chimerism, but the full donor engraftment was observed in the T lymphocyte compartment. Immunologic recovery resulted in T cell production and lack of symptomatic infections. Regular intravenous immunoglobulin supplementation was needed until IgG production recovered, which depended on pretransplant serotherapy. During the observation period patients did not require hospital admission, and none of the transplanted patients developed sinopulmonary infections. Neurologic functions in reported patients were impaired and slowly deteriorated after transplantation, but no immediate toxicities were observed. The following hallmark features of AT were present after SCT: neurologic symptoms, growth failure, telangiectasia formation, or increased serum alpha fetoprotein. SCT can help control immune deficiency constituting 1 of the features of AT , and elimination of autologous hematopoiesis reduces the risk of lymphoid malignancies. Resolving crucial problems with qualification for SCT depends on balancing the risk and benefits of transplant therapy.
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