Hypereosinophilic Syndrome and Related Disorders

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Masri, A., Bhattacharya, P., Medoff, B., Ejaz, A. U., Elman, M. R., Chandrashekar, P., Ives, L., Santos, A. M., Teruya, S. L., Zhao, Y., Huang, S., Wang, X., Sperry, B. W., Maurer, M. S., Soman, P., & Hanna, M. (2025). A Multicenter Study of Contemporary Long-Term Tafamidis Outcomes in Transthyretin Amyloid Cardiomyopathy. JACC: CardioOncology, 7(3), 282–293. https://doi.org/10.1016/j.jaccao.2024.12.005
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Gonzalez-Lopez, E., Maurer, M. S., & Garcia-Pavia, P. (2025). Transthyretin amyloid cardiomyopathy: a paradigm for advancing precision medicine. European Heart Journal. https://doi.org/10.1093/eurheartj/ehae811
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Dellon, E. S., Muir, A. B., Katzka, D. A., Shah, S. C., Sauer, B. G., Aceves, S. S., Furuta, G. T., Gonsalves, N., & Hirano, I. (2025). ACG Clinical Guideline: Diagnosis and Management of Eosinophilic Esophagitis. American Journal of Gastroenterology, 120(1), 31–59. https://doi.org/10.14309/ajg.0000000000003194
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Hirano, I., Collins, M. H., Katzka, D. A., Mukkada, V. A., Falk, G. W., Terreri, B., Boules, M., Zhang, W., Desai, N. K., & Dellon, E. S. (2024). Effect of Esophageal Dilation History on Efficacy Outcomes in Patients With Eosinophilic Esophagitis Receiving Budesonide Oral Suspension. American Journal of Gastroenterology. https://doi.org/10.14309/ajg.0000000000003197
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Adeuyan, O., Trager, M. H., Gordon, E. R., Lapolla, B. A., Schreidah, C. M., Fahmy, L. M., Chen, C., Magro, C. M., & Geskin, L. J. (2024). A unique case of paraneoplastic lymphomatoid Papuloerythroderma of Ofuji with atypical clinical features. JAAD Case Reports, 50, 102–107. https://doi.org/10.1016/j.jdcr.2024.02.009
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Song, A., Li, J. Y., Cockey, S. G., Shao, R., & Zhang, H. (2024). EBV‐Positive Pleomorphic Variant Transformation of CD5‐Negative Mantle Cell Lymphoma: A Rare Case Report and Literature Review. Case Reports in Hematology, 2024(1). Portico. https://doi.org/10.1155/2024/3267739
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Masuda, M. Y., Pyon, G. C., Luo, H., LeSuer, W. E., Putikova, A., Dao, A., Ortiz, D. R., Schulze, A. R., Fritz, N., Kobayashi, T., Iijima, K., Klein-Szanto, A. J., Shimonosono, M., Flashner, S., Morimoto, M., Pai, R. K., Rank, M. A., Nakagawa, H., Kita, H., … Doyle, A. D. (2024). Epithelial overexpression of IL-33 induces eosinophilic esophagitis dependent on IL-13. Journal of Allergy and Clinical Immunology, 153(5), 1355–1368. https://doi.org/10.1016/j.jaci.2024.01.017
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Lee, M. H., Song, A., & Li, J. Y. (2024). Erythroid variant evolving from chronic myeloid leukemia resistant to multiple tyrosine kinase inhibitors: a rare case report. Diagnostic Pathology, 19(1). https://doi.org/10.1186/s13000-024-01446-9
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Hughes, M., & Lentzsch, S. (2023). Safety and Efficacy of Subcutaneous Daratumumab in Systemic AL Amyloidosis. Therapeutics and Clinical Risk Management, Volume 19, 1063–1074. https://doi.org/10.2147/tcrm.s325859
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Tashi, T., Hermine, O., Castells, M., Guilarte, M., Sabato, V., Maurer, M., Panse, J., Alvarez-Twose, I., Cabral, R., Bird, R., Barete, S., Bouillet, L., Hermans, M., Van Daele, P., González-De-Olano, D., Griffiths, E. A., Jurcic, J., Dybedal, I., Damaj, G. L., … Livideanu, C. B. (2023). Elenestinib, an Investigational, Next Generation KIT D816V Inhibitor, Reduces Mast Cell Burden, Improves Symptoms, and Has a Favorable Safety Profile in Patients with Indolent Systemic Mastocytosis: Analysis of the Harbor Trial. Blood, 142(Supplement 1), 76–76. https://doi.org/10.1182/blood-2023-188904
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Orlando, E. H., Gould, P., Cuzzo, B., Ford, M., Chen, Y., Sanjurjo, A., May, B., Tsapepas, D., Leeman-Neill, R. J., Bhagat, G., Dube, G., Morris, H., Arcasoy, S., Latif, F., Martinez, M., Pereira, M., Lipsky, A. H., Sawas, A., Reshef, R., … Amengual, J. E. (2023). The Effect of Preemptive Rituximab Treatment in Patients with EBV Viremia after Solid Organ Transplantation on the Development of Post-Transplant Lymphoproliferative Disorder. Blood, 142(Supplement 1), 990–990. https://doi.org/10.1182/blood-2023-177823
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Lapolla, B., Gordon, E., Fahmy, L., Schreidah, C., Adeuyan, O., Magro, C., & Geskin, L. (2023). 214 Angiolymphoid hyperplasia with eosinophilia and kimura disease: a case of clinical and histopathological discrepancy and response to dupilumab. Journal of Investigative Dermatology, 143(11), S368. https://doi.org/10.1016/j.jid.2023.09.222
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Yu, Z., Fidler, T. P., Ruan, Y., Vlasschaert, C., Nakao, T., Uddin, M. M., Mack, T., Niroula, A., Heimlich, J. B., Zekavat, S. M., Gibson, C. J., Griffin, G. K., Wang, Y., Peloso, G. M., Heard-Costa, N., Levy, D., Vasan, R. S., Aguet, F., Ardlie, K. G., … Natarajan, P. (2023). Genetic modification of inflammation- and clonal hematopoiesis–associated cardiovascular risk. Journal of Clinical Investigation, 133(18). https://doi.org/10.1172/jci168597
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Murthy, G. S. G., Kim, S., Estrada-Merly, N., Abid, M. B., Aljurf, M., Assal, A., Badar, T., Badawy, S. M., Ballen, K., Beitinjaneh, A., Cerny, J., Chhabra, S., DeFilipp, Z., Dholaria, B., Perez, M. A. D., Farhan, S., Freytes, C. O., Gale, R. P., Ganguly, S., … Saber, W. (2023). Association between the choice of the conditioning regimen and outcomes of allogeneic hematopoietic cell transplantation for myelofibrosis. Haematologica, 108(7), 1900–1908. https://doi.org/10.3324/haematol.2022.281958
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Hirano, I., Dellon, E. S., Gupta, S. K., Katzka, D. A., Collins, M. H., Wojtowicz, A. M., Terreri, B., Zhang, W., Boules, M., Bhatia, S., & Desai, N. K. (2023). Safety of an investigational formulation of budesonide (budesonide oral suspension) for eosinophilic oesophagitis: an integrated safety analysis of six phase 1–3 clinical trials. Alimentary Pharmacology & Therapeutics, 57(10), 1117–1130. Portico. https://doi.org/10.1111/apt.17430
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Salinas, S. A., Mace, E. M., Conte, M. I., Park, C. S., Li, Y., Rosario-Sepulveda, J. I., Mahapatra, S., Moore, E. K., Hernandez, E. R., Chinn, I. K., Reed, A. E., Lee, B. J., Frumovitz, A., Gibbs, R. A., Posey, J. E., Forbes Satter, L. R., Thatayatikom, A., Allenspach, E. J., Wensel, T. G., … Orange, J. S. (2022). An ELF4 hypomorphic variant results in NK cell deficiency. JCI Insight, 7(23). https://doi.org/10.1172/jci.insight.155481
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Brannagan, T. H., Coelho, T., Wang, A. K., Polydefkis, M. J., Dyck, P. J., Berk, J. L., Drachman, B., Gorevic, P., Whelan, C., Conceição, I., Plante-Bordeneuve, V., Merlini, G., Obici, L., Plana, J. M. C., Gamez, J., Kristen, A. V., Mazzeo, A., Gentile, L., … Narayana, A. (2022). Long-term efficacy and safety of inotersen for hereditary transthyretin amyloidosis: NEURO-TTR open-label extension 3-year update. Journal of Neurology, 269(12), 6416–6427. https://doi.org/10.1007/s00415-022-11276-8
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DeAngelo, D. J., Pullarkat, V. A., Piris-Villaespesa, M., George, T. I., Patel, J. L., Ustun, C., Bose, P., Heaney, M. L., Sachs, J., Sun, L., Pilla, A., Exter, B., Jolin, H. A., & Tashi, T. (2022). Preliminary Safety and Efficacy from Apex, a Phase 2 Study of Bezuclastinib (CGT9486), a Novel, Highly Selective, Potent KIT D816V Tyrosine Kinase Inhibitor, in Adults with Advanced Systemic Mastocytosis (AdvSM). Blood, 140(Supplement 1), 1512–1513. https://doi.org/10.1182/blood-2022-156311
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Gonzalez-Lopez, E., Escobar-Lopez, L., Obici, L., Saturi, G., Bezard, M., Saith, S. E., AbouEzzeddine, O. F., Mussinelli, R., Gagliardi, C., Kharoubi, M., Griffin, J. M., Dispenzieri, A., Vilches, S., Perlini, S., Longhi, S., Oghina, S., Rivas, A., Grogan, M., Maurer, M. S., … Garcia-Pavia, P. (2022). Prognosis of Transthyretin Cardiac Amyloidosis Without Heart Failure Symptoms. JACC: CardioOncology, 4(4), 442–454. https://doi.org/10.1016/j.jaccao.2022.07.007
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Dellon, E. S., Gonsalves, N., Abonia, J. P., Alexander, J. A., Arva, N. C., Atkins, D., Attwood, S. E., Auth, M. K. H., Bailey, D. D., Biederman, L., Blanchard, C., Bonis, P. A., Bose, P., Bredenoord, A. J., Chang, J. W., Chehade, M., Collins, M. H., Di Lorenzo, C., Dias, J. A., … Aceves, S. S. (2022). International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature. Clinical Gastroenterology and Hepatology, 20(11), 2474-2484.e3. https://doi.org/10.1016/j.cgh.2022.02.017
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Nathanson, J. T., Chawla, S., Blackett, J., & Garcia-Carrasquillo, R. J. (2022). S2307 Esophageal Hemorrhage After Transesophageal Echocardiography in a Patient With Undiagnosed Eosinophilic Esophagitis. American Journal of Gastroenterology, 117(10S), e1553–e1553. https://doi.org/10.14309/01.ajg.0000865868.81054.8f
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Tran, P., Gober, L., Garabedian, E. K., Fuleihan, R. L., Puck, J. M., Sullivan, K. E., Spergel, J. M., & Ruffner, M. A. (2022). Eosinophilic gastrointestinal disorders in patients with inborn errors of immunity: Data from the USIDNET registry. Frontiers in Immunology, 13. https://doi.org/10.3389/fimmu.2022.987895
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Westin, O., Fosbøl, E. L., Maurer, M. S., Leicht, B. P., Hasbak, P., Mylin, A. K., Rørvig, S., Lindkær, T. H., Johannesen, H. H., & Gustafsson, F. (2022). Screening for Cardiac Amyloidosis 5 to 15 Years After Surgery for Bilateral Carpal Tunnel Syndrome. Journal of the American College of Cardiology, 80(10), 967–977. https://doi.org/10.1016/j.jacc.2022.06.026
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DeAngelo, D. J., Pullarkat, V., Piris-Villaespesa, M., George, T. I., Patel, J. L., Ustun, C., Bose, P., Heaney, M. L., Pilla, A., Massaro, M., Exter, B., Jolin, H. A., Mikhak, Z., & Tashi, T. (2022). P1049: A Phase 2 Study of Bezuclastinib (CGT9486), a Novel, Highly Selective, Potent Kit D816V Inhibitor, in Adults With Advanced Systemic Mastocytosis (Apex): Methods, Baseline Data, and Early Insights. HemaSphere, 6, 939–940. https://doi.org/10.1097/01.hs9.0000847064.38588.9b

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Fichadiya, H., Noori, M. A. M., Patel, B. D., Bhavsar, M., Visveswaran, G. K., Sommer, R. J., & Joshi, M. (2022). A RARE CASE OF MULTIPLE PULMONARY VEIN STENOSIS ASSOCIATED WITH DERMATOMYOSITIS. Journal of the American College of Cardiology, 79(9), 3197. https://doi.org/10.1016/s0735-1097(22)04188-2
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Hara, T., Kasagi, Y., Wang, J., Sasaki, M., Aaron, B., Karami, A., Shimonosono, M., Shimonosono, R., Maekawa, H., Dolinsky, L., Wilkins, B., Klein, J., Wei, J., Nunes, K., Lynch, K., Spergel, J. M., Hamilton, K. E., Ruffner, M. A., Karakasheva, T. A., … Muir, A. B. (2022). CD73+ Epithelial Progenitor Cells That Contribute to Homeostasis and Renewal Are Depleted in Eosinophilic Esophagitis. Cellular and Molecular Gastroenterology and Hepatology, 13(5), 1449–1467. https://doi.org/10.1016/j.jcmgh.2022.01.018
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Gotlib, J., Reiter, A., Radia, D. H., Deininger, M. W., George, T. I., Panse, J., Vannucchi, A. M., Platzbecker, U., Alvarez-Twose, I., Mital, A., Hermine, O., Dybedal, I., Hexner, E. O., Hicks, L. K., Span, L., Mesa, R., Bose, P., Pettit, K. M., Heaney, M. L., … DeAngelo, D. J. (2021). Efficacy and safety of avapritinib in advanced systemic mastocytosis: interim analysis of the phase 2 PATHFINDER trial. Nature Medicine, 27(12), 2192–2199. https://doi.org/10.1038/s41591-021-01539-8
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