Long-term outcome of a group of Japanese children with myelin-oligodendrocyte glycoprotein encephalomyelitis without preventive immunosuppressive therapy

2019 ◽  
Vol 41 (9) ◽  
pp. 790-795 ◽  
Author(s):  
Naomi Hino-Fukuyo ◽  
Kazuhiro Haginoya ◽  
Toshiyuki Takahashi ◽  
Ichiro Nakashima ◽  
Kazuo Fujihara ◽  
...  
2016 ◽  
Vol 105 (12) ◽  
pp. 1011-1020 ◽  
Author(s):  
Felicitas Escher ◽  
Uwe Kühl ◽  
Dirk Lassner ◽  
Wolfgang Poller ◽  
Dirk Westermann ◽  
...  

2020 ◽  
Vol 99 (11) ◽  
pp. 2529-2538
Author(s):  
Beatrice Drexler ◽  
Felicitas Zurbriggen ◽  
Tamara Diesch ◽  
Romaine Viollier ◽  
Joerg P. Halter ◽  
...  

Abstract Introduction Since the 1970s outcome of aplastic anemia (AA) patients has improved significantly due to the introduction of immunosuppressive therapy (IST) and allogeneic hematopoietic transplantation (HCT). However, patients may suffer from persistent disease, relapse, clonal evolution, graft-versus-host disease and other late effects. Here, we analyse very long-term outcome of all AA patients at our institution comparing not only survival, but also response status and complications. Methods Patient charts of all 302 AA patients treated between 1973 and 2017 at the University Hospital Basel, Switzerland, were retrospectively analysed. Results First line treatment was IST in 226 (75%) and HCT in 76 (25%) patients. Overall survival at 30 years was similar in patients treated initially by HCT and IST (44% (±14%), and 40% (± 9%) respectively, with better results in more recent years. Partial and no response occurred more frequently after IST, relapse incidence after IST was 24 %, whereas non-engraftment and graft failure was documented in 15 patients (19 %) after HCT. Clonal evolution to myelodysplastic syndrome / acute myeloid leukemia was 16 % at 25 years in IST patients, 1.3 % in HCT patients, iron overload (18 versus 4 %, p = 0.002) and cardiovascular events (11 versus 1 %, p=0.011) occured significantly more often in IST than HCT treated patients. The majority of long-term survivors, 96% of those alive at 25 years, were in complete remission at last follow up, irrespective of the initial treatment modality. Conclusion Very long term survivors after AA are those with stable hematopoietic recovery.


2019 ◽  
Vol 34 (11) ◽  
pp. 2417-2421
Author(s):  
Shuichiro Fujinaga ◽  
Daishi Hirano ◽  
Tomohiko Nishino ◽  
Chisato Umeda ◽  
Yoshitaka Watanabe ◽  
...  

Author(s):  
N. N. Koloskova ◽  
V. N. Poptsov ◽  
A. О. Shevchenko

Heart transplantation is the «gold standard» of treatment severe heart failure. Immunosuppressive therapy aimed at the prevention of acute allograft rejection is the cornerstone of post-transplant management. In addition to its direct effects, immunosuppressive therapy is also involved in the generation of a number of post-transplant morbidities that limit the long-term outcome of heart transplant recipients. Given these data it appears that the individual tailoring of immunosuppressive therapy is of paramount importance in determining the outcome of heart transplantation. The goal of immunosuppressive therapy is to prevent rejection of the transplanted heart, while minimizing drug-related effects, such as infection, malignancy, diabetes, hypertension, and renal insuffi ciency. This review aimed is to analyze the protocols for the appointment of immunosuppressive therapy in various groups of recipients after heart transplantation.


2009 ◽  
Vol 87 (12) ◽  
pp. 1821-1829 ◽  
Author(s):  
Georg Heinze ◽  
Rainer Oberbauer ◽  
Alexander Kainz ◽  
Christa Mitterbauer ◽  
Christian Koppelstaetter ◽  
...  

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 997-997
Author(s):  
Bunchoo Pongtanakul ◽  
Prabodh K. Das ◽  
Yigal Dror

Abstract Background: Immunosuppressive therapy (IST) is the alternative first line treatment in children with aplastic anemia (AA) who have no HLA match siblings available. The long-term outcome of patients with AA who survive after IST is unknown. We evaluated outcomes of children with AA treated with IST at long-term follow up. Methods: we retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with horse-derived antithymocyte globulin (hATG) 160 mg/Kg over 4 days, short course of prednisone and cyclosporine (CS). Results: Forty two patients were treated with IST (25 boys, 18 girls). The median age at diagnosis was 8.5 years. Twenty nine (69%), eight (19%) and five (12%) patients were diagnosed with severe, very severe, and moderate AA, respectively. Nine patients (21%) had hepatitis associated AA. Twenty seven patients (64%) received one course of ATG and fifteen (36%) received 2 courses (8 received 2 courses of hATG and 7 received 1 course hATG and 1 course rabbit derived ATG). Eleven patients (26%) required G-CSF. Median follow up time was 53.3 months (range 3–244 months). Twenty six patients (62%) had a complete response (CR), eight (19%) had a partial response (PR) and eight (19%) had no response (NR). Two patients relapsed after one course of IST and needed a second course of IST and both of them had a partial response. Median time to discontinuation of CS was 13 months. Nine patients (21%) died (7 with NR and 2 with PR) and 33 patients (79%) are alive. Two patients developed myelodysplastic syndrome (MDS) 21 and 19 months post IST; both received long-term G-CSF (18 and 14 months) and had PR after 2 courses of IST. Five of 33 patients (15%) who survived had significant hypertension after CS was discontinued and one required continuous antihypertensive medication. Conclusion: The results of this study shows promising response in children with AA treated with IST. Hypertension and MDS are late complication. Longer follow up in these patients is warranted to definite the accurate rates of the late complications and risk factors.


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