A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O), so called TAFRO syndrome, was first reported in 2010.1 Considered a variant of idiopathic multicentric Castleman’s disease, the recent discovery and rarity of this syndrome poses significant challenges to diagnosis and management. In this case report, we review the youngest patient to be reported in the literature with TAFRO syndrome. We highlight the patient’s diagnosis, management, and follow-up in the context of current recommendations for treatment and management of TAFRO syndrome.

Introduction: Many children and adolescents who were vaccinated prior to cancer treatment lose humoral immunity after completion of therapy. Pediatricians and pediatric oncologists often recommend re-immunization, although there is little consensus on timing and approach to serologic testing. However, vaccine hesitancy in the U.S. is a growing problem. It is not known whether parents who initially permitted vaccination might demonstrate secondary hesitancy regarding re-immunization. Methods: We conducted a qualitative study to explore parental attitudes toward re-immunization after completion of cancer therapy. Twenty primary caregivers of current pediatric cancer patients participated in structured interviews exploring knowledge and understanding of immunity and vaccination; previous experiences with vaccines; and attitudes toward vaccines and revaccination. Results: Of those interviewed, 80% were female and 90% were White Non-Hispanic. Of interviewees’ children with cancer, 60% were male, 75% had been diagnosed within the past 6 months, and 45% had leukemia or lymphoma. All caregivers demonstrated a basic understanding of vaccination, but only 65% understood that it was possible to lose immunity even with previous vaccination. All caregivers were willing to have their children immunized if tests showed lack of humoral immunity, with 85% expressing a preference for testing prior to revaccination. Conclusions: Primary caregivers of children with cancer are willing to consider re-immunization interest but do express some secondary hesitancy and strongly prefer that the need for re-immunization be established via serologic testing, rather than performed empirically. Caregivers’ beliefs and preferences regarding re-immunization in pediatric oncology should be considered in the development of post-treatment guidelines.

Severe congenital neutropenia (SCN) is a rare disorder, often due to pathogenic variants in genes such as ELANE, HAX1, and SBDS. SRP54 pathogenic variants are associated with SCN and Shwachman‐Diamond‐like syndrome. Thirty‐eight patients with SRP54‐related SCN are reported in the literature. We present an infant with SCN, without classic Shwachman‐Diamond syndrome features, who presented with recurrent bacterial infections and an SRP54 (c.349_351del) pathogenic variant. Despite ongoing granulocyte colony‐stimulating factor therapy, this patient has no evidence of malignant transformation. Here we establish a framework for the future development of universal guidelines to care for this patient population.

Severe congenital neutropenia (SCN) is a rare disorder, often due to pathogenic variants (PV) in genes such as ELANE, HAX1, and SBDS.SRP54 PVs are associated with SCN and Shwachman-Diamond-like syndrome. Thirty-eight patients with SRP54-related SCN are reported in the literature. We present an infant with SCN, without classic Shwachman-Diamond syndrome features, who presented with recurrent bacterial infections and an SRP54 (c.349_351del) PV. Despite ongoing granulocyte colony-stimulating factor (G-CSF) therapy, there has been no evidence of malignant transformation. Here we establish a framework for future development of universal guidelines to care for this patient population.