Article
Immunology
Geraldine Blanchard-Rohner, Anna Peirolo, Ludivine Coulon, Christian Korff, Judit Horvath, Pierre R. Burkhard, Fabienne Gumy-Pause, Emmanuelle Ranza, Peter Jandus, Harpreet Dibra, Alexander Malcolm R. Taylor, Joel Fluss
Summary: Ataxia-telangiectasia (A-T) is a neurodegenerative and primary immunodeficiency disorder characterized by various symptoms. This study presents a case series highlighting the phenotypic variability of A-T and emphasizes the importance of early diagnosis of variant A-T and classical A-T.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Infectious Diseases
Robina Aerts, Toine Mercier, Marielle Beckers, Helene Schoemans, Katrien Lagrou, Johan Maertens
Summary: This study investigated the use of PCR screening and preemptive treatment in Toxoplasma-seropositive allogeneic hematopoietic cell transplant recipients. The results showed that the overall 1-year cumulative incidence of toxoplasmosis was 4.1% and it was 8.8% in seropositive recipients. Reactivation was associated with second transplant, myeloablative conditioning, total body irradiation, acute graft-versus-host disease, and use of high-dose corticosteroids. Multivariate analysis identified acute graft-versus-host disease as the sole independent predictor for reactivation.
CLINICAL MICROBIOLOGY AND INFECTION
(2022)
Article
Immunology
Ulrich Baumann, Johannes H. Schulte, Jonathan P. Gross, Rita Beier, Marius Ludwig, Volker Wahn, Jorg Hofmann, Britta Maecker-Kolhoff, Martin Sauer, Petra Kaiser-Labusch, Negin Karimian, Ulrike Blume-Peytavi, Franziska Ghoreschi, Hagen Ott, Ludmila Perelygina, Christian Klemann, Oliver Blankenstein, Horst von Bernuth, Renate Krueger
Summary: We report two patients with DNA repair disorders who developed destructive skin granulomas after receiving live-attenuated rubella vaccinations. Both patients exhibited reduced naive T cells. Hematopoietic stem cell transplantation resulted in rapid resolution of skin lesions. However, one patient with severe complications died 6 months after HSCT. Newborn screening may be beneficial in preventing patients with moderate T-cell deficiencies from receiving rubella vaccine that may lead to granulomas.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Alexandra Laberko, Anna Mukhina, Elena Machneva, Olga Pashchenko, Tatiana Bykova, Larisa Vahonina, Gleb Bronin, Yulia Skvortsova, Elena Skorobogatova, Irina Kondratenko, Larisa Fechina, Anna Shcherbina, Ludmila Zubarovskaya, Dmitry Balashov, Alexander Rumiantsev
Summary: This study collected and analyzed data on HSCT activity in IEI patients in Russia. The results showed that the number of IEI patients receiving HSCT has increased significantly over the years, and there has been a change in the distribution of IEI diagnoses. The study suggests the need for additional transplant beds for IEI in Russia.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Stefan Zielen, Ruth Pia Duecker, Sandra Woelke, Helena Donath, Sharhzad Bakhtiar, Aileen Buecker, Hermann Kreyenberg, Sabine Huenecke, Peter Bader, Nizar Mahlaoui, Stephan Ehl, Sabine M. El-Helou, Barbara Pietrucha, Alessandro Plebani, Michiel van der Flier, Koen van Aerde, Sara S. Kilic, Shereen M. Reda, Larysa Kostyuchenko, Elizabeth McDermott, Nermeen Galal, Claudio Pignata, Juan Luis Santos Perez, Hans-Juergen Laws, Tim Niehues, Necil Kutukculer, Markus G. Seidel, Laura Marques, Peter Ciznar, John David M. Edgar, Pere Soler-Palacin, Horst von Bernuth, Renate Krueger, Isabelle Meyts, Ulrich Baumann, Maria Kanariou, Bodo Grimbacher, Fabian Hauck, Dagmar Graf, Luis Ignacio Gonzalez Granado, Seraina Prader, Ismail Reisli, Mary Slatter, Carlos Rodriguez-Gallego, Peter D. Arkwright, Claire Bethune, Elena Deripapa, Svetlana O. Sharapova, Kai Lehmberg, E. Graham Davies, Catharina Schuetz, Gerhard Kindle, Ralf Schubert
Summary: The study analyzed mortality and immunity data of patients with ataxia-telangiectasia (A-T) in relation to IgA deficiency, finding that patients with IgA deficiency have significantly lower survival rates, lymphocyte counts, and subsets compared to A-T patients without IgA deficiency. This suggests that IgA deficiency may indicate a poorer prognosis for classical A-T patients.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Article
Immunology
Raena Kaur, Duncan Purtill, Julian Cooney, Paul Cannell, Matthew Wright, Tandy-Sue Copeland, Matthew Mcguire, Peter Boan
Summary: The study found that Letermovir was more effective than Valganciclovir in controlling cytomegalovirus viremia caused by cytopenias and improving cytopenias after alloHCT.
TRANSPLANT INFECTIOUS DISEASE
(2023)
Article
Immunology
Yue Lu, Jian-Ping Zhang, Yan-Li Zhao, Min Xiong, Rui-Juan Sun, Xing-Yu Cao, Zhi-Jie Wei, Jia-Rui Zhou, De-Yan Liu, Jun-Fang Yang, Xian Zhang, Dao-Pei Lu, Peihua Lu
Summary: This study retrospectively analyzed 199 hematological malignancy patients who received a second allogeneic stem cell transplantation (allo-HSCT2) after relapse following the first allo-HSCT (allo-HSCT1), aiming to evaluate prognostic factors. The results showed that disease status of MRD-negative complete remission, HCT-CI score of 0 prior to allo-HSCT2, and a new mismatched haplotype donor were predictive factors for improved overall survival and leukemia-free survival. Based on these factors, a predictive scoring system was developed.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Hematology
Shahrzad Bakhtiar, Emilia Salzmann-Manrique, Helena Donath, Sandra Woelke, Ruth P. Duecker, Stefanie Fritzemeyer, Ralf Schubert, Sabine Huenecke, Matthias Kieslich, Thomas Klingebiel, Peter Bader, Stefan Zielen
Summary: Ataxia-telangiectasia (A-T) is a hereditary immune system disorder with neurodegeneration that presents with ataxic gait and progressive muscle weakness. Patients with A-T have a significantly increased risk of cancer, particularly hematological malignancies, which can lead to early mortality. The study highlights the importance of cancer surveillance, prevention, and optimal treatment in this patient population.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Immunology
Satoshi Miyamoto, Katsutsugu Umeda, Mio Kurata, Akira Nishimura, Masakatsu Yanagimachi, Masataka Ishimura, Maho Sato, Tomonari Shigemura, Motohiro Kato, Yoji Sasahara, Akihiro Iguchi, Takashi Koike, Yoshiyuki Takahashi, Michiko Kajiwara, Masami Inoue, Yoshiko Hashii, Hiromasa Yabe, Koji Kato, Yoshiko Atsuta, Kohsuke Imai, Tomohiro Morio
Summary: This nationwide study in Japan found that umbilical cord blood (UCB) transplantation can be a potential donor source for SCID patients. Additionally, early age at HCT and absence of active infection are critical for a better prognosis in SCID patients.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Article
Multidisciplinary Sciences
Ka-Won Kang, Min Ji Jeon, Eun Sang Yu, Dae Sik Kim, Byung-Hyun Lee, Se Ryeon Lee, Chul Won Choi, Yong Park, Byung Soo Kim, Hwa Jung Sung
Summary: This study retrospectively analyzed the patterns and survival of CMV reactivation in patients undergoing pre-emptive therapy after allo-HSCT, and assessed high-risk patients who could benefit from more aggressive CMV prophylaxis in endemic areas.
Review
Oncology
Kristine Doney, Wendy Leisenring, Hannah Linden
Summary: This study compared the outcomes of allogeneic stem cell transplantation in breast cancer survivors to a control group. The results showed that a history of breast cancer did not significantly affect the transplant outcomes, indicating that breast cancer survivors should not be excluded from consideration for this treatment.
BREAST CANCER RESEARCH AND TREATMENT
(2022)
Review
Immunology
Michelle K. Yong, Monica A. Slavin, Roy F. Chemaly, Genovefa A. Papanicolaou
Summary: In recent years, there has been a significant shift in the preferred strategy for preventing CMV in at-risk populations in alloHCT. Letermovir prophylaxis is now offered to all CMV seropositive recipients, resulting in significant reductions in CMV infection and other important outcomes. However, there are limitations such as delayed onset CMV infections and increased drug costs.
TRANSPLANT INFECTIOUS DISEASE
(2023)
Review
Immunology
Tannaz Moeini Shad, Reza Yazdani, Parisa Amirifar, Samaneh Delavari, Marzieh Heidarzadeh Arani, Seyed Alireza Mahdaviani, Mahnaz Sadeghi-Shabestari, Asghar Aghamohammadi, Nima Rezaei, Hassan Abolhassani
Summary: Ataxia-telangiectasia (AT) is a rare autosomal recessive neurological disorder, and some patients may present with atypical symptoms. Through data collection and literature review on Iranian AT patients, it was found that some patients have later onset age and slower disease progression, along with immunological features and increased cancer risk. Overall, atypical presentations may be associated with deleterious mutations and known modifying factors.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Veterinary Sciences
Renan B. Sper, Jessica Proctor, Odessa Lascina, Ling Guo, Kathryn Polkoff, Tobias Kaeser, Sean Simpson, Luke Borst, Katherine Gleason, Xia Zhang, Bruce Collins, Yanet Murphy, Jeffrey L. Platt, Jorge A. Piedrahita
Summary: Mice with severe combined immunodeficiency are commonly used as hosts for human cells, but their size, longevity, and physiology limit their ability to model human systems. In this study, researchers generated immunodeficient pigs and successfully transplanted human cells into them, demonstrating that the pig thymus provides a suitable microenvironment for engraftment and development of human T-cells.
FRONTIERS IN VETERINARY SCIENCE
(2022)
Article
Cardiac & Cardiovascular Systems
Hayley T. Dillon, Stephen Foulkes, Yuki A. Horne-Okano, David Kliman, David W. Dunstan, Robin M. Daly, Steve F. Fraser, Sharon Avery, Bronwyn A. Kingwell, Andre La Gerche, Erin J. Howden
Summary: This study evaluated the short-term cardiovascular impact of allogeneic hematopoietic cell transplantation (allo-HCT) on hematological cancer patients. The results showed that allo-HCT severely impaired the patients' VO(2)peak, indicating central and peripheral dysfunction. This study highlights the rapid acceleration of cardiovascular aging in this high-risk group.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)