scholarly journals Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia

2016 ◽  
Vol 22 (7) ◽  
pp. 1257-1263 ◽  
Author(s):  
Carmem Bonfim ◽  
Lisandro Ribeiro ◽  
Samantha Nichele ◽  
Marco Bitencourt ◽  
Gisele Loth ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Fanconi Anemia ◽  
Organ Function ◽  
Term Survival ◽  
Hematopoietic Stem

scholarly journals ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR ADULT PATIENTS WITH FANCONI ANEMIA.

2016 ◽  
Vol 8 ◽  
pp. 2016054 ◽  
Author(s):  
Hosein Kamranzadeh fumani ◽  
Mohammad Zokaasadi ◽  
Amir Kasaeian ◽  
Kamran Alimoghaddam ◽  
Asadollah Mousavi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Fanconi Anemia ◽  
Bone Marrow Failure ◽  
Term Survival ◽  
Hematopoietic Stem

Background & objectives: Fanconi anemia (FA) is a rare genetic disorder caused by an impaired DNA repair mechanism which leads to an increased tendency toward malignancies and progressive bone marrow failure. The only curative management available for hematologic abnormalities in FA patients is hematopoietic stem cell transplantation (HSCT). This study aimed to evaluate the role of HSCT in FA patients.Methods: Twenty FA patients with ages of 16 or more who underwent HSCT between 2002 and 2015 enrolled in this study. All transplants were allogeneic and the stem cell source was peripheral blood and all patients had a full HLA-matched donor.Results: Eleven patients were female and 9 male (55% and 45%). Mean age was 24.05 years. Mortality rate was 50% (n=10) and the main cause of death was GVHD. Survival analysis showed an overall 5-year survival of 53.63% and 13 year survival of 45.96 % among patients.Conclusion: HSCT is the only curative management for bone marrow failure in FA patients and despite high rate of mortality and morbidity it seems to be an appropriate treatment with an acceptable long term survival rate for adolescent and adult group.

scholarly journals Prediction of Response and Survival Following Treatment with Azacitidine for Relapse of Acute Myeloid Leukemia and Myelodysplastic Syndromes after Allogeneic Hematopoietic Stem Cell Transplantation

Cancers ◽  
2020 ◽  
Vol 12 (8) ◽  
pp. 2255 ◽  
Author(s):  
Christina Rautenberg ◽  
Anika Bergmann ◽  
Ulrich Germing ◽  
Caroline Fischermanns ◽  
Sabrina Pechtel ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Term Outcome ◽  
Term Survival ◽  
Hematopoietic Stem ◽  
Long Term Outcome

To provide long-term outcome data and predictors for response and survival, we retrospectively analyzed all 151 patients with relapse of myeloid neoplasms after allogeneic hematopoietic stem cell transplantation (allo-HSCT) who were uniformly treated with first-line azacitidine (Aza) salvage therapy at our center. Patients were treated for molecular (39%) or hematologic relapse (61%), with a median of 5 cycles of Aza and at least one donor lymphocyte infusion in 70% of patients. Overall response was 46%, with 41% achieving complete (CR) and 5% achieving partial remission. CR was achieved after a median of 4 cycles and lasted for a median of 11 months (range 0.9 to 120 months). With a median follow-up of 22 months (range: 1 to 122 months), the 2-year survival rate was 38% ± 9%, including 17 patients with ongoing remission for >5 years. Based on results from multivariate analyses, molecular relapse and time to relapse were integrated into a score, clearly dividing patients into 3 subgroups with CR rates of 71%, 39%, and 29%; and 2-year survival rates of 64%, 38%, and 27%, respectively. In the subgroup of MDS and secondary AML, receiving upfront transplantation was associated with superior response and survival, and therefore pretransplant strategy was integrated together with relapse type into a MDS–sAML-specific score. Overall, Aza enables meaningful responses and long-term survival, which is a predictable with a simple-to-use scoring system.

Negative Impact of Pre-Transplant Anemia on Long-Term Outcome after Allogeneic Hematopoietic Stem Cell Transplantation.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1844-1844
Author(s):  
Chris Lazongas ◽  
Cindy J. Wong ◽  
David M. Sutton ◽  
Jeffrey H. Lipton ◽  
Anargyros Xenocostas ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Causes Of Death ◽  
Term Survival ◽  
Hematopoietic Stem

Abstract Background: Anemia is commonly present in patients with malignancies and is associated with reduced survival times. Recently, we described that low (<110 g/L) pre-transplant hemoglobin levels (PT-Hb) are associated with decreased early survival after allogeneic hematopoietic stem cell transplantation (alloHSCT)(Xenocostas et al. Transfusion2003;43:373–382). We are now presenting data on long-term survival and causes of death in BMT recipients with and without anemia prior to alloHSCT. Study Design and Methods: A retrospective analysis of 511 patients consecutively transplanted between January 1995 and March 2000 was performed to evaluate survival, cause of death, and PT-Hb. The end date for follow-up was June 2002. The median follow-up time was 993 days. Causes of death were categorized either as relapse, treatment-related mortality (TRM), or other. PT-Hb levels were determined within 2 weeks prior to transplantation, after commencing conditioning chemotherapy. Comparisons between groups were done using chi-squared tests. Results: The 180-day survival of patients with low PT-Hb levels (<110 g/L) was significantly worse than that of patients with PT-Hb levels ≥110 g/L (57.1% versus 83.1%, p<0.0001). The survival difference remained significant at 5 years (36.2% versus 59.4%, p<0.0001). The difference in 180-day survival was contributed to by an increase in TRM (36.1% versus 14.9%, p<0.0001) as well as a higher relapse rate (4.4% versus 0.3%, p=0.019 by Fisher’s exact test). For patients surviving more than 180 days, there was no difference in TRM (16.7% versus 16.7%, p=0.987) or relapse rate (12.0% versus 7.3%, p=0.150). No difference in the rate of other causes of death was found between the groups at either the 180-day or 5-year time points. Conclusions: Pre-transplant anemia is an independent risk factor for increased mortality following alloHSCT. Relapse and treatment-related deaths are both more likely to occur early in the post-transplant course of patients experiencing pre-transplant anemia. Differences in long-term survival are predominantly related to treatment-related deaths rather than relapse.

scholarly journals Long-Term Survival After Hematopoietic Stem Cell Transplantation for Complete STAT1 Deficiency

2017 ◽  
Vol 37 (7) ◽  
pp. 701-706 ◽  
Author(s):  
Samuele Naviglio ◽  
Elena Soncini ◽  
Donatella Vairo ◽  
Arnalda Lanfranchi ◽  
Raffaele Badolato ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Term Survival ◽  
Hematopoietic Stem ◽  
Long Term Survival
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