Background Childhood cancer is a highly curable disease when timely diagnosis and appropriate therapy are provided. A negative impact of the coronavirus disease 2019 (COVID‐19) pandemic on access to care for children with cancer is likely but has not been evaluated. METHODS A 34‐item survey focusing on barriers to pediatric oncology management during the COVID‐19 pandemic was distributed to heads of pediatric oncology units within the Pediatric Oncology East and Mediterranean (POEM) collaborative group, from the Middle East, North Africa, and West Asia. Responses were collected on April 11 through 22, 2020. Corresponding rates of proven COVID‐19 cases and deaths were retrieved from the World Health Organization database. Results In total, 34 centers from 19 countries participated. Almost all centers applied guidelines to optimize resource utilization and safety, including delaying off‐treatment visits, rotating and reducing staff, and implementing social distancing, hand hygiene measures, and personal protective equipment use. Essential treatments, including chemotherapy, surgery, and radiation therapy, were delayed in 29% to 44% of centers, and 24% of centers restricted acceptance of new patients. Clinical care delivery was reported as negatively affected in 28% of centers. Greater than 70% of centers reported shortages in blood products, and 47% to 62% reported interruptions in surgery and radiation as well as medication shortages. However, bed availability was affected in <30% of centers, reflecting the low rates of COVID‐19 hospitalizations in the corresponding countries at the time of the survey. Conclusions Mechanisms to approach childhood cancer treatment delivery during crises need to be re‐evaluated, because treatment interruptions and delays are expected to affect patient outcomes in this otherwise largely curable disease.

Iron-refractory iron-deficiency anemia (IRIDA) is a rare autosomal-recessive disorder characterized by hypochromic microcytic anemia, low transferrin saturation, and inappropriate high levels of the iron hormone hepcidin. The disease is caused by variants in the transmembrane protease serine 6 (TMPRSS6) gene that encodes the type II serine protease matriptase-2, a negative regulator of hepcidin transcription. Sequencing analysis of the TMPRSS6 gene in 21 new IRIDA patients from 16 families with different ethnic origin reveal 17 novel mutations, including the most frequent mutation in Southern Italy (p.W590R). Eight missense mutations were analyzed in vitro. All but the p.T287N variant impair matriptase-2 autoproteotylic activation, decrease the ability to cleave membrane HJV and inhibit the HJV-dependent hepcidin activation. Genotype-phenotype studies in IRIDA patients have been so far limited due to the relatively low number of described patients. Our genotype-phenotype correlation analysis demonstrates that patients carrying two nonsense mutations present a more severe anemia and microcytosis and higher hepcidin levels than the other patients. We confirm that TMPRSS6 mutations are spread along the gene and that mechanistically they fully or partially abrogate hepcidin inhibition. Genotyping IRIDA patients help in predicting IRIDA severity and may be useful for predicting response to iron treatment.

Our results indicate that AFP with fluconazole and early empirical antifungal therapy may be effective in reducing the incidence and mortality of IFI in children with acute leukemia.

Our study suggests that hepcidin levels do not contribute to the development of iron deficiency anemia in pediatric obese individuals.

Our data suggest that disseminated intravascular coagulation score greater than or equal to 5 can be used in the definition of high-risk patients. Early recognition of poor risk factors has important prognostic and therapeutic implications.

Chediak Higashi syndrome (CHS) is an autosomal-recessive disorder characterized by oculocutaneous albinism, recurrent infections and a progressive primary neurological disease. Here, we describe two siblings with CHS due to a novel homozygous R1836X mutation in the LYST gene associated with loss of NK cell degranulation and cytotoxicity. While one sibling was born with fair skin and hair and died of hemophagocytic lymphohistiocytosis (HLH) at 5 months of age, the other sibling had dark black hair and skin and developed HLH at the age of 4 years.

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There is growing interest in developing criteria that will allow efficient prospective discrimination between cancer patients at high and at low risk for complex fever and neutropenia. The objective of this study was to determine whether there were differences in patterns of documented infections and outcome of episodes of fever and neutropenia in pediatric patients with leukemia and those with solid tumors, a potential risk factor. A total of 283 febrile neutropenia episodes in pediatric cancer patients at a single center were retrospectively reviewed; 38% of the patients concerned had leukemia and 62% had solid tumors. Fever of unexplained origin was seen in 73% and 74% of episodes in patients with leukemia and solid tumor, respectively. Bacteremia occurred in 18% and 16% of patients in these respective groups. There was no difference in the type of microorganisms that were isolated in the groups, with gram positives predominating in both. The median duration of fever was 2 days in both groups. The depth of neutropenia was similar, with 75% of leukemia patients and 70% of solid tumor patients presenting with ANC of 100 cells/microl or lower. The median duration of neutropenia was 9 days in patients with leukemia and 6 days in solid tumor patients. The median duration of antibiotic treatment was 9 days and 7.5 days in the same respective groups. Antibiotic modification occurred in 25% episodes of febrile neutropenia in leukemia patients and in 11% of episodes in solid tumor patients. No deaths occurred in either group. Subgroup analysis of leukemic patients suggested that patients in the induction phase of therapy have a higher rate of bacteremia and pneumonia. No substantial difference in course or outcome was seen between the leukemia and solid tumor groups, possibly because of the intensive treatment administered to pediatric patients with solid tumors. Risk assessment strategies based on chemotherapy dose intensity and patient comorbidities rather than underlying malignancy should be prospectively studied.