COVID-19 in unvaccinated patients with inborn errors of immunity-polish experience.

At the beginning of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic, patients with inborn errors of immunity (IEI) appeared to be particularly vulnerable to a severe course of the disease. It quickly turned out that only some IEI groups are associated with a high risk of severe infection. However, data on the course of Coronavirus Disease 2019 (COVID-19) in patients with IEI are still insufficient, especially in children; hence, further analyses are required. The retrospective study included 155 unvaccinated people with IEI: 105 children and 50 adults (67.7% and 32.3%, respectively). Male patients dominated in the study group (94 people, 60.6%). At least two comorbidities were found in 50 patients (32.3%), significantly more often in adults (56% vs. 21%). Adult patients presented significantly more COVID-19 symptoms. Asymptomatic and mildly symptomatic course of COVID-19 was demonstrated in 74.8% of the entire group, significantly more often in children (88.6% vs. 46%). Moderate and severe courses dominated in adults (54% vs. 11.4%). Systemic antibiotic therapy was used the most frequently, especially in adults (60% vs. 14.3%). COVID-19-specific therapy was used almost exclusively in adults. In the whole group, complications occurred in 14.2% of patients, significantly more often in adults (30% vs. 6.7%). In the pediatric group, there were two cases (1.9%) of multisystem inflammatory syndrome in children. Deaths were reported only in the adult population and accounted for 3.9% of the entire study group. The death rate for all adults was 12%, 15.4% for adults diagnosed with common variable immunodeficiency, 12.5% for those with X-linked agammaglobulinemia, and 21.4% for patients with comorbidity. The results of our study imply that vaccinations against COVID-19 should be recommended both for children and adults with IEI. Postexposure prophylaxis and early antiviral and anti-SARS-CoV-2 antibody-based therapies should be considered in adults with IEI, especially in those with severe humoral immune deficiencies and comorbidity.

Bone marrow aplasia following donor lymphocyte infusion in 4-year-old patient with chronic granulomatous disease after allogeneic stem cell transplantation: case report.

Donor lymphocyte infusion (DLI) is typically used in 3 clinical situations: therapeutically for proven relapse of malignancy, prophylactically in patients with high-risk of relapse, and in case of mixed chimerism. Mixed chimerism, which occur after transplantation can be a sign of possible rejection. In case of increased mixed chimerism, immunotherapy with donor lymphocyte infusions could reverse this process. After DLI, both acute and chronic graft-versus-host disease and marrow aplasia are well-known toxicities. In this paper, we present a case report of young patient with chronic granulomatous disease (CGD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT), with successful immunotherapy following mixed chimerism, which was complicated by bone marrow aplasia that required a second stem cell infusion. DLI seems to be an effective and highly promising treatment method of transplant rejection in patients with CGD but can induce bone marrow aplasia and may require a second stem cell infusion.

Influence of Mixed Chimerism on Outcome in Children With Anaemia After Haematopoietic Stem Cell Transplantation.

BACKGROUND/AIM: In patients with non-malignant diseases, mixed chimerism is not a rare phenomenon. The clinical impacts of chimerism following allogeneic haematopoietic stem cell transplantation (allo-HSCT) in children with congenital anaemia (CA) and severe aplastic anaemia (SAA) were analysed. PATIENTS AND METHODS: We studied twenty-seven consecutive children with congenital and acquired anaemia who had undergone allogeneic haematopoietic stem cell transplantations. In the observed group of patients, the median of the follow-up was 6.12 years (2.00-14.8 years). RESULTS: Overall survival (OS) did not depend on the type of disease p=0.1. OS did not significantly differ in patients who received more than 5x106/kg stem cells (91%) and those who received less than 5x106/kg (85%) (p=0.61). Two patterns of stable mixed chimerism (SMC) were observed: SMC (95-97% cells of the donor), and SMC with a fluctuation between 50-90% of the cells of the donor. None of the surviving patients received immunosuppression treatments of chronic Graft-versus-Host Disease (cGvHD). CONCLUSION: Our results showed that mixed chimerism did not influence the survival of children with congenital and aplastic anaemia following allo-HSCT.