SARS-CoV-2 Infection Presenting as Acute Chest Syndrome in a Child With Hemoglobin SD-Los Angeles Disease: A Case Report and Review of Literature.

Hemoglobin D-Los Angeles is a variant of hemoglobin that can polymerize in the deoxygenated state. When co-inherited with Hemoglobin S (HbSD-Los Angeles disease) a severe sickling syndrome similar to HbSS can result. Corona virus infectious disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome-corona virus-2. It has been associated with acute chest syndrome (ACS) in individuals with sickle cell disease (SCD), but this complication has not previously been reported in patients with HbSD-Los Angeles. Dexamethasone has been shown to improve outcomes in non-SCD patients with severe acute respiratory syndrome-corona virus-2 pneumonia or acute respiratory distress syndrome; however, its use in SCD patients with ACS is controversial due to a reported increased risk of complications including vaso-occlusive painful episodes. Herein, we reported a patient with HbSD-Los Angeles and COVID-19-associated ACS whom we treated with dexamethasone without transfusion. The patient experienced a rapid recovery without sequelae from steroid use. To further evaluate the use of steroids, we conducted a literature review focusing on the management of pediatric SCD patients with COVID-19-associated ACS. We identified a total of 39 pediatric patients with SCD and COVID-19, of whom 21 (54%) had ACS. Packed red blood cell transfusion (n=11), exchange transfusion (n=4), or a combination of exchange transfusion and packed red blood cell transfusion (n=4) were the most frequently reported treatment, with hydroxychloroquine (n=5), remdesivir (n=1), and tocilizumab (n=1) also being reported. Three patients were treated with dexamethasone. All patients recovered and no adverse outcomes from steroid use were reported. Even though transfusion is considered the standard of care for children with ACS and steroids are not routinely recommended, our experience suggested that COVID-19-associated ACS may be an important exception, especially for patients who refuse transfusion or are in resource-poor nations where blood transfusions may not be readily available. Further studies are warranted to confirm these observations.

Mammalian Target of Rapamycin Inhibitor Induced Complete Remission of a Recurrent Subependymal Giant Cell Astrocytoma in a Patient Without Features of Tuberous Sclerosis Complex.

The majority of patients with subependymal giant cell astrocytoma (SEGA) have tuberous sclerosis complex (TSC). In such patients, the mammalian target of rapamycin (mTOR) inhibitor everolimus has been shown to induce responses. Isolated SEGA have been reported in patients without clinical or genetic features of TSC. The treatment of these patients with everolimus has not previously been reported. We treated a patient with a recurrent isolated SEGA with an mTOR inhibitor. The patient tolerated therapy well and had a sustained complete remission. MTOR inhibitors may be useful for the treatment of isolated SEGA. Further study is warranted.

Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms.

Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement.

Transfusion related iron overload in pediatric oncology patients treated at a tertiary care centre and treatment with chelation therapy.

We conducted a retrospective chart review to determine prevalence of, risk factors for, and liver toxicity associated with Transfusion Related Iron Overload (TRIO) in pediatric cancer patients, and report our experience with Iron Chelation Therapy (ICT). Total number of transfusions was identified as the major risk factor, with a prevalence of 37% in patients receiving ≥10 transfusions. Four patients with TRIO and abnormal liver function tests (LFT) received ICT. Significant decrease in serum ferritin and improvement in LFT were observed, with no serious adverse effects from ICT noted. Guidelines for screening and treatment of TRIO in pediatric oncology are needed.

High-risk neuroblastoma in Ontario: a report of experience from 1989 to 1995.

PURPOSE: We did a population-based study of children with high-risk neuroblastoma to determine their survival and look for factors that had an impact on survival. METHODS: We carried out a retrospective cohort study of patients diagnosed in Ontario from 1989 to 1995. 162 cases of neuroblastoma were diagnosed in the province with 70 (43%) considered high-risk: all were older than one year of age, with 15 patients classified as International Neuroblastoma Staging System (INSS) stage 3, and 55 INSS stage 4. RESULTS: Stage 3 patients did significantly better than Stage 4 patients with a 5-year overall survival of 67.7% and 22.7% respectively (P = 0.015). In stage 4 patients achieving at least a partial response to up-front therapy and surviving for at least 9.5 months after diagnosis (the median time to transplant), there was no difference in survival between the 19 transplant patients and the 17 treated with chemotherapy alone (P = 0.75). However, patients transplanted by peripheral stem cell (PSC) collection did significantly better than both the bone marrow transplantation (P = 0.002) and chemotherapy-alone group (P = 0.047). There was a significant difference in up-front chemotherapy and use of radiation in the three groups (P < 0.001 and P = 0.01 respectively), but no difference in the incidence of bone and bone marrow metastases, MYCN amplification or unfavorable histology. CONCLUSIONS: In this nonrandomized study, we found that stage 4 neuroblastoma patients alive more than 9.5 months after diagnosis, with at least a partial response to initial therapy, did significantly better with PSC transplant compared with bone marrow transplantation or chemotherapy alone.