The understanding of Coronavirus disease 2019 (COVID-19) immune dysregulation is evolving. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with alternations in both innate and adaptive immunity, probably caused by a complex interplay of genetics and environmental exposure with various triggers. A rare hematological complication of SLE as well as recently reported in adult with COVID-19 is thrombotic thrombocytopenic purpura (TTP). We report a pediatric case with features suggestive of multisystem inflammatory syndrome in children (MIS-C) with coronary artery ectasia, TTP, autoimmune hemolytic anemia (AIHA) and thrombocytopenia with new onset SLE as well.

Retinoblastoma is the most common ocular tumor of childhood with cure rates exceeding 95%. Patients with high-risk features typically relapse within 3 years of diagnosis. We report a patient with low-risk bilateral retinoblastoma who suffered systemic relapse after eight years. His disease at first relapse was chemosensitive without PET avidity or bone marrow disease following therapy. Six months later, he experienced an isolated CNS relapse and succumbed to refractory disease. “Oncoseq” exome sequencing confirmed the presence of germline RB mutation among all tissues as well as somatic changes which may provide insights into the biology of relapse and tumor.

Non Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) are rare in the pediatric age group, accounting for 1-7% of all pediatric tumors. Leiomyosarcoma, a subtype of NRSTS, is exceedingly rare in pediatric population. Due to the rarity of this condition, management is extrapolated from other common NRSTS which involves surgery, chemotherapy and radiation. Chemotherapy is not very effective in management of pediatric leiomyosarcoma and molecular information may help guide targeted therapies. We describe a patient with a FGFR1-TACC1 gene rearrangement which, based on other models, predicts for sensitivity to FGFR inhibitors.

Treatment of refractory Evans syndrome (ES) remains a challenge in Hematology practice. Due to rarity of this condition, evidence-based approaches are limited and often treatment choices stem from small case series or anectodal experiences. Here, we describe three very refractory pediatric ES cases treated on bortezomib without adverse effects. Two of the three patients had dramatical and long-lasting recovery that started following the first doses of the drug. Clinical trials to assess bortezomib role in ES treatment are warrented and results may lead to inclusion of this drug as an option, even as a first-line therapy.