Primary intramedullary spinal cord lymphoma (PISCL) is a rare cause of myelopathies. As PISCL is often underrecognized, delaying appropriate treatment, we sought to describe its presentation. We report two clinical cases of pediatric patients. The diagnosis of PISCL must be considered in a patient with symptoms of acute myelopathies.

Background: Music therapy (MT) is a complementary therapy offered to children, young adults and their families in pediatric oncology and palliative care. We performed a survey to collect information about MT in pediatric oncology in Italy. The outbreak of COVID-19 unavoidably changed the scenario of MT, suggesting some considerations presented in this survey. Methods: 27/32 (84,4%) Centres belonging to the Italian Association of Pediatric Hematology and Oncology (AEIOP) completed in 2 different time points (T1 and T2) an online survey on MT, before and after Covid-19 pandemia. Results: Different kinds of music approach were used taking care of patients in 21/27 centers, while in 14/21 (66%) a specific project of MT conducted by a music therapist was present. In 6/14 Centres MT activities were delivered for <3 hours/week, in 3 Centres for >3 and <10 hours/week, in the remaining 5 for >3 hours/week. MT sessions were in different areas, day hospital or ward (patient rooms, operating rooms, waiting rooms), on an individual basis or by groups. Patients were invited to MT by psychologists, caring physician or nurse, or on equipé decision. MT was evaluated with tools self-made by Music therapist in 11/14 centres. After Covid-19. MT has been withdrawal in 3 centres, sessions in the waiting rooms were reduced, individual sessions were preferred and enrollment by multidisciplinary teams increased. Conclusion: This survey represents the starting platform to compare and discuss different experience of MT in AIEOP Centres, to implement MT in pediatric oncology for a more qualified assistance to patients and to improve quality of care.

Purpose. To describe ecthyma gangrenosum (EG) characteristics and complications in a large multicenter pediatric retrospective collection of children with malignancies or bone marrow failure syndromes. Methods. EG episodes diagnosed in the period 2009-2019 were identified by a retrospective review of clinical charts at centers belonging to the Italian Pediatric Hematology Oncology Association. Results. EG occurred in 38 children (male/female 16/22; median age 5.2 years) with hematological malignancy (33), allogeneic stem cell transplantation (2) or relapsed/refractory solid tumor (3). The involved sites were: perineal region (19), limbs (10), trunk (6), head and the iliac crest (3). Bacteremia was present in 22 patients. Overall, the germs isolated were Pseudomonas aeruginosa (34), Stenotrophomonas maltophilia (3) and Escherichia Coli (1); 31% of them were MDR. All patients received antibacterial treatment while surgery was performed in 24 patients (63.1%). Predisposing underlying conditions for EG were: severe neutropenia (97.3%), corticosteroid treatment (71%), iatrogenic diabetes (23.7%). All patients recovered, but EG recurred in 5 patients. Nine patients (24%) showed sequelae (deep scars, with muscle atrophy in 2). Four patients (10.5%) died, 1 due to relapse of EG with KPC co-infection, 3 due to the underlying disease. Conclusions. EG requires early recognition and a proper and timely treatment to obtain the recovery and to avoid larger necrotic evolution. The occurrence of scarring sequelae might affect the quality of life of patients.

Background Immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA) and autoimmune neutropenia (AIN) are disorders characterized by immune-mediated destruction of hematopoietic cell lineages. A link between pediatric immune cytopenias and inborn errors of immunity (IEI) was established in particular in the combined and chronic forms. Objective Identification of predictive factors of IEI in immune cytopenic children is an important objective for a prompt immunological diagnosis and appropriate management. Aim of this study is to detect clinical and laboratory signs of IEI, in particular the latter by an extensive lymphocyte immunophenotyping. Methods We retrospectively collected 47 pediatric patients with at least one hematological disorder among which persistent/chronic ITP, AIHA and AIN, aged 0–18 years at onset of immune cytopenias and/or immune-dysregulation. The cohort was divided into 2 groups (IEI+ and IEI-), based on the presence/absence of underlying IEI diagnosis. IEI+ group, formed by 19/47 individuals, included: Common variable immune deficiency (9/19), Autoimmune lymphoproliferative syndrome (4/19), DiGeorge syndrome (1/19) and unclassified IEI (5/19). Results IEI prevalence among patients with ITP, AIHA, AIN and Evans Syndrome was respectively of 42%, 64%, 36% and 62%. In IEI+ the extended lymphocyte immunophenotyping identified the presence of statistically significant (p-value<0.05) specific characteristics, namely T/B lymphopenia, decrease in naїve T-cells%, switched memory B-cells%, plasmablasts% and/or immunoglobulins, increase in effector/central memory T-cells% and CD21low B-cells%. Conclusion A wide focused clinical/immunophenotypical characterization of pediatric patients with immune cytopenia can highlight specific signs of IEI, potentially helpful in the diagnostic and clinical management, identifying children worthy of IEI-related molecular analysis.