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Engraftment syndrome

Nirali N Shah, Theresa M Watson, Bonnie Yates, David J Liewehr, Seth M Steinberg, David Jacobsohn, Terry J Fry
BACKGROUND: Diagnosis of engraftment syndrome (ES) following allogeneic hematopoietic stem cell transplantation (HSCT) can be a challenge due to the systemic presentation and alternative etiologies. With a goal of establishing biomarkers to more accurately distinguish ES, we prospectively analyzed levels of cytokines during HSCT. PROCEDURES: We performed a prospective study of children ≤21 years who underwent allogeneic HSCT. Blood samples for interleukin (IL)-6, IL-8, IL-10, IL-1b, IL-12p70, interferon-γ, tumor necrosis factor alpha (TNF-α) and procalcitonin were obtained from each subject prior to conditioning, at day 0, and then biweekly through engraftment and at days 30, 60 and 100...
October 20, 2016: Pediatric Blood & Cancer
Young-Ho Lee, Wee-Jin Rah
No abstract text is available yet for this article.
September 2016: Blood Research
Alberto Mussetti, Nancy A Kernan, Susan E Prockop, Andromachi Scaradavou, Rachel Lehrman, Julianne M Ruggiero, Kevin Curran, Rachel Kobos, Richard O'Reilly, Farid Boulad
Nonmalignant hematologic disorders (NMHD) of childhood comprise a variety of disorders, including acquired severe aplastic anemia and inherited marrow failure syndromes. Patients with high-risk NMHD without matched related donors fare poorly with allogeneic hematopoietic alternative donor stem cell transplantation (allo-HSCT) and are at high risk for developing graft-versus-host disease following unmodified grafts. The authors retrospectively analyzed data on 18 patients affected by NMHD, lacking a human leukocyte antigen (HLA)-identical sibling donor, who underwent an alternative donor allo-HSCT at their institution between April 2005 and May 2013...
October 7, 2016: Pediatric Hematology and Oncology
Mark Wunderlich, Courtney Stockman, Mahima Devarajan, Navin Ravishankar, Christina Sexton, Ashish R Kumar, Benjamin Mizukawa, James C Mulloy
Transgenic expression of key myelosupportive human cytokines in immune-deficient mice corrects for the lack of cross-species activities of stem cell factor (SCF), IL-3, and GM-CSF. When engrafted with human umbilical cord blood (UCB), these triple-transgenic mice produce BM and spleen grafts with much higher myeloid composition, relative to nontransgenic controls. Shortly after engraftment with UCB, these mice develop a severe, fatal macrophage activation syndrome (MAS) characterized by a progressive drop in rbc numbers, increased reticulocyte counts, decreased rbc half-life, progressive cytopenias, and evidence of chronic inflammation, including elevated human IL-6...
September 22, 2016: JCI Insight
Prashant Sharma, Shivani S Shinde, Moussab Damlaj, Mehrdad Hefazi Rorghabeh, Shahrukh K Hashmi, Mark R Litzow, William J Hogan, Naseema Gangat, Michelle A Elliott, Aref Al-Kali, Ayalew Tefferi, Mrinal M Patnaik
MDS/MPN (myelodysplastic syndrome/myeloproliferative neoplasm) overlap syndromes are myeloid malignancies for which allogeneic hematopoietic stem cell transplant (allo-HSCT) is potentially curative. We describe transplant outcomes of 43 patients - 35 with chronic myelomonocytic leukemia, CMML (of which 17 had blast transformation, BT) and eight with MDS/MPN-unclassifiable (MDS/MPN,U). At median follow-up of 21 months, overall survival (OS), cumulative incidence of relapse (CIR) and non-relapse mortality (NRM) were 55%, 29%, and 25% respectively in CMML without BT and 47%, 40%, and 34% respectively in CMML with BT...
August 11, 2016: Leukemia & Lymphoma
Robert Chiesa, Robert F Wynn, Paul Veys
PURPOSE OF REVIEW: This review summarizes the main results of haematopoietic stem cell transplantation (HSCT) in selected inborn errors of metabolism (IEMs). RECENT FINDINGS: Early diagnosis and immediate referral to an IEM specialist is of paramount importance to improve clinical outcome: patients who are transplanted early or in their presymptomatic phase generally achieve better correction of their somatic symptoms and neurocognitive development. Long-term outcome in children with Hurler syndrome is influenced by age at HSCT, baseline clinical status and post-HSCT enzyme levels...
November 2016: Current Opinion in Hematology
T Ikezoe, J Yang, C Nishioka, K Umezawa, A Yokoyama
Recombinant human soluble thrombomodulin (rTM) counteracted capillary leakage and alleviated edema in individuals with sinusoidal obstruction syndrome and engraftment syndrome after hematopoietic stem cell transplantation. We previously showed that rTM increased levels of antiapoptotic protein Mcl-1 and protected endothelial cells from calcineurin inhibitor cyclosporine A (CsA)-induced apoptosis. However, the molecular mechanisms by which rTM enhances barrier function in vascular endothelial cells remain unknown...
September 19, 2016: Bone Marrow Transplantation
Gordon Cook, Simona Iacobelli, Anja van Biezen, Dimitris Ziagkos, Veronique LeBlond, Julie Abraham, Grant McQuaker, Stefan Schoenland, Alessandro Rambaldi, Kazimierz Halaburda, Maria Rovira, Simona Sica, Jenny Byrne, Ramon Garcia Sanz, Arnon Nagler, Niels W C J van de Donk, Marjatta Sinisalo, Mark Cook, Nicolaus Kröger, Theo De Witte, Curly Morris, Laurent Garderet
POEMS syndrome is a rare para-neoplastic syndrome secondary to a plasma cell dyscrasia. Effective treatment can control the disease-related symptom complex. We describe the clinical outcome of autologous stem cell transplantation for patients with POEMS syndrome, determining the impact of patient and disease-specific factors on prognosis. 127 patients underwent an autologous stem cell transplantation between 1997-2010 with a median age of 50 years (range 26-69). The median time from diagnosis to ASCT was 7...
September 15, 2016: Haematologica
Petra Becker, Arnd Schwebig, Susanne Brauninger, Heike Bialleck, Beate Luxembourg, Miriam Schulz, Chrysanthi Tsamadou, Markus Wiesneth, Peter Reinhardt, Joannis Mytilineos, Christian Seidl, Sreekanth Gattu, Natalia Kaliakina, Pritibha Singh, Hubert Schrezenmeier, Erhard Seifried, Halvard Bonig
BACKGROUND: Biosimilar granulocyte-colony-stimulating factors (G-CSFs) have been available in the European Union since 2008, and Sandoz' biosimilar filgrastim was approved in the United States in March 2015 for all of the reference product's indications except acute radiation syndrome. Biosimilar G-CSFs have been largely embraced by the medical community, except for some reservations about healthy-donor stem cell mobilization, for which use outside of clinical studies was cautioned against by some members of the scientific community...
September 16, 2016: Transfusion
Krzysztof Marycz, Katarzyna Kornicka, Monika Marędziak, Paweł Golonka, Jakub Nicpoń
Adipose-derived mesenchymal stem cells (ASC) hold great promise in the treatment of many disorders including musculoskeletal system, cardiovascular and/or endocrine diseases. However, the cytophysiological condition of cells, used for engraftment seems to be fundamental factor that might determine the effectiveness of clinical therapy. In this study we investigated growth kinetics, senescence, accumulation of oxidative stress factors, mitochondrial biogenesis, autophagy and osteogenic differentiation potential of ASC isolated from horses suffered from equine metabolic syndrome (EMS)...
September 14, 2016: Journal of Cellular and Molecular Medicine
Filippo Milano, Fabiola Merriam, Ian Nicoud, Jianqiang Li, Ted A Gooley, Shelly Heimfeld, Suzan Imren, Colleen Delaney
: : The hematopoietic syndrome of acute radiation syndrome (h-ARS) is characterized by severe bone marrow aplasia, resulting in a significant risk for bleeding, infections, and death. To date, clinical management of h-ARS is limited to supportive care dictated by the level of radiation exposure, with a high incidence of mortality in those exposed to high radiation doses. The ideal therapeutic agent would be an immediately available, easily distributable single-agent therapy capable of rapid in vivo hematopoietic reconstitution until recovery of autologous hematopoiesis occurs...
September 13, 2016: Stem Cells Translational Medicine
Anastasia Gromova, Dmitry A Voronov, Miya Yoshida, Suharika Thotakura, Robyn Meech, Darlene A Dartt, Helen P Makarenkova
: : In humans, the lacrimal gland (LG) is the primary contributor to the aqueous layer of the tear film. Production of tears in insufficient quantity or of inadequate quality may lead to aqueous-deficiency dry eye (ADDE). Currently there is no cure for ADDE. The development of strategies to reliably isolate LG stem/progenitor cells from the LG tissue brings great promise for the design of cell replacement therapies for patients with ADDE. We analyzed the therapeutic potential of epithelial progenitor cells (EPCPs) isolated from adult wild-type mouse LGs by transplanting them into the LGs of TSP-1(-/-) mice, which represent a novel mouse model for ADDE...
August 15, 2016: Stem Cells Translational Medicine
Nelli Bejanyan, John Rogosheske, Todd E DeFor, Aleksandr Lazaryan, Mukta Arora, Shernan G Holtan, Pamala A Jacobson, Margaret L MacMillan, Michael R Verneris, Bruce R Blazar, Daniel J Weisdorf, John E Wagner, Claudio G Brunstein
The use of calcineurin inhibitors (CNIs) to reduce the risk of graft-versus-host disease (GVHD) after hematopoietic cell transplantation (HCT) requires intensive post-transplantation toxicity monitoring. Sirolimus-based GVHD prophylaxis is associated with a favorable toxicity profile and requires less intensive monitoring. However, the efficacy of sirolimus-based regimen compared with CNI-based regimen has not been evaluated in the setting of reduced-intensity conditioning (RIC) double umbilical cord blood (UCB) HCT...
November 2016: Biology of Blood and Marrow Transplantation
Shahin Rafii, Michael Ginsberg, Joseph Scandura, Jason M Butler, Bi-Sen Ding
Current therapeutic approaches for treatment of exposure to radiation involve the use of antioxidants, chelating agents, recombinant growth factors and transplantation of stem cells (e.g., hematopoietic stem cell transplantation). However, exposure to high-dose radiation is associated with severe damage to the vasculature of vital organs, often leading to impaired healing, tissue necrosis, thrombosis and defective regeneration caused by aberrant fibrosis. It is very unlikely that infusion of protective chemicals will reverse severe damage to the vascular endothelial cells (ECs)...
August 2016: Radiation Research
Mary H Foster, Elizabeth S Buckley, Benny J Chen, Kwan-Ki Hwang, Amy G Clark
Autoantibodies mediate organ destruction in multiple autoimmune diseases, yet their origins in patients remain poorly understood. To probe the genetic origins and structure of disease-associated autoantibodies, we engrafted immunodeficient mice with human CD34+ hematopoietic stem cells and immunized with the non-collagenous-1 (NC1) domain of the alpha3 chain of type IV collagen. This antigen is expressed in lungs and kidneys and is targeted by autoantibodies in anti-glomerular basement membrane (GBM) nephritis and Goodpasture syndrome (GPS), prototypic human organ-specific autoimmune diseases...
August 2016: Molecular Immunology
Reut Shainer, Osnat Almogi-Hazan, Arye Berger, Liad Hinden, Martin Mueller, Chaya Brodie, Cedric Simillion, Michael Paidas, Eytan R Barnea, Reuven Or
Acute Radiation Syndrome (ARS) may lead to cancer and death and has few effective countermeasures. Efficacy of synthetic PIF treatment was demonstrated in preclinical autoimmune and transplantation models. PIF protected against inflammation and mortality following lethal irradiation in allogeneic bone marrow transplant (BMT) model. Herein, we demonstrate that PIF imparts comprehensive local and systemic protection against lethal and sub-lethal ARS in murine models. PIF treatment 2 h after lethal irradiation led to 100% survival and global hematopoietic recovery at 2 weeks after therapy...
July 16, 2016: Oncotarget
Ahmad Alhuraiji, Hazza Alzahrani, Fahad Al Mohareb, Naeem Chaudhri, Fahad Alsharif, Said Mohamed, Walid Rasheed, Ghuzayel Aldawsari, Syed Osman Ahmed, Mahmoud Aljurf
OBJECTIVES: Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital anomalies and increased risk of cancer. Hematopoietic stem cell transplant is a potentially curative modality for bone marrow failure in Fanconi anemia patients. Here, we report our center's experience regarding adolescent and young adult patients with Fanconi anemia and hematopoietic stem cell transplant. MATERIALS AND METHODS: We conducted a retrospective patient record analyses of patients who presented at our center from 1988 to 2014...
July 22, 2016: Experimental and Clinical Transplantation
Jared W Glenn, Mark J Cody, Meghann P McManus, Michael A Pulsipher, Joshua D Schiffman, Christian Con Yost
Overwhelming infection causes significant morbidity and mortality among patients treated with bone marrow transplantation (BMT) for primary immune deficiencies, syndromes of bone marrow failure, or cancer. The polymorphonuclear leukocyte (PMN; neutrophil) is the first responder to microbial invasion and acts within the innate immune system to contain and clear infections. PMNs contain, and possibly clear, infections in part by forming neutrophil extracellular traps (NETs). NETs are extensive lattices of extracellular DNA and decondensed chromatin decorated with antimicrobial proteins and degradative enzymes, such as histones, myeloperoxidase, and neutrophil elastase...
2016: Frontiers in Immunology
Hawk Kim, Je-Hwan Lee, Young-Don Joo, Sung-Hwa Bae, Sang Min Lee, Jae-Cheol Jo, Yunsuk Choi, Jung-Hee Lee, Dae-Young Kim, Hun Mo Ryoo, Kyoo-Hyung Lee
We performed a study on allogeneic hematopoietic cell transplantation (alloHCT) from an HLA-haplo-identical familial donor (haploFD) using a busulfan-fludarabine-antithymocyte globulin conditioning regimen for severe aplastic anemia (sAA) and hypoplastic myelodysplastic syndrome. For the comparison between a haploFD and an alternative donor (AD; matched unrelated or partially matched donor) for sAA in adults, we collected haploFD data retrospectively and prospectively. Forty-eight AD cases were selected for the comparison with 16 haploFD cases...
2016: Acta Haematologica
Egil S Blix, Anne Husebekk
Endothelial cells (EC) are crucial for normal angiogenesis and important for patients with leukemia, myeloma, and lymphoma during and after hematopoietic stem cell transplantation (HSCT). Knowledge of endothelial dysfunction in hematologic malignancies is provided by translational studies analyzing soluble endothelial markers, morphologic and functional changes of EC cultured in patients' sera or enumeration of circulating EC or endothelial progenitor cells (EPC). EC are important for stem cell homing and maintenance...
July 10, 2016: Leukemia & Lymphoma
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