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Response to rituximab in children and adults with immune thrombocytopenia (ITP)

Harris, Emily M; Hillier, Kirsty; Al-Samkari, Hanny; Berbert, Laura; Grace, Rachael F
Background/UNASSIGNED:Rituximab is a monoclonal anti-CD20 antibody used as a second-line treatment for immune thrombocytopenia (ITP). As additional treatments for ITP emerge, identifying the most appropriate patients and optimal timing for rituximab are important but challenging without established predictors of response to therapy. Objectives/UNASSIGNED:The purpose of this study was to describe demographic, clinical, and laboratory characteristics of pediatric and adult patients with ITP to identify differences in evaluation before rituximab administration and correlates of platelet response. Methods/UNASSIGNED:This is a retrospective cohort study describing the characteristics of patients with ITP treated with rituximab from 2010 to 2020 at two academic tertiary care centers. Results/UNASSIGNED:A total of 64 patients met criteria for inclusion. Complete rituximab response (56%) was not significantly different between children (58%, n = 24) and adults (55%, n = 40). Response rate was similar in those with primary versus secondary ITP (53% vs 62%). Among patients treated with rituximab, Evans Syndrome was more common in children than adults (42% vs 18%). Immunologic labs assessed before rituximab varied by age and were more commonly evaluated in children (lymphocyte subsets 88% vs 22%). Immunologic markers, including antinuclear antibody, direct antiglobulin testing, immunoglobulin levels, and lymphocyte subsets, did not predict response to rituximab in pediatric or adult patients with ITP. Conclusions/UNASSIGNED:Pre-rituximab immunologic evaluation varied significantly between adults and children, which could represent institution-specific practice patterns or a more general practice difference. If the latter, underlying immunodeficiency in adults with ITP may be underrecognized. Standardized guidance for pre-rituximab immunologic evaluation is needed.
PMID: 34466770
ISSN: 2475-0379
CID: 5037032

Factors Impacting Time to Engraftment in Patients With High-risk Neuroblastoma Following Autologous Stem Cell Transplant

Hillier, Kirsty; Cheng, W Susan; Whittle, Sarah B; Krance, Robert; Foster, Jennifer H
BACKGROUND:Despite advances in supportive measures, myeloablative chemotherapy with stem cell rescue remains limited by toxicity and treatment-related mortality. The purpose of this study was to identify factors influencing the rate of hematopoietic recovery following autologous stem cell transplant in high-risk neuroblastoma. PROCEDURE:We retrospectively studied 54 patients with high-risk neuroblastoma who received a single autologous stem cell transplant between 2006 and 2016. Race, sex, conditioning regimen, chemotherapy delays and bone marrow involvement were analyzed using Kaplan-Meier Log-Rank test while the amount of cells infused, age, and length of hospital stay were analyzed using univariate Cox Proportional Hazards Regression. RESULTS:The conditioning regimen administered was significant (P=0.016) for time to engraftment of neutrophils, with busulfan/melphalan (Bu/Mel) at 16.6 days, and carboplatin/etoposide/melphalan at 12.1 days. A delay of chemotherapy during induction (n=24) was significant (P<0.001) for time to platelet engraftment of >75,000/µL. Female patients had a longer time to engraftment (P=0.029). CONCLUSION:Patients receiving Bu/Mel as a conditioning regimen, patients who had a delay in induction chemotherapy and patients of female sex were found to be significant for delayed engraftment of neutrophils, platelets, and hemoglobin, respectively, in patients with high-risk neuroblastoma undergoing autologous stem cell transplant. Knowing these factors may lead to new expectations and possible interventions to decrease the morbidity and mortality of treatment and recovery.
PMID: 32032244
ISSN: 1536-3678
CID: 4966582

A Novel ALK Fusion in Pediatric Medullary Thyroid Carcinoma [Case Report]

Hillier, Kirsty; Hughes, Amy; Shamberger, Robert C; Shusterman, Suzanne; Perez-Atayde, Antonio R; Wassner, Ari J; Iafrate, Anthony John; Dubuc, Adrian; Janeway, Katherine A; Rothenberg, S Michael; Cox, Michael C; Randolph, Gregory W; Wirth, Lori J; Tsai, Harrison; Church, Alanna; DuBois, Steven G
Medullary thyroid carcinoma (MTC) is most commonly associated with RET gene mutations. ALK fusions have rarely been described, although not previously in pediatrics and not previously partnered with CCDC6 in MTC or any other cancer. A 10-year-old boy with progressive stridor was found to have metastatic MTC, including lung, lymph node, and adrenal metastases. Baseline calcitonin was 6703 pg/mL. While molecular testing was pending, he was treated empirically with the investigational selective RET inhibitor, LOXO-292, without improvement. Molecular testing revealed a novel CCDC6-ALK fusion. His therapy was changed to crizotinib and then to alectinib for improved tolerability. Calcitonin decreased to 663 pg/mL after 6 days of ALK inhibition. He remains on alectinib with ongoing response. A novel CCDC6-ALK fusion has now been implicated in a pediatric case of metastatic MTC. This fusion has profound clinical sensitivity to ALK inhibitors. This report expands the spectrum of ALK fusions seen in MTC, including the first pediatric case of ALK translocated MTC. This novel fusion with CCDC6 has not previously been reported in other human cancers. Given the dramatic response to ALK inhibition in this case, identifying patients with ALK fusion MTC has important therapeutic implications.
PMID: 31650892
ISSN: 1557-9077
CID: 4966572

Disaster Plans: Resident Involvement and Well-Being During Hurricane Harvey

Hillier, Kirsty; Paskaradevan, Janaki; Wilkes, J Kevin; Copeland, Emily S
PMID: 31024642
ISSN: 1949-8357
CID: 4966562

A 3-Week-Old With an Isolated "Blueberry Muffin" Rash [Case Report]

Darby, John B; Valentine, Gregory; Hillier, Kirsty; Hunt, Raegan; Healy, C Mary; Smith, Valeria; Allen-Rhoades, Wendy
A 3-week-old boy, former 39-week term infant, presented to the emergency department with a rash. One week before presentation, he developed dark, purple papules on his forehead, which then spread to the abdomen and inguinal regions. Throughout this time, he was eating well, gaining weight, developing appropriately, and was afebrile without cough, congestion, or rhinorrhea. On presentation, the patient was well appearing with normal vital signs. His weight was 4.83 kg (86th percentile for age), his length was 56 cm (47th percentile for age), and his head circumference was 37 cm (62nd percentile for age). On skin examination, there were scattered purpuric papules and macules on the scalp, forehead, trunk, abdomen, and inguinal region. Initial laboratory studies were remarkable only for mild anemia. Our expert panel examines the case, offers a differential for a child with a "blueberry muffin" rash, and makes diagnostic considerations.
PMID: 28759392
ISSN: 1098-4275
CID: 4966552

Early feeding and neonatal hypoglycemia in infants of diabetic mothers

Cordero, Leandro; Ramesh, Shilpa; Hillier, Kirsty; Giannone, Peter J; Nankervis, Craig A
OBJECTIVES/OBJECTIVE:To examine the effects of early formula feeding or breast-feeding on hypoglycemia in infants born to 303 A1-A2 and 88 Class B-RF diabetics. METHODS:Infants with hypoglycemia (blood glucose < 40 mg/dL) were breast-fed or formula-fed, and those with recurrences were given intravenous dextrose. RESULTS:Of 293 infants admitted to the well-baby nursery, 87 (30%) had hypoglycemia, corrected by early feeding in 75 (86%), while 12 (14%) required intravenous dextrose. In all, 98 infants were admitted to the newborn intensive care unit for respiratory distress (40%), prematurity (33%) or prevention of hypoglycemia (27%). Although all newborn intensive care unit patients received intravenous dextrose, 22 (22%) had hypoglycemia. Of 109 hypoglycemia episodes, 89 (82%) were single low occurrences. At discharge, 56% of well-baby nursery and 43% of newborn intensive care unit infants initiated breast-feeding. CONCLUSIONS:Hypoglycemia among infants of diabetic mothers can be corrected by early breast-feeding or formula feeding.
PMID: 26770697
ISSN: 2050-3121
CID: 4966542