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1. COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

Author

McDonnell, John, Cousins, Kimberley, Younger, M. Elizabeth M., Lane, Adam, Abolhassani, Hassan, Abraham, Roshini S., Al-Tamemi, Salem, Aldave-Becerra, Juan Carlos, Al-Faris, Eman Hesham, Alfaro-Murillo, Alberto, AlKhater, Suzan A., Alsaati, Nouf, Doss, Alexa Michelle Altman, Anderson, Melissa, Angarola, Ernestina, Ariue, Barbara, Arnold, Danielle E., Assa’ad, Amal H., Aytekin, Caner, Bank, Meaghan, Bergerson, Jenna R. E., Bleesing, Jack, Boesing, John, Bouso, Carolina, Brodszki, Nicholas, Cabanillas, Diana, Cady, Carol, Callahan, Meghan A., Caorsi, Roberta, Carbone, Javier, Carrabba, Maria, Castagnoli, Riccardo, Catanzaro, Jason R., Chan, Samantha, Chandra, Sharat, Chapdelaine, Hugo, Chavoshzadeh, Zahra, Chong, Hey Jin, Connors, Lori, Consonni, Filippo, Correa-Jimenez, Oscar, Cunningham-Rundles, Charlotte, D’Astous-Gauthier, Katherine, Delmonte, Ottavia Maria, Demirdag, Yesim Yilmaz, Deshpande, Deepti R., Diaz-Cabrera, Natalie M., Dimitriades, Victoria R., El-Owaidy, Rasha, ElGhazali, Gehad, Al-Hammadi, Suleiman, Fabio, Giovanna, Faure, Astrid Schellnast, Feng, Jin, Fernandez, James M., Fill, Lauren, Franco, Guacira R., Frenck, Robert W., Fuleihan, Ramsay L., Giardino, Giuliana, Galant-Swafford, Jessica, Gambineri, Eleonora, Garabedian, Elizabeth K., Geerlinks, Ashley V., Goudouris, Ekaterini, Grecco, Octavio, Pan-Hammarström, Qiang, Khani, Hedieh Haji Khodaverdi, Hammarström, Lennart, Hartog, Nicholas L., Heimall, Jennifer, Hernandez-Molina, Gabriela, Horner, Caroline C., Hostoffer, Robert W., Hristova, Nataliya, Hsiao, Kuang-Chih, Ivankovich-Escoto, Gabriela, Jaber, Faris, Jalil, Maaz, Jamee, Mahnaz, Jean, Tiffany, Jeong, Stephanie, Jhaveri, Devi, Jordan, Michael B., Joshi, Avni Y., Kalkat, Amanpreet, Kanarek, Henry J., Kellner, Erinn S., Khojah, Amer, Khoury, Ruby, Kokron, Cristina M., Kumar, Ashish, Lecerf, Kelsey, Lehman, Heather K., Leiding, Jennifer W., Lesmana, Harry, Lim, Xin Rong, Lopes, Joao Pedro, López, Ana Laura, Tarquini, Lucia, Lundgren, Ingrid S., Magnusson, Julieann, Marinho, Ana Karolina B. B., Marseglia, Gian Luigi, Martone, Giulia M., Mechtler, Annamaria G., Mendonca, Leonardo, Milner, Joshua D., Mustillo, Peter J., Naderi, Asal Gharib, Naviglio, Samuele, Nell, Jeremy, Niebur, Hana B., Notarangelo, Luigi, Oleastro, Matias, Ortega-López, María Claudia, Patel, Neil R., Petrovic, Gordana, Pignata, Claudio, Porras, Oscar, Prince, Benjamin T., Puck, Jennifer M., Qamar, Nashmia, Rabusin, Marco, Raje, Nikita, Regairaz, Lorena, Risma, Kimberly A., Ristagno, Elizabeth H., Routes, John, Roxo-Junior, Persio, Salemi, Negin, Scalchunes, Christopher, Schuval, Susan J., Seneviratne, Suranjith L., Shankar, Ashwin, Sherkat, Roya, Shin, Junghee Jenny, Siddiqi, Abeer, Signa, Sara, Sobh, Ali, Lima, Fabiana Mascarenhas Souza, Stenehjem, Kristen K., Tam, Jonathan S., Tang, Monica, Barros, Myrthes Toledo, Verbsky, James, Vergadi, Eleni, Voelker, Dayne H., Volpi, Stefano, Wall, Luke A., Wang, Christine, Williams, Kelli W., Wu, Eveline Y., Wu, Shan Shan, Zhou, Jessie J., Cook, Alexandria, Sullivan, Kathleen E., and Marsh, Rebecca

Published
2024
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2. Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab

Author

Weinstein, Joanna, Hinson, Ashley P., Allen, Carl E., Eckstein, Olive S., Gulati, Nitya, Chandrakasan, Shanmuganathan, Haines, Hilary, Nakano, Taizo, Patel, Sachit A., Behrens, Edward M, Intzes, Stefanos, Hays, Allyson, Dávila Saldaña, Blachy, Jordan, Michael B., Zoref-Lorenz, Adi, Hanna, Rabi, Isakoff, Michael, Ray, Anish K., Rothman, Jennifer, Tandra, Anand, Cooper, Robert, Leiding, Jennifer W., Chien, May, Sarangi, Susmita, Gidvani, Diaz, Satwani, Prakesh, Carter, John, Henry, Michael, Gloude, Nicholas, Bhatt, Sima, Bhatla, Deepika, Draper, Lauren, Panigrahi, Arun, Hermiston, Michelle, Modica, Renee, Riskalla, Mona, Baker, Ashley, Ward, Brant, Behrens, Edward M., Henry, Michael M., Hermiston, Michelle L., Oladapo, Abiola, Pednekar, Priti, Sarangi, Susmita N., Walkovich, Kelly J., and Yee, John D.

Published
2024
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3. High risk of relapsed disease in patients with NK/T cell chronic active Epstein-Barr virus disease outside of Asia

Author

Saldaña, Blachy J Dávila, John, Tami D, Bonifant, Challice L, Buchbinder, David, Chandra, Sharat, Chandrakasan, Shanmuganathan, Chang, Weni, Chen, Leon, Elfassy, Hannah L, Geerlinks, Ashley V, Giller, Roger H, Goyal, Rakesh Kumar, Hagin, David, Islam, Shahidul, Mallhi, Kanwaldeep K, Miller, Holly K, Owen, William C, Pacheco, Martha, Patel, Niraj, Querfeld, Christiane, Quigg, Troy, Richard, Nameeta, Schiff, Deborah E, Shereck, Evan, Stenger, Elizabeth O, Jordan, Michael B, Heslop, Helen E, Bollard, Catherine M, and Cohen, Jeffrey I

Subjects
Lymphoma, Stem Cell Research, Stem Cell Research - Nonembryonic - Human, Cancer, Clinical Research, Hematology, Transplantation, Rare Diseases, Regenerative Medicine, Good Health and Well Being, Asia, Chronic Disease, Epstein-Barr Virus Infections, Herpesvirus 4, Human, Humans, Lymphoproliferative Disorders, Natural Killer T-Cells, Retrospective Studies, United States
Abstract

Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is characterized by high levels of EBV predominantly in T and/or natural killer cells with lymphoproliferation, organ failure due to infiltration of tissues with virus-infected cells, hemophagocytic lymphohistiocytosis, and/or lymphoma. The disease is more common in Asia than in the United States and Europe. Although allogeneic hematopoietic stem cell transplantation (HSCT) is considered the only curative therapy for CAEBV, its efficacy and the best treatment modality to reduce disease severity prior to HSCT is unknown. Here, we retrospectively assessed an international cohort of 57 patients outside of Asia. Treatment of the disease varied widely, although most patients ultimately proceeded to HSCT. Though patients undergoing HSCT had better survival than those who did not (55% vs 25%, P < .01), there was still a high rate of death in both groups. Mortality was largely not affected by age, ethnicity, cell-type involvement, or disease complications, but development of lymphoma showed a trend with increased mortality (56% vs 35%, P = .1). The overwhelming majority (75%) of patients who died after HSCT succumbed to relapsed disease. CAEBV remains challenging to treat when advanced disease is present. Outcomes would likely improve with better disease control strategies, earlier referral for HSCT, and close follow-up after HSCT including aggressive management of rising EBV DNA levels in the blood.

Published
2022

6. IFN-γ Signature in the Plasma Proteome Distinguishes Pediatric Hemophagocytic Lymphohistiocytosis from Sepsis and SIRS

Author

Lin, Howard, Scull, Brooks P, Goldberg, Baruch R, Abhyankar, Harshal A, Eckstein, Olive S, Zinn, Daniel J, Lubega, Joseph, Agrusa, Jennifer, El-Mallawanay, Nader K, Gulati, Nitya, Forbes, Lisa R, Chinn, Ivan K, Chakraborty, Rikhia, Velazquez, Jessica, Goldman, Jordana, Bashir, Dalia A, Lam, Fong Wilson, Muscal, Eyal, Henry, Michael M, Greenberg, Jay, Ladisch, Stephan, Hermiston, Michelle L, Meyer, Lauren K, Jeng, Michael Raymond, Naqvi, Ahmed, McClain, Kenneth L, Nguyen, Trung, Wong, Hector R, Man, Tsz-Kwong, Jordan, Michael B, and Allen, Carl E

Subjects
Pediatric, Infectious Diseases, Rare Diseases, Hematology, Clinical Research, Sepsis, Detection, screening and diagnosis, 2.1 Biological and endogenous factors, Aetiology, 4.1 Discovery and preclinical testing of markers and technologies, Inflammatory and immune system, Child, Diagnosis, Differential, Humans, Interferon-gamma, Lymphohistiocytosis, Hemophagocytic, Proteome, Systemic Inflammatory Response Syndrome
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by pathologic immune activation in which prompt recognition and initiation of immune suppression is essential for survival. Children with HLH have many overlapping clinical features with critically ill children with sepsis and systemic inflammatory response syndrome (SIRS) in whom alternative therapies are indicated. To determine whether plasma biomarkers could differentiate HLH from other inflammatory conditions and to better define a core inflammatory signature of HLH, concentrations of inflammatory plasma proteins were compared in 40 patients with HLH to 47 pediatric patients with severe sepsis or SIRS. Fifteen of 135 analytes were significantly different in HLH plasma compared with SIRS/sepsis, including increased interferon-γ (IFN-γ)-regulated chemokines CXCL9, CXCL10, and CXCL11. Furthermore, a 2-analyte plasma protein classifier including CXCL9 and interleukin-6 was able to differentiate HLH from SIRS/sepsis. Gene expression in CD8+ T cells and activated monocytes from blood were also enriched for IFN-γ pathway signatures in peripheral blood cells from patients with HLH compared with SIRS/sepsis. This study identifies differential expression of inflammatory proteins as a diagnostic strategy to identify critically ill children with HLH, and comprehensive unbiased analysis of inflammatory plasma proteins and global gene expression demonstrates that IFN-γ signaling is uniquely elevated in HLH. In addition to demonstrating the ability of diagnostic criteria for HLH and sepsis or SIRS to identify groups with distinct inflammatory patterns, results from this study support the potential for prospective evaluation of inflammatory biomarkers to aid in diagnosis of and optimizing therapeutic strategies for children with distinctive hyperinflammatory syndromes.

Published
2021

8. Alemtuzumab and CXCL9 levels predict likelihood of sustained engraftment after reduced-intensity conditioning HCT

Author

Geerlinks, Ashley V., Scull, Brooks, Krupski, Christa, Fleischmann, Ryan, Pulsipher, Michael A., Eapen, Mary, Connelly, James A., Bollard, Catherine M., Pai, Sung-Yun, Duncan, Christine N., Kean, Leslie S., Baker, K. Scott, Burroughs, Lauri M., Andolina, Jeffrey R., Shenoy, Shalini, Roehrs, Philip, Hanna, Rabi, Talano, Julie-An, Schultz, Kirk R., Stenger, Elizabeth O., Lin, Howard, Zoref-Lorenz, Adi, McClain, Kenneth L., Jordan, Michael B., Man, Tsz-Kwong, Allen, Carl E., and Marsh, Rebecca A.

Published
2023
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9. List Of Contributors

Author

Abraham, Roshini Sarah, primary, Afzali, Behdad, additional, Águeda, Ana, additional, Akin, Cem, additional, Albanesi, Cristina, additional, Antiochos, Brendan, additional, Aranow, Cynthia, additional, Atkinson, John P., additional, Aune, Thomas M., additional, Babu, Subash, additional, Balko, Justin, additional, Ballow, Mark, additional, Bean, Rachel, additional, Belavgeni, Alexia, additional, Berek, Claudia, additional, Beukelman, Timothy, additional, Beziat, Vivien, additional, Bimler, Lynn, additional, Andrew Bird, J., additional, Blutt, Sarah E., additional, Boguniewicz, Mark, additional, Boisson, Bertrand, additional, Boisson-Dupuis, Stéphanie, additional, Borzova, Elena, additional, Bottazzi, Maria, additional, Boyaka, Prosper N., additional, Bridges, John, additional, Browne, Sarah K., additional, Burks, A. Wesley, additional, Bustamante, Jacinta, additional, Casanova, Jean-Laurent, additional, Chan, Alice, additional, Chan, Edwin S.L., additional, Chatham, Walter Winn, additional, Chinen, Javier, additional, Christopher-Stine, Lisa, additional, Coates, Emily, additional, Cope, Andrew P., additional, Corry, David B., additional, Cosme, Joana, additional, Cron, Randy Q., additional, Dalakas, Marinos C., additional, Dann, Sara M., additional, Das, Satya, additional, Daughety, Molly M., additional, Diamond, Betty, additional, Dispenzieri, Angela, additional, Durham, Stephen R., additional, Eagar, Todd N., additional, Al-Hosni, Michelle, additional, Elitzur, Sarah, additional, Elmets, Craig A., additional, Erkan, Doruk, additional, Fleisher, Thomas A., additional, Fonacier, Luz, additional, Fontenot, Andrew P., additional, Fragoulis, George, additional, Francischetti, Ivo M.B., additional, Freiwald, Tilo, additional, Frew, Anthony J., additional, Fujihashi, Kohtaro, additional, Gadina, Massimo, additional, Gapin, Laurent, additional, Gatt, Moshe E., additional, Gershwin, M. Eric, additional, Gillespie, Susan L., additional, Gordon, Lynn K., additional, Goronzy, Jörg J., additional, Grattan, Clive E., additional, Greenspan, Neil S., additional, Gschwend, Anna, additional, Gustafson, Claire E., additional, Hackett, Tillie-Louise, additional, Hamilton, Robert G., additional, Happe, Myra, additional, Harrison, Leonard C., additional, Helbling, Arthur, additional, Heckmann, Emmaline, additional, Hogquist, Kristin, additional, Hohl, Tobias M., additional, Holland, Steven M., additional, Hotez, Peter J., additional, Houser, Katherine, additional, Huntingdon, Nicholas D., additional, Hwangpo, Tracy, additional, Izraeli, Shai, additional, Jaffe, Elaine S., additional, Jalkanen, Sirpa, additional, Java, Anuja, additional, Johnson, Douglas B., additional, Johnson, Tory, additional, Jordan, Michael B., additional, Joshi, Shyam R., additional, Jouanguy, Emmanuelle, additional, Kaminski, Henry J., additional, Kaufmann, Stefan H.E., additional, Khan, David A., additional, Kheradmand, Farrah, additional, Khokar, Dilawar Singh, additional, Khoury, Paneez, additional, Klein, Bruce S., additional, Klion, Amy D., additional, Kohn, Donald B., additional, Kono, Michihito, additional, Korngold, Robert, additional, Koulouri, Vasiliki, additional, Kuhns, Douglas B., additional, Kulkarni, Hrishikesh S., additional, Kuo, Caroline Y., additional, Kusner, Linda L., additional, Lahouti, Arash, additional, Lane, Laura C., additional, Laurence, Arian, additional, Lee, Joyce S., additional, Lee, S. Thera, additional, Leung, Donald Y.M., additional, Levy, Ofer, additional, Lewis, Dorothy E., additional, Li, Evan, additional, Libby, Peter, additional, Lichtman, Andrew H., additional, Linkermann, Andreas, additional, Lionakis, Michail S., additional, Liszewski, M. Kathryn, additional, Lockshin, Michael D., additional, Priel, Debra Long, additional, Lorenz, Adi Zoref, additional, Ludwig, Ralf J., additional, Luong, Amber, additional, Luqmani, Raashid Ahmed, additional, Mackay, Meggan, additional, Mahr, Alfred, additional, Malley, Tamir, additional, Mannon, Elinor C., additional, Mannon, Peter J., additional, Mannon, Roslyn B., additional, Manns, Michael P., additional, Maresso, Anthony, additional, Matson, Scott M., additional, Mavragani, Clio P., additional, Maynard, Craig L., additional, McDonald, Douglas, additional, Meylan, Françoise, additional, Miller, Stephen D., additional, Mitchell, Anna L., additional, Monos, Dimitri S., additional, Mueller, Scott N., additional, Mulders-Manders, Catharina M., additional, Munshi, Pashna N., additional, Murphy, Philip M., additional, Noel, Pierre, additional, Notarangelo, Luigi D., additional, Nunes-Santos, Cristiane J., additional, Nussbaum, Robert L., additional, Nutman, Thomas B., additional, Nutt, Stephen L., additional, O'Neill, Lorraine, additional, O'Shea, John J., additional, Ortel, Thomas L., additional, Pai, Sung-Yun, additional, Paul, Mary E., additional, Pearce, Simon, additional, Peterson, Erik J., additional, Pittaluga, Stefania, additional, Polverino, Francesca, additional, Puck, Jennifer M., additional, Puel, Anne, additional, Radbruch, Andreas, additional, Rajalingam, Raja, additional, Reece, Stephen T., additional, Reveille, John D., additional, Rich, Robert R., additional, Ridley, Lauren K., additional, Romeo, Andrew R., additional, Rooney, Cliona M., additional, Rosen, Antony, additional, Rosenzweig, Sergio, additional, Rouse, Barry T., additional, Rowley, Scott D., additional, Sahiner, Umit Murat, additional, Sakaguchi, Shimon, additional, Salinas, Whitney, additional, Salmi, Marko, additional, Satola, Sarah, additional, Schechter, Marcos, additional, Schmidt, Enno, additional, Schroeder, Harry W., additional, Schwartzberg, Pamela L., additional, Sciumè, Giuseppe, additional, Segal, Benjamin M., additional, Selmi, Carlo, additional, Sharabi, Amir, additional, Shimano, Kristin Ammon, additional, Sikorski, Patricia M., additional, Simon, Anna, additional, Smith, Gideon P., additional, Song, Joo Y., additional, Stephens, David S., additional, Stephens, Robin, additional, Sun, Michel M., additional, Beretta-Piccoli, Benedetta Terziroli, additional, Tonnus, Wulf, additional, Torgerson, Troy R., additional, Torres, Raul Martin, additional, Treat, Jennifer D., additional, Tsokos, George C., additional, Uzel, Gülbü, additional, Uzonna, Jude E., additional, van der Hilst, Jeroen C.H., additional, van der Meer, Jos W.M., additional, Varga, John, additional, Waldman, Meryl, additional, Weatherhead, Jill, additional, Weiser, Peter, additional, Weyand, Cornelia M., additional, Wigley, Fredrick M., additional, Wing, James B., additional, Wood, Kathryn J., additional, Wilde, Shyra, additional, Xu, Hui, additional, Yusuf, Nabiha, additional, Zerbe, Christa S., additional, Zhang, Qian, additional, Ben-Yehuda, Dina, additional, Zhang, Shen-Ying, additional, and Zieske, Arthur W., additional

Published
2023
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11. Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey.

Author

Chan, Alice Y, Leiding, Jennifer W, Liu, Xuerong, Logan, Brent R, Burroughs, Lauri M, Allenspach, Eric J, Skoda-Smith, Suzanne, Uzel, Gulbu, Notarangelo, Luigi D, Slatter, Mary, Gennery, Andrew R, Smith, Angela R, Pai, Sung-Yun, Jordan, Michael B, Marsh, Rebecca A, Cowan, Morton J, Dvorak, Christopher C, Craddock, John A, Prockop, Susan E, Chandrakasan, Shanmuganathan, Kapoor, Neena, Buckley, Rebecca H, Parikh, Suhag, Chellapandian, Deepak, Oshrine, Benjamin R, Bednarski, Jeffrey J, Cooper, Megan A, Shenoy, Shalini, Davila Saldana, Blachy J, Forbes, Lisa R, Martinez, Caridad, Haddad, Elie, Shyr, David C, Chen, Karin, Sullivan, Kathleen E, Heimall, Jennifer, Wright, Nicola, Bhatia, Monica, Cuvelier, Geoffrey DE, Goldman, Frederick D, Meyts, Isabelle, Miller, Holly K, Seidel, Markus G, Vander Lugt, Mark T, Bacchetta, Rosa, Weinacht, Katja G, Andolina, Jeffrey R, Caywood, Emi, Chong, Hey, de la Morena, Maria Teresa, Aquino, Victor M, Shereck, Evan, Walter, Jolan E, Dorsey, Morna J, Seroogy, Christine M, Griffith, Linda M, Kohn, Donald B, Puck, Jennifer M, Pulsipher, Michael A, and Torgerson, Troy R

Subjects
Animals, Humans, Treatment Outcome, Hematopoietic Stem Cell Transplantation, Adolescent, Adult, Middle Aged, Child, Child, Preschool, Infant, T-Lymphocytes, Regulatory, Young Adult, Surveys and Questionnaires, Primary Immunodeficiency Diseases, autoimmunity, genetics, hematopoietic cell transplant, immune dysregulation, primary immune deficiencies, Clinical Research, Rare Diseases, Transplantation, 5.2 Cellular and gene therapies, Development of treatments and therapeutic interventions, Inflammatory and immune system, Immunology, Medical Microbiology
Abstract

Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management.

Published
2020

12. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Author

Jordan, Michael B, Allen, Carl E, Greenberg, Jay, Henry, Michael, Hermiston, Michelle L, Kumar, Ashish, Hines, Melissa, Eckstein, Olive, Ladisch, Stephan, Nichols, Kim E, Rodriguez‐Galindo, Carlos, Wistinghausen, Birte, and McClain, Kenneth L

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Rare Diseases, Hematology, Adult, Age of Onset, Child, Clinical Trials as Topic, Diagnosis, Differential, Disease Management, Drug Eruptions, Fetal Diseases, Hematopoietic Stem Cell Transplantation, Humans, Immunocompromised Host, Infant, Infant, Newborn, Liver Failure, Lymphohistiocytosis, Hemophagocytic, Macrophage Activation, Metabolism, Inborn Errors, Neoplasms, Phenotype, Sepsis, hematology, hemophagocytic lymphohistiocytosis, immunology, Oncology and Carcinogenesis, Paediatrics and Reproductive Medicine, Oncology & Carcinogenesis, Oncology and carcinogenesis, Paediatrics
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, often associated with genetic defects of lymphocyte cytotoxicity. Though a distinctive constellation of features has been described for HLH, diagnosis remains challenging as patients have diverse presentations associated with a variety of triggers. We propose two concepts to clarify how HLH is diagnosed and treated: within the broader syndrome of HLH, "HLH disease" should be distinguished from "HLH disease mimics" and HLH subtypes should be categorized by specific etiologic associations, not the ambiguous dichotomy of "primary" and "secondary." We provide expert-based advice regarding the diagnosis and initiation of treatment for patients with HLH, rooted in improved understanding of its pathophysiology.

Published
2019

14. Prospective two center study of CD38 bright CD8+ effector memory T-cells as a predictor of acute GVHD

Author

Khandelwal, Pooja, Chaturvedi, Vijaya, Owsley, Erika, Efebera, Yvonne A., Choe, Hannah, Bostic, Matthew, Kumchala, Prashanti, Rajgolikar, Girish, Ranganathan, Parvathi, Garzon, Ramiro, Lake, Kelly, Litts, Bridget, Duell, Alexandra, Elder, Patrick, Davies, Stella M., Lane, Adam, Jordan, Michael B., Vasu, Sumithra, Devine, Steven, and Marsh, Rebecca A.

Published
2022
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16. Reduced-Intensity/Reduced-Toxicity Conditioning Approaches Are Tolerated in XIAP Deficiency but Patients Fare Poorly with Acute GVHD

Author

Arnold, Danielle E., Nofal, Rofida, Wakefield, Connor, Lehmberg, Kai, Wustrau, Katharina, Albert, Michael H., Morris, Emma C., Heimall, Jennifer R., Bunin, Nancy J., Kumar, Ashish, Jordan, Michael B., Cole, Theresa, Choo, Sharon, Brettig, Tim, Speckmann, Carsten, Ehl, Stephan, Salamonowicz, Malgorzata, Wahlstrom, Justin, Rao, Kanchan, Booth, Claire, Worth, Austen, and Marsh, Rebecca A.

Published
2022
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18. An improved index for diagnosis and mortality prediction in malignancy-associated hemophagocytic lymphohistiocytosis

Author

Zoref-Lorenz, Adi, Murakami, Jun, Hofstetter, Liron, Iyer, Swaminathan, Alotaibi, Ahmad S., Mohamed, Shehab Fareed, Miller, Peter G., Guber, Elad, Weinstein, Shiri, Yacobovich, Joanne, Nikiforow, Sarah, Ebert, Benjamin L., Lane, Adam, Pasvolsky, Oren, Raanani, Pia, Nagler, Arnon, Berliner, Nancy, Daver, Naval, Ellis, Martin, and Jordan, Michael B.

Published
2022
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19. High risk of relapsed disease in patients with NK/T-cell chronic active Epstein-Barr virus disease outside of Asia

Author

Dávila Saldaña, Blachy J., John, Tami, Bonifant, Challice, Buchbinder, David, Chandra, Sharat, Chandrakasan, Shanmuganathan, Chang, Weni, Chen, Leon, Elfassy, Hannah L., Geerlinks, Ashley V., Giller, Roger H., Goyal, Rakesh, Hagin, David, Islam, Shahidul, Mallhi, Kanwaldeep, Miller, Holly K., Owen, William, Pacheco, Martha, Patel, Niraj C., Querfeld, Christiane, Quigg, Troy, Richard, Nameeta, Schiff, Deborah, Shereck, Evan, Stenger, Elizabeth, Jordan, Michael B., Heslop, Helen E., Bollard, Catherine M., and Cohen, Jeffrey I.

Published
2022
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20. Loss of STAT2 may be dangerous in a world filled with viruses

Author

Jordan, Michael B.

Subjects
Health care industry
Abstract

Type I IFNs, a family of cytokines that signal through a single receptor and signaling mechanism, were originally named for their ability to interfere with viral replication. While type II IFN (IFN-[gamma]) largely protects against intracellular bacteria and protozoa, type I IFNs largely protect from viral infections. Inborn errors of immunity in humans have demonstrated this point and its clinical relevance with increasing clarity. In this issue of the JCI, Bucciol, Moens, et al. report the largest series of patients to date with deficiency of STAT2, an important protein for type I IFN signaling. Individuals with STAT2 loss demonstrated a clinical phenotype of viral susceptibility and inflammatory complications, many of which remain poorly understood. These findings further illustrate the very specific and critical role that type I IFNs play in host defense against viruses., IFN signaling and resistance to viral infections In this issue of the JCI, Bucciol, Moens, et al. describe the phenotypes of an extended cohort of patients with STAT2 deficiency, extending [...]

Published
2023
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21. Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab

Author

Chandrakasan, Shanmuganathan, primary, Jordan, Michael B., additional, Baker, Ashley, additional, Behrens, Edward M, additional, Bhatla, Deepika, additional, Chien, May, additional, Eckstein, Olive S, additional, Henry, Michael M, additional, Hermiston, Michelle L., additional, Hinson, Ashley, additional, Leiding, Jennifer W, additional, Oladapo, Abiola, additional, Patel, Sachit A., additional, Pednekar, Priti, additional, Ray, Anish, additional, Dávila Saldaña, Blachy J, additional, Sarangi, Susmita N., additional, Walkovich, Kelly, additional, Yee, John D, additional, Zoref-Lorenz, Adi, additional, and Allen, Carl E, additional

Published
2024
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23. Manipulating DNA damage-response signaling for the treatment of immune-mediated diseases

Author

McNally, Jonathan P, Millen, Scott H, Chaturvedi, Vandana, Lakes, Nora, Terrell, Catherine E, Elfers, Eileen E, Carroll, Kaitlin R, Hogan, Simon P, Andreassen, Paul R, Kanter, Julie, Allen, Carl E, Henry, Michael M, Greenberg, Jay N, Ladisch, Stephan, Hermiston, Michelle L, Joyce, Michael, Hildeman, David A, Katz, Jonathan D, and Jordan, Michael B

Subjects
Vaccine Related, Underpinning research, 1.1 Normal biological development and functioning, Development of treatments and therapeutic interventions, 5.1 Pharmaceuticals, Cancer, Inflammatory and immune system, Animals, Cell Cycle Checkpoints, DNA Damage, Disease Models, Animal, Drug Evaluation, Preclinical, Encephalomyelitis, Autoimmune, Experimental, Etoposide, Humans, Immune System Diseases, Lymphocyte Activation, Lymphohistiocytosis, Hemophagocytic, Mice, Mice, 129 Strain, Mice, Inbred BALB C, Mice, Inbred C57BL, Mice, Knockout, Mice, Transgenic, Multiple Sclerosis, Proto-Oncogene Proteins c-mdm2, Signal Transduction, T-Lymphocytes, Tumor Suppressor Protein p53, autoimmunity, immune regulation, DNA damage response, therapeutics
Abstract

Antigen-activated lymphocytes undergo extraordinarily rapid cell division in the course of immune responses. We hypothesized that this unique aspect of lymphocyte biology leads to unusual genomic stress in recently antigen-activated lymphocytes and that targeted manipulation of DNA damage-response (DDR) signaling pathways would allow for selective therapeutic targeting of pathological T cells in disease contexts. Consistent with these hypotheses, we found that activated mouse and human T cells display a pronounced DDR in vitro and in vivo. Upon screening a variety of small-molecule compounds, we found that potentiation of p53 (via inhibition of MDM2) or impairment of cell cycle checkpoints (via inhibition of CHK1/2 or WEE1) led to the selective elimination of activated, pathological T cells in vivo. The combination of these strategies [which we termed "p53 potentiation with checkpoint abrogation" (PPCA)] displayed therapeutic benefits in preclinical disease models of hemophagocytic lymphohistiocytosis and multiple sclerosis, which are driven by foreign antigens or self-antigens, respectively. PPCA therapy targeted pathological T cells but did not compromise naive, regulatory, or quiescent memory T-cell pools, and had a modest nonimmune toxicity profile. Thus, PPCA is a therapeutic modality for selective, antigen-specific immune modulation with significant translational potential for diverse immune-mediated diseases.

Published
2017

26. Genotype and functional correlates of disease phenotype in deficiency of adenosine deaminase 2 (DADA2)

Author

Lee, Pui Y., Kellner, Erinn S., Huang, Yuelong, Furutani, Elissa, Huang, Zhengping, Bainter, Wayne, Alosaimi, Mohammed F., Stafstrom, Kelsey, Platt, Craig D., Stauber, Tali, Raz, Somech, Tirosh, Irit, Weiss, Aaron, Jordan, Michael B., Krupski, Christa, Eleftheriou, Despina, Brogan, Paul, Sobh, Ali, Baz, Zeina, Lefranc, Gerard, Irani, Carla, Kilic, Sara S., El-Owaidy, Rasha, Lokeshwar, M.R., Pimpale, Pallavi, Khubchandani, Raju, Chambers, Eugene P., Chou, Janet, Geha, Raif S., Nigrovic, Peter A., and Zhou, Qing

Published
2020
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28. Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults

Author

Hines, Melissa R., von Bahr Greenwood, Tatiana, Beutel, Gernot, Beutel, Karin, Hays, J. Allyson, Horne, AnnaCarin, Janka, Gritta, Jordan, Michael B., van Laar, Jan A. M., Lachmann, Gunnar, Lehmberg, Kai, Machowicz, Rafal, Miettunen, Päivi, La Rosée, Paul, Shakoory, Bita, Zinter, Matt S., and Henter, Jan-Inge

Published
2021
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31. Survival in primary hemophagocytic lymphohistiocytosis 2016-2021: etoposide is better than its reputation

Author

Böhm, Svea, primary, Wustrau, Katharina, additional, Pachlopnik Schmid, Jana, additional, Prader, Seraina, additional, Ahlman, Martina, additional, Yacobovich, Joanne, additional, Beier, Rita, additional, Speckmann, Carsten, additional, Behnisch, Wolfgang, additional, Ifversen, Marianne, additional, Jordan, Michael B., additional, Marsh, Rebecca A, additional, Naumann-Bartsch, Nora, additional, Mauz-Koerholz, Christine, additional, Honig, Manfred, additional, Schulz, Ansgar S, additional, Malinowska, Iwona, additional, Hines, Melissa Ruth, additional, Nichols, Kim E, additional, Gil-Herrera, Juana, additional, Talano, Julie-An, additional, Crooks, Bruce, additional, Formankova, Renata, additional, Jorch, Norbert, additional, Bakhtiar, Shahrzad, additional, Kühnle, Ingrid, additional, Streiter, Monika, additional, Nathrath, Michaela, additional, Russo, Alexandra, additional, Duerken, Matthias, additional, Lang, Peter, additional, Lindemans, Caroline A., additional, Henter, Jan-Inge, additional, Lehmberg, Kai, additional, and Ehl, Stephan, additional

Published
2023
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33. Identifying and targeting pathogenic PI3K/AKT/ mTOR signaling in IL-6 blockade-refractory idiopathic multicentric Castleman disease

Author

Fajgenbaum, David C., Langan, Ruth-Anne, Japp, Alberto Sada, Partridge, Helen L., Pierson, Sheila K., Singh, Amrit, Arenas, Daniel J., Ruth, Jason R., Nabel, Christopher S., Stone, Katie, Okumura, Mariko, Schwarer, Anthony, Jose, Fabio Freire, Hamerschlak, Nelson, Wertheim, Gerald B., Jordan, Michael B., Cohen, Adam D., Krymskaya, Vera, Rubenstein, Arthur, Betts, Michael R., Kambayashi, Taku, van Rhee, Frits, and Uldrick, Thomas S.

Subjects
Precision medicine -- Analysis -- Health aspects, Interleukins -- Analysis -- Health aspects, Inflammation -- Care and treatment -- Analysis -- Health aspects, Tocilizumab -- Analysis -- Health aspects, C-reactive protein -- Analysis -- Health aspects, Immunohistochemistry -- Analysis -- Health aspects, Vascular endothelial growth factor -- Analysis -- Health aspects, Medical research -- Analysis -- Health aspects, Siltuximab -- Analysis -- Health aspects, T cells -- Analysis -- Health aspects, Cytokines, Endothelial growth factors, Proteomics, Thrombocytopenia, Rapamycin, Sirolimus, University research, Novels, Health care industry
Abstract

BACKGROUND. Idiopathic multicentric Castleman disease (iMCD) is a hematologic illness involving cytokine-induced lymphoproliferation, systemic inflammation, cytopenias, and life-threatening multi-organ dysfunction. The molecular underpinnings of interleukin-6 (IL-6) blockade-refractory patients remain unknown; no targeted therapies exist. In this study, we searched for therapeutic targets in IL-6 blockade-refractory iMCD patients with the thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin myelofibrosis, renal dysfunction, organomegaly (TAFRO) clinical subtype. METHODS. We analyzed tissues and blood samples from 3 IL-6 blockade-refractory iMCD-TAFRO patients. Cytokine panels, quantitative serum proteomics, flow cytometry of PBMCs, and pathway analyses were employed to identify novel therapeutic targets. To confirm elevated mTOR signaling, a candidate therapeutic target from the above assays, immunohistochemistry was performed for phosphorylated S6, a read-out of mTOR activation, in 3 iMCD lymph node tissue samples and controls. Proteomic, immunophenotypic, and clinical response assessments were performed to quantify the effects of administration of the mTOR inhibitor sirolimus. RESULTS. Studies of 3 IL-6 blockade-refractory iMCD cases revealed increased [CD8.sup.+] T cell activation, VEGF-A, and PI3K/Akt/mTOR pathway activity. Administration of sirolimus substantially attenuated [CD8.sup.+] T cell activation and decreased VEGF-A levels. Sirolimus induced clinical benefit responses in all 3 patients with durable and ongoing remissions of 66, 19, and 19 months. CONCLUSION. This precision medicine approach identifies PI3K/Akt/mTOR signaling as the first pharmacologically targetable pathogenic process in IL-6 blockade-refractory iMCD. Prospective evaluation of sirolimus in treatment- refractory iMCD is planned (NCT03933904). FUNDING. This study was supported by the Castleman's Awareness & Research Effort/Castleman Disease Collaborative Network, Penn Center for Precision Medicine, University Research Foundation, Intramural NIH funding, and the National Heart Lung and Blood Institute., Introduction Multicentric Castleman disease (MCD) is characterized by polyclonal lymphoproliferation, hypervascularized lymph nodes containing dysmorphic germinal centers, cytokine-driven systemic inflammation, cytopenias, and multi-organ dysfunction. The estimated annual incidence of MCD [...]

Published
2019
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34. CCR5 inhibitor as novel acute graft versus host disease prophylaxis in children and young adults undergoing allogeneic stem cell transplant: results of the phase II study

Author

Khandelwal, Pooja, Fukuda, Tsuyoshi, Teusink-Cross, Ashley, Kashuba, Angela D. M., Lane, Adam, Mehta, Parinda A., Marsh, Rebecca A., Jordan, Michael B., Grimley, Michael S., Myers, Kasiani C., Nelson, Adam S., El-Bietar, Javier, Chandra, Sharat, Bleesing, Jacob J., Krupski, Mary C., and Davies, Stella M.

Published
2020
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36. The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis

Author

Bode, Sebastian Fn, Ammann, Sandra, Al-Herz, Waleed, Bataneant, Mihaela, Dvorak, Christopher C, Gehring, Stephan, Gennery, Andrew, Gilmour, Kimberly C, Gonzalez-Granado, Luis I, Groß-Wieltsch, Ute, Ifversen, Marianne, Lingman-Framme, Jenny, Matthes-Martin, Susanne, Mesters, Rolf, Meyts, Isabelle, van Montfrans, Joris M, Pachlopnik Schmid, Jana, Pai, Sung-Yun, Soler-Palacin, Pere, Schuermann, Uta, Schuster, Volker, Seidel, Markus G, Speckmann, Carsten, Stepensky, Polina, Sykora, Karl-Walter, Tesi, Bianca, Vraetz, Thomas, Waruiru, Catherine, Bryceson, Yenan T, Moshous, Despina, Lehmberg, Kai, Jordan, Michael B, and Ehl, Stephan

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Rare Diseases, Hematology, Clinical Research, Aetiology, 2.1 Biological and endogenous factors, Inflammatory and immune system, Adolescent, Adult, Bacterial Infections, Child, Child, Preschool, Diagnosis, Differential, Europe, Female, Humans, Immunoglobulins, Intravenous, Immunologic Deficiency Syndromes, Immunologic Factors, Infant, Infant, Newborn, Killer Cells, Natural, Leishmaniasis, Lymphohistiocytosis, Hemophagocytic, Lymphoproliferative Disorders, Male, Mycoses, Opportunistic Infections, Registries, Steroids, T-Lymphocytes, Terminology as Topic, Virus Diseases, Inborn Errors Working Party of the EBMT, Cardiorespiratory Medicine and Haematology, Immunology, Cardiovascular medicine and haematology
Abstract

Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive therapy. However, these criteria also identify patients with infection-associated hemophagocytic inflammatory states lacking genetic defects typically predisposing to hemophagocytic lymphohistiocytosis. These patients include those with primary immunodeficiencies, in whom the pathogenesis of the inflammatory syndrome may be distinctive and aggressive immunosuppression is contraindicated. To better characterize hemophagocytic inflammation associated with immunodeficiencies, we combined an international survey with a literature search and identified 63 patients with primary immunodeficiencies other than cytotoxicity defects or X-linked lymphoproliferative disorders, presenting with conditions fulfilling current criteria for hemophagocytic lymphohistiocytosis. Twelve patients had severe combined immunodeficiency with

Published
2015

38. Hypomorphic caspase activation and recruitment domain 11 (CARD11) mutations associated with diverse immunologic phenotypes with or without atopic disease

Author

Dorjbal, Batsukh, Stinson, Jeffrey R., Ma, Chi A., Weinreich, Michael A., Miraghazadeh, Bahar, Hartberger, Julia M., Frey-Jakobs, Stefanie, Weidinger, Stephan, Moebus, Lena, Franke, Andre, Schäffer, Alejandro A., Bulashevska, Alla, Fuchs, Sebastian, Ehl, Stephan, Limaye, Sandhya, Arkwright, Peter D., Briggs, Tracy A., Langley, Claire, Bethune, Claire, Whyte, Andrew F., Alachkar, Hana, Nejentsev, Sergey, DiMaggio, Thomas, Nelson, Celeste G., Stone, Kelly D., Nason, Martha, Brittain, Erica H., Oler, Andrew J., Veltri, Daniel P., Leahy, T. Ronan, Conlon, Niall, Poli, Maria C., Borzutzky, Arturo, Cohen, Jeffrey I., Davis, Joie, Lambert, Michele P., Romberg, Neil, Sullivan, Kathleen E., Paris, Kenneth, Freeman, Alexandra F., Lucas, Laura, Chandrakasan, Shanmuganathan, Savic, Sinisa, Hambleton, Sophie, Patel, Smita Y., Jordan, Michael B., Theos, Amy, Lebensburger, Jeffrey, Atkinson, T. Prescott, Torgerson, Troy R., Chinn, Ivan K., Milner, Joshua D., Grimbacher, Bodo, Cook, Matthew C., and Snow, Andrew L.

Published
2019
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39. Hemophagocytic lymphohistiocytosis: A disorder of T cell activation, immune regulation, and distinctive immunopathology.

Author

Jordan, Michael B.

Subjects
*T cells, *HEMOPHAGOCYTIC lymphohistiocytosis, *IMMUNOPATHOLOGY, *MYELOID cells, *CANCER chemotherapy, *MACROPHAGE activation syndrome
Abstract

Summary: Hemophagocytic lymphohistiocytosis (HLH) is a disorder that has been recognized since the middle of the last century. In recent decades, increasing understanding of the genetic roots and pathophysiology of HLH has led to improved diagnosis and treatment of this once universally fatal disorder. HLH is best conceptualized as a maladaptive state of excessive T cell activation driving life‐threatening myeloid cell activation, largely via interferon‐gamma (IFN‐γ). In familial forms of HLH (F‐HLH), inherited defects of lymphocyte cytotoxic biology underlie excessive T cell activation, demonstrating the importance of the perforin/granzyme pathway as a negative feedback loop limiting acute T cell activation in response to environmental factors. HLH occurring in other contexts and without apparent inherited genetic predisposition remains poorly understood, though it may share some downstream aspects of pathophysiology including excessive IFN‐γ action and activation of innate immune effectors. Iatrogenic forms of HLH occurring after immune‐activating therapies for cancer are providing new insights into the potential toxicities of inadequately controlled T cell activation. Diagnosing HLH increasingly relies on context‐specific measures of T cell activation, IFN‐γ activity, and inflammation. Treatment of HLH largely relies on cytotoxic chemotherapy, though targeted therapies against T cells, IFN‐γ, and other cytokines are increasingly utilized. [ABSTRACT FROM AUTHOR]

Published
2024
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41. The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

Author

Shakoory, Bita, primary, Geerlinks, Ashley, additional, Wilejto, Marta, additional, Kernan, Kate, additional, Hines, Melissa, additional, Romano, Micol, additional, Piskin, David, additional, Ravelli, Angelo, additional, Sinha, Rashmi, additional, Aletaha, Daniel, additional, Allen, Carl, additional, Bassiri, Hamid, additional, Behrens, Edward M., additional, Carcillo, Joseph, additional, Carl, Linda, additional, Chatham, Winn, additional, Cohen, Jeffrey I., additional, Cron, Randy Q., additional, Drewniak, Erik, additional, Grom, Alexei A., additional, Henderson, Lauren A., additional, Horne, Annacarin, additional, Jordan, Michael B., additional, Nichols, Kim E., additional, Schulert, Grant, additional, Vastert, Sebastiaan, additional, Demirkaya, Erkan, additional, Goldbach‐Mansky, Raphaela, additional, de Benedetti, Fabrizio, additional, Marsh, Rebecca A., additional, and Canna, Scott W., additional

Published
2023
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42. HOW TO BE A MOVIE TODAY: YOU MAKE MOVIES THAT MATTER. YOU MAKE CHOICES THAT MEAN SOMETHING. YOU USE YOUR HOLLYWOOD POWER TO HELP PEOPLE ANY WAY YOU CAN. AND IN THIS WAY, YOU SHALL BE REMEMBERED, CASE STUDY: JORDAN, MICHAEL

Author

Jordan, Michael B.

Subjects
Mobile home industry -- Case studies, Parking lots, Fashion and beauty, General interest
Abstract

NEWARK ARTS HIGH SCHOOL, FOR THE PHOTO SHOOT Up ahead, there's a production moho (what industry folks call a mobile home) in the parking lot behind the school. The moho [...]

Published
2020

43. Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society

Author

Ehl, Stephan, Astigarraga, Itziar, von Bahr Greenwood, Tatiana, Hines, Melissa, Horne, AnnaCarin, Ishii, Eiichi, Janka, Gritta, Jordan, Michael B., La Rosée, Paul, Lehmberg, Kai, Machowicz, Rafal, Nichols, Kim E., Sieni, Elena, Wang, Zhao, and Henter, Jan-Inge

Published
2018
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44. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS)

Author

CTI Van Loosdregt, Research UMC Utrecht, Immuno/reuma onderzoek 1 (Vastert), Immunologie/Reumatologie, Child Health, Shakoory, Bita, Geerlinks, Ashley, Wilejto, Marta, Kernan, Kate, Hines, Melissa, Romano, Micol, Piskin, David, Ravelli, Angelo, Sinha, Rashmi, Aletaha, Daniel, Allen, Carl, Bassiri, Hamid, Behrens, Edward M., Carcillo, Joseph, Carl, Linda, Chatham, Winn, Cohen, Jeffrey I., Cron, Randy Q., Drewniak, Erik, Grom, Alexei A., Henderson, Lauren A., Horne, Annacarin, Jordan, Michael B., Nichols, Kim E., Schulert, Grant, Vastert, Sebastiaan, Demirkaya, Erkan, Goldbach-Mansky, Raphaela, De Benedetti, Fabrizio, Marsh, Rebecca A., Canna, Scott W., CTI Van Loosdregt, Research UMC Utrecht, Immuno/reuma onderzoek 1 (Vastert), Immunologie/Reumatologie, Child Health, Shakoory, Bita, Geerlinks, Ashley, Wilejto, Marta, Kernan, Kate, Hines, Melissa, Romano, Micol, Piskin, David, Ravelli, Angelo, Sinha, Rashmi, Aletaha, Daniel, Allen, Carl, Bassiri, Hamid, Behrens, Edward M., Carcillo, Joseph, Carl, Linda, Chatham, Winn, Cohen, Jeffrey I., Cron, Randy Q., Drewniak, Erik, Grom, Alexei A., Henderson, Lauren A., Horne, Annacarin, Jordan, Michael B., Nichols, Kim E., Schulert, Grant, Vastert, Sebastiaan, Demirkaya, Erkan, Goldbach-Mansky, Raphaela, De Benedetti, Fabrizio, Marsh, Rebecca A., and Canna, Scott W.

Published
2023

46. A Prospective Study of Alemtuzumab as a Second-Line Agent for Steroid-Refractory Acute Graft-versus-Host Disease in Pediatric and Young Adult Allogeneic Hematopoietic Stem Cell Transplantation

Author

Khandelwal, Pooja, Emoto, Chie, Fukuda, Tsuyoshi, Vinks, Alexander A., Neumeier, Lisa, Dandoy, Christopher E., El-Bietar, Javier, Chandra, Sharat, Davies, Stella M., Bleesing, Jacob J., Jordan, Michael B., Mehta, Parinda A., Jodele, Sonata, Grimley, Michael S., Kumar, Ashish, Myers, Kasiani C., and Marsh, Rebecca A.

Published
2016
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49. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Author

Emile, Jean-François, Abla, Oussama, Fraitag, Sylvie, Horne, Annacarin, Haroche, Julien, Donadieu, Jean, Requena-Caballero, Luis, Jordan, Michael B., Abdel-Wahab, Omar, Allen, Carl E., Charlotte, Frédéric, Diamond, Eli L., Egeler, R.Maarten, Fischer, Alain, Herrera, Juana Gil, Henter, Jan-Inge, Janku, Filip, Merad, Miriam, Picarsic, Jennifer, Rodriguez-Galindo, Carlos, Rollins, Barret J., Tazi, Abdellatif, Vassallo, Robert, and Weiss, Lawrence M.

Published
2016
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50. Contributors

Author

Abdel-Wahab, Omar, primary, Abrahm, Janet L., additional, Adams, Sharon, additional, Adewoye, Adeboye H., additional, Allen, Carl, additional, Ambinder, Richard F., additional, Anasetti, Claudio, additional, Anastasi, John, additional, Anderson, Julia A., additional, Antin, Joseph H., additional, Antony, Aśok C., additional, Araten, David J., additional, Armand, Philippe, additional, Armstrong, Gillian, additional, Armstrong, Scott A., additional, Arnold, Donald M., additional, Artz, Andrew S., additional, Awan, Farrukh T., additional, Baglin, Trevor P., additional, Benson, Don M., additional, Benz, Edward J., additional, Berliner, Nancy, additional, Bhagat, Govind, additional, Bhardwaj, Nina, additional, Bhatia, Ravi, additional, Bhatia, Smita, additional, Bhatt, Mihir D., additional, Bhatt, Vijaya Raj, additional, Bitan, Menachem, additional, Blinderman, Craig D., additional, Bollard, Catherine M., additional, Braun, Benjamin S., additional, Brenner, Malcolm K., additional, Brittenham, Gary M., additional, Brodsky, Robert A., additional, Brown, Myles, additional, Broxmeyer, Hal E., additional, Brummel-Ziedins, Kathleen, additional, Brunner, Andrew M., additional, Buadi, Francis K., additional, Burkhardt, Birgit, additional, Burns, Melissa, additional, Byrd, John C., additional, Caimi, Paolo F., additional, Caligiuri, Michael A., additional, Canavan, Michelle, additional, Cantor, Alan B., additional, Carcao, Manuel, additional, Carroll, Michael C., additional, Carty, Shannon A., additional, Castillo, Jorge J., additional, Chan, Anthony K.C., additional, Chapin, John, additional, Chiu, April, additional, Chute, John P., additional, Clark, David B., additional, Coates, Thomas D., additional, Cogle, Christopher R., additional, Connell, Nathan T., additional, Cooke, Elizabeth, additional, Cooley, Sarah, additional, Corradini, Paolo, additional, Creager, Mark A., additional, Creger, Richard J., additional, Cromwell, Caroline, additional, Crowther, Mark A., additional, Cushing, Melissa M., additional, Cutler, Corey, additional, Dang, Chi V., additional, Danial, Nika N., additional, Dave, Sandeep S., additional, DeCaprio, James A., additional, Dinauer, Mary C., additional, Dinner, Shira, additional, Diz-Küçükkaya, Reyhan, additional, Dodd, Roger Y., additional, Donato, Michele L., additional, Dorshkind, Kenneth, additional, Dotti, Gianpietro, additional, Dror, Yigal, additional, Dunleavy, Kieron, additional, Dvorak, Christopher C., additional, Ebert, Benjamin L., additional, Eck, Michael J., additional, Eikelboom, John W., additional, Epperla, Narendranath, additional, Ershler, William B., additional, Evans, William E., additional, Faderl, Stefan, additional, Ferrara, James L.M., additional, Filipovich, Alexandra Hult, additional, Fischer, Martin, additional, Fredenburgh, James C., additional, Friedman, Kenneth D., additional, Fuchs, Ephraim, additional, Fuller, Stephen J., additional, Gailani, David, additional, Galipeau, Jacques, additional, Gallagher, Patrick G., additional, Ganapathi, Karthik A., additional, Gardner, Lawrence B., additional, Gee, Adrian P., additional, Gerson, Stanton L., additional, Gertz, Morie A., additional, Giardina, Patricia J., additional, Gibson, Christopher J., additional, Golan, Karin, additional, Golub, Todd R., additional, Gonzales, Matthew J., additional, Gotlib, Jason, additional, Gottschalk, Stephen, additional, Grant, Marianne A., additional, Graubert, Timothy A., additional, Gregg, Xylina T., additional, Gribben, John G., additional, Gross, Dawn M., additional, Gruber, Tanja A., additional, Guitart, Joan, additional, Gurbuxani, Sandeep, additional, Gur-Cohen, Shiri, additional, Gutierrez, Alejandro, additional, Hamadani, Mehdi, additional, Hari, Parameswaran N., additional, Hartwig, John H., additional, Hayman, Suzanne R., additional, Hayward, Catherine P.M., additional, Hebbel, Robert P., additional, Heslop, Helen E., additional, Hillis, Christopher, additional, Hillyer, Christopher D., additional, Ho, Karin, additional, Hockenbery, David M., additional, Hoffman, Ronald, additional, Hogg, Kerstin E., additional, Holtan, Shernan G., additional, Horny, Hans-Peter, additional, Hsu, Yen-Michael S., additional, Hunter, Zachary R., additional, Huntington, James A., additional, Iancu-Rubin, Camelia, additional, Iqbal, Ali, additional, Isenman, David E., additional, Israels, Sara J., additional, Italiano, Joseph E., additional, Jaffe, Elaine S., additional, Jaffer, Iqbal H., additional, Jagannath, Sundar, additional, Jäger, Ulrich, additional, Jain, Nitin, additional, James, Paula, additional, Jeha, Sima, additional, Jordan, Michael B., additional, Josephson, Cassandra D., additional, Jung, Moonjung, additional, Kager, Leo, additional, Kambayashi, Taku, additional, Kanakry, Jennifer A., additional, Kantarjian, Hagop M., additional, Kaplan, Jason, additional, Karafin, Matthew S., additional, Karsan, Aly, additional, Kaufman, Randal J., additional, Kaufman, Richard M., additional, Keller, Frank G., additional, Kelly, Kara M., additional, Kessler, Craig M., additional, Key, Nigel S., additional, Keyzner, Alla, additional, Khandoga, Alexander G., additional, Khanna-Gupta, Arati, additional, Khatib-Massalha, Eman, additional, Klein, Harvey G., additional, Knoechel, Birgit, additional, Kollet, Orit, additional, Konkle, Barbara A., additional, Kontoyiannis, Dimitrios P., additional, Koreth, John, additional, Koretzky, Gary A., additional, Kotecha, Dipak, additional, Kremyanskaya, Marina, additional, Kumari, Anju, additional, Kuzel, Timothy M., additional, Küppers, Ralf, additional, Lacy, Martha Q., additional, Ladas, Elana, additional, Landier, Wendy, additional, Lapid, Kfir, additional, Lapidot, Tsvee, additional, Larson, Peter J., additional, Levi, Marcel, additional, Lewis, Russell E., additional, Liebman, Howard A., additional, Lillicrap, David, additional, Lim, Wendy, additional, Lin, Judith C., additional, Lindblad, Robert, additional, Lip, Gregory Y.H., additional, Little, Jane A., additional, Lohr, Jens G., additional, López, José A., additional, Luscinskas, Francis W., additional, Maciejewski, Jaroslaw P., additional, Majhail, Navneet S., additional, Manches, Olivier, additional, Mandle, Robert J., additional, Mann, Kenneth G., additional, Manno, Catherine S., additional, Marcogliese, Andrea N., additional, Mariani, Guglielmo, additional, Marincola, Francesco M., additional, Mascarenhas, John, additional, Massberg, Steffen, additional, McEver, Rodger P., additional, McGrath, Emer, additional, McKinney, Matthew S., additional, Mehta, Rohtesh S., additional, Mentzer, William C., additional, Merlini, Giampaolo, additional, Merryman, Reid, additional, Michel, Marc, additional, Migliaccio, Anna Rita, additional, Miller, Jeffrey S., additional, Mims, Martha P., additional, Mondoro, Traci Heath, additional, Moorehead, Paul, additional, Muniz, Luciana R., additional, Munshi, Nikhil C., additional, Najfeld, Vesna, additional, Nayak, Lalitha, additional, Nazy, Ishac, additional, Neff, Anne T., additional, Ness, Paul M., additional, Notarangelo, Luigi D., additional, O'Brien, Sarah H., additional, O'Connor, Owen A., additional, O'Donnell, Martin, additional, Olson, Amanda, additional, Orkin, Stuart H., additional, Pai, Menaka, additional, Pai, Sung-Yun, additional, Paidas, Michael, additional, Panch, Sandhya R., additional, Pande, Reena L., additional, Papayannopoulou, Thalia, additional, Parikh, Rahul, additional, Petersdorf, Effie W., additional, Peterson, Shane E., additional, Pittaluga, Stefania, additional, Ponce, Doris M., additional, Popolo, Laura, additional, Prchal, Josef T., additional, Pui, Ching-Hon, additional, Puigserver, Pere, additional, Rak, Janusz, additional, Ramos, Carlos A., additional, Rand, Jacob H., additional, Rand, Margaret L., additional, Rao, Dinesh S., additional, Ravandi, Farhad, additional, Rawlings, David J., additional, Reddy, Pavan, additional, Reding, Mark T., additional, Reiter, Andreas, additional, Rice, Lawrence, additional, Riese, Matthew J., additional, Ritchey, Arthur Kim, additional, Roberts, David J., additional, Roman, Elizabeth, additional, Rooney, Cliona M., additional, Rosen, Steven T., additional, Rosenthal, David S., additional, Rossmann, Marlies P., additional, Rot, Antal, additional, Rowley, Scott D., additional, Rubnitz, Jeffrey E., additional, Rydz, Natalia, additional, Salama, Mohamed E., additional, Sauk, Steven, additional, Saunthararajah, Yogen, additional, Savage, William, additional, Scadden, David, additional, Schaefer, Kristen G., additional, Schiffman, Fred, additional, Schneidewend, Robert, additional, Schrier, Stanley L., additional, Schuchman, Edward H., additional, Scullion, Bridget Fowler, additional, Selvaggi, Kathy J., additional, Senoo, Keitaro, additional, Shaheen, Montaser, additional, Shaz, Beth H., additional, Shelburne, Samuel A., additional, Shpall, Elizabeth J., additional, Shurin, Susan B., additional, Siegal, Deborah, additional, Silberstein, Leslie E., additional, Silberstein, Lev, additional, Silverstein, Roy L., additional, Sloan, Steven R., additional, Smith, Franklin O., additional, Smith, James W., additional, Smith, Katy, additional, Steensma, David P., additional, Steinberg, Martin H., additional, Stock, Wendy, additional, Storry, Jill R., additional, Stramer, Susan L., additional, Strauss, Ronald G., additional, Stroncek, David F., additional, Taylor, Justin, additional, Thota, Swapna, additional, Treon, Steven P., additional, Tulpule, Anil, additional, Valdes, Roberto Ferro, additional, Valent, Peter, additional, Vedantham, Suresh, additional, Vercellotti, Gregory M., additional, Verneris, Michael R., additional, Vichinsky, Elliott P., additional, von Andrian, Ulrich H., additional, Vose, Julie M., additional, Wagner, Andrew J., additional, Wang, Ena, additional, Wang, Jia-huai, additional, Warkentin, Theodore E., additional, Wasserstein, Melissa P., additional, Webster, Ann, additional, Weisdorf, Daniel J., additional, Weitz, Jeffrey I., additional, Westhoff, Connie M., additional, Wheeler, Allison P., additional, Widick, Page, additional, Wiley, James S., additional, William, Basem M., additional, Williams, David A., additional, Wilson, Wyndham H., additional, Wolfe, Joanne, additional, Wolgast, Lucia R., additional, Wood, Deborah, additional, Wu, Jennifer, additional, Yahalom, Joachim, additional, Yee, Donald L., additional, Younes, Anas, additional, Young, Neal S., additional, and Zeller, Michelle P., additional

Published
2018
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