Publications by authors named "Kelly J Walkovich"
Article Synopsis
- Rheumatologic disease-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and serious condition that may arise from underlying autoimmune diseases; real-world data on the use of the monoclonal antibody emapalumab for treatment is limited.
- A study called REAL-HLH reviewed medical records from 33 hospitals to evaluate the use and outcomes of emapalumab in patients with rheumatologic disease-associated HLH, finding that 14.3% of 105 patients studied had this subtype.
- Results showed that emapalumab treatment led to improvements in key laboratory parameters, significantly reduced glucocorticoid use, and had a high survival rate, indicating its potential effectiveness in managing
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome. Emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon gamma, is approved in the United States to treat primary HLH (pHLH) in patients with refractory, recurrent, or progressive disease, or intolerance with conventional HLH treatments. REAL-HLH, a retrospective study, conducted across 33 US hospitals, evaluated real-world treatment patterns and outcomes in patients treated with ≥1 dose of emapalumab between 20 November 2018 and 31 October 2021.
View Article and Find Full Text PDFHemophagocytic lymphohistiocytosis (HLH) is an immune dysregulatory syndrome characterized by severe inflammation and end-organ damage. Due to significant organ dysfunction, patients often require extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT). In this report, we describe consideration for adjusting treatment in the context of extracorporeal organ support.
View Article and Find Full Text PDFPurpose: Both pediatric and adult patients with a primary immunodeficiency/immune dysregulation (PID/PIDR) diagnosis report inferior quality of life (QOL) and patient-reported outcomes (PROs) as compared with their healthy peers. Recognition of the negative impact on QOL and PROs provides an opportunity for clinicians to intervene with supportive measures. However, provider perceptions of PID/PIDR patients' quality of life, physical well-being, psychosocial health and neurocognition, and access to supportive resources have yet to be systematically evaluated.
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- The study analyzed the outcomes of immunosuppressive therapy in 314 children with severe aplastic anemia treated between 2002 and 2014 across 25 institutions in North America.
- A significant majority (71.2%) responded positively to horse anti-thymocyte globulin and cyclosporine, with high rates of complete (59.8%) and partial responses.
- Despite a 93% overall survival rate after five years, only 64% remained event-free without further treatment, indicating a need for better long-term therapies for this condition.
Severe congenital neutropenia (SCN) and Shwachman-Diamond syndrome (SDS) are congenital neutropenia syndromes with a high rate of leukemic transformation. Hematopoietic stressors may contribute to leukemic transformation by increasing the mutation rate in hematopoietic stem/progenitor cells (HSPCs) and/or by promoting clonal hematopoiesis. We sequenced the exome of individual hematopoietic colonies derived from 13 patients with congenital neutropenia to measure total mutation burden and performed error-corrected sequencing on a panel of 46 genes on 80 patients with congenital neutropenia to assess for clonal hematopoiesis.
View Article and Find Full Text PDFConsiderable variation in the management of fever and neutropenia (FN) exists, with factors associated with treatment variation not well described. An online survey of 90 pediatric cancer providers in Michigan was performed in Spring 2014. The survey frame was pediatric patients with cancer receiving treatment, with a Port-a-cath, who were clinically stable.
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- * A significant portion of FN cases (40%) had a short length of stay (SLOS) of 3 days or less, leading to approximately $65.5 million in hospital charges, often linked with minor infections like upper respiratory infections and acute otitis media.
- * Factors influencing SLOS included geographic location, specific diagnoses (like acute otitis media and viral infections), and types of cancer, indicating that FN hospitalizations are common but often not associated with severe infections.