scholarly journals Corticosteroids Combined with Cyclosporine a Is Superior to Corticosteroids As First-Line Treatment for Autoimmune Hemolytic Anemia after Allogeneic Hematopoietic Stem Cell Transplantation

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5736-5736
Author(s):  
Weiran Lv ◽  
Hong Qu ◽  
Meiqing Wu ◽  
Zhiping Fan ◽  
Fen Huang ◽  
...  

Abstract We retrospectively investigated the incidence and outcomes of allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients with autoimmune hemolytic anemia (AIHA). A total of 1,377 patients enrolled in this study, and the 3-year cumulative incidence of AIHA was 2.2±0.4%. Twenty-five patients with AIHA received treatment, including 15 patients who received corticosteroids combined with Cyclosporine A(CsA )and 10 who received corticosteroids monotherapy. After four weeks of treatment, the patients treated with corticosteroids combined with CsA had a higher complete response rate than those with corticosteroids monotherapy (66.7% vs. 11.1%; P=0.013). One patient (6.7%) experienced AIHA relapse in the combined group, and 5 (50.0%) relapsed in the monotherapy group (P=0.023). The 5-year cumulative incidence of malignant disease relapse was 5.5±5.1% and 27.4±1.4% (P=0.032), the OS was 86.3 ± 7.4% and 65.2 ± 1.6% (P=0.046), and the transplant-related mortality (TRM) post-transplantation was 9.3±6.4% and 22.8±1.6% (P=0.140), respectively, for patients with AIHA and those without AIHA. Our results indicate that corticosteroids combined with CsA is superior to corticosteroids as first-line treatment for AIHA, and AIHA does not contribute to increased primary disease relapse and TRM. Disclosures No relevant conflicts of interest to declare.

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S157-S158
Author(s):  
C E Kanakis ◽  
K Gvozdjan

Abstract Introduction/Objective Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment option for patients with myelodysplastic syndrome (MDS). Nonetheless, a large proportion of patients with MDS experience disease relapse. Declining donor chimerism and detection of recurrent gene mutations have been used as indicators of graft failure and recurrent disease. Blood Bank serologic findings have rarely been described as first indicators of disease relapse in this setting and could potentialy add to engraftment and relapse surveillance testing. Methods/Case Report A 72-year-old, ABO group O, RhD positive male with history of anti-Fyb alloimmunization underwent allo-HSCT from an ABO group B, RhD negative, Fyb positive donor as part of the treatment for MDS. Successful engraftment was achieved, and the patient’s red blood cell phenotype transitioned to ABO group B, RhD negative, Fyb positive. Two years following allo-HSCT, the patient received chemotherapy for recurrent cholangiocarcinoma. Supportive blood component transfusions were provided, all of which typed as RhD negative. However, new antibody with anti-D specificity was detected in serum while the patient still typed as ABO group B, RhD negative, preceding anti-D and later recurrent anti-Fyb detection in eluate, and prompting further chimerism testing. Declining donor chimerism was noted (72% donor, compared to >98% donor on prior chimerism testing). Chemotherapy and donor-lymphocyte infusion were initiated. Results (if a Case Study enter NA) NA Conclusion The early detection of de novowlvw anti-D was most consistent with resurgence of patient’s erythroids within the bone marrow in the presence of donor’s immune system. This was followed by sufficient peripheralization of patient’s red blood cells and detection of anti-D in the eluate. Lastly, the switch to recipient’s immune system is evidenced by recurrent detection of anti-Fyb in the eluate. This case, therefore, emphasizes the utility of Blood Bank serology in raising suspicion for disease relapse and guiding further allo-HSCT patient management. More systematic use of Blood Bank serology may serve as a time- and cost-effective adjunct to the current strategies employed for detection of disease recurrence in allo-HSCT recipients.


2020 ◽  
Vol 11 ◽  
pp. 204062072092104
Author(s):  
Ja Min Byun ◽  
Sung-Soo Yoon ◽  
Youngil Koh ◽  
Chang-Ki Min ◽  
Jae Hoon Lee ◽  
...  

Background: Traditionally believed to be an integral part of multiple myeloma (MM) treatment, the role of hematopoietic stem-cell transplantation (HSCT) is being challenged. As such, we sought to evaluate the impact of HSCT in the era of novel agents. Methods: A multicenter, retrospective, longitudinal cohort study was carried out between January 2016 and December 2018. A total of 55 patients who received VTD (bortezomib-thalidomide-dexamethasone) as first-line treatment and KRd (carfilzomib-lenalidomide-dexamethasone) as second-line treatment were analyzed for outcomes. Results: The enrolled patients were divided into Group 1, defined as those who continued KRd treatment until progression ( n = 41), versus Group 2, defined as those who underwent HSCT after a certain number of cycles of KRd ( n = 14). Both groups showed a generally favorable response to KRd, with overall response rate (ORR) of 87.9% and clinical benefit rate of 92.8% after a median of seven cycles in Group 1, and ORR 92.8% and clinical benefit rate 100% after median of five cycles in Group 2. However, significantly poorer progression-free survival (PFS) ( p = 0.004) was observed in Group 1 (median 12 months) compared with Group 2 (median not reached). Multivariate analyses identified HSCT after KRd as potential risk factors associated with PFS. Also, in Group 1, bortezomib refractoriness was associated with significantly shorter PFS compared with those who were responsive (median 12 months versus 14 months, respectively, p = 0.039). Conclusions: In conclusion, even with the advent of novel agents, HSCT still remains a valuable treatment modality with additive efficacy.


2017 ◽  
Vol 1 (24) ◽  
pp. 2138-2146 ◽  
Author(s):  
Young Ae Kim ◽  
Ja Min Byun ◽  
Keeho Park ◽  
Gi Hwan Bae ◽  
Dukhyoung Lee ◽  
...  

Key Points Etoposide addition/chemo-intensification has little role in first-line treatment of PTCL in Asian populations, regardless of subtype or age. Upfront hematopoietic stem-cell transplantation as consolidation seems like a legitimate choice in patients with PTCL.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4669-4669
Author(s):  
C.J.M. Halkes ◽  
M.H. Vermeer ◽  
E.M. Noordijk ◽  
R. Willemze ◽  
Roelof Willemze

Abstract Aggressive cutaneous NK/T-cell lymphomas are extremely rare with an estimated yearly incidence of less than 1 per 2,000,000 and a disease-specific 5 year survival of 0–16%. This dismal prognosis seems independent of the presence of extracutaneous localizations at diagnosis. Only a minority of patients respond to CHOP-like multiagent chemotherapy. Sustained complete remissions (CR) have been observed after stem cell transplantation of some heavily pre-treated patients. We hypothesized that stem cell transplantation may be the only treatment modality that may induce long term remissions in this very resistant disease. In order to get as many possible patients eligible for a stem cell transplantation we designed an intensive protocol in which patients with newly diagnosed aggressive cutaneous NK/T-cell lymphoma were treated with AML-like induction and consolidation courses followed by an allogeneic or an autologous stem cell transplantation. All patients with newly diagnosed aggressive cutaneous NK/T-cell lymphoma who were referred to our centre between 06-2003 and 06-2006 were screened for this study. Patients who were able to undergo this intensive therapy, were enrolled after informed consent. Induction treatment consisted of etoposide 120 mg/m2 and amsacrine 115 mg/m2 on days 1 and 7, methylprednisolone 60 mg/m2 on days 1–7, intrathecal methotrexate on day 1 and cytarabine on days 1–6: either 1000 mg/m2 twice daily (age <61) or 100 mg/m2 daily (age > 60). In case of partial remission (PR), induction treatment was repeated. In case of CR, consolidation treatment was given consisting of amsacrine and etoposide in similar doses as during induction, and cytarabin 3000 mg/m2 twice daily on days 1–4 or 300 mg/m2 twice daily on days 1–4 in older patients. In case of persisting CR after consolidation therapy, patients were eligible for allogeneic stem cell transplantation with total body irradiation (TBI) and high dose cyclophosphamide as conditioning regimen. In the absence of a HLA matched donor, autologous transplantation was planned. Six patients (3 males, 3 females) were treated for cutaneous peripheral T cell lymphoma not otherwise specified (n=3), cutaneous blastic NK cell lymphoma (n=2) or cutaneous NK-T cell lymphoma, nasal type (n=1). Mean age was 52 years (range 33–65). In two patients no extracutaneous localizations were found. After induction treatment two patients had progressive disease, and one patient showing PR died suddenly during leukopenic period after the second induction cycle. Three patients achieved the CR. One of them died due to an intracerebral hemorrhage just before transplantation. Two patients underwent stem cell transplantation (one autologous and one allogeneic) but both relapsed, 204 and 218 days after transplantation, respectively and died within weeks. Median overall survival was 214 days (range 30–382).First line treatment of aggressive cutaneous NK/T cell lymphoma using an intensive AML-like chemotherapy approach resulted in CR in 3 out of 6 patients. Only two of them reached a stem cell transplantation, but both patients died of relapsing disease. Based on these outcomes, first line treatment of aggressive cutaneous NK/T-cell lymphoma consisting of AML-like induction and consolidation therapy did not result in durable remissions, necessary to perform stem cell transplantation.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3886-3886 ◽  
Author(s):  
Bernd Gruhn ◽  
Susan Wittig ◽  
Clemens Arndt ◽  
Claudia Seifert

Abstract Background:Hepatic sinusoidal obstruction syndrome (SOS), commonly known as veno-occlusive disease of the liver is a life-threatening early complication after hematopoietic stem cell transplantation (HSCT). Until now, examinations about the influence of genetic risk factors are extremely rare. Heparanase (HPSE), a pivotal endoglycosidase responsible for heparan sulfate degradation, is expressed by activated endothelial cells. HPSE has been shown to be involved in inflammation and may therefore play an important role in the pathogenesis of hepatic SOS. The purpose of this study was to identify an association between HPSE single nucleotide polymorphisms (SNPs) and hepatic SOS in children undergoing allogeneic HSCT. Methods:160 children (median age, 14 years) who underwent allogeneic bone marrow (n=91) or peripheral blood stem cell transplantation (n= 69) in a single center and their respective donors were genotyped of HPSE for rs4693608 and rs4364254 using TaqMan real-time polymerase chain reaction. The donor was HLA-matched unrelated in 63% of transplants and HLA-identical related in 25% of transplants. Conditioning regimen was myeloablative in all cases and based on busulfan in 46% of transplants or total body irradiation in 33% of transplants. Two forms of post-transplant immunosuppression predominated, cyclosporine A and methotrexate in 50% of transplants and cyclosporine A alone in 30% of transplants. Results:160 donor/patient pairs were analyzed. Cell samples from the patient were available in 155 cases and from the donor in 153 cases. Genotype AG of HPSE rs4693608 SNP was found in 82 patients (53%), AA in 49 patients (32%), and 24 patients were homozygous for GG (15%). Analysis of HPSE rs4364254 SNP revealed a similar distribution for TC (n=69, 44%) and TT (n=68, 44%) and a frequency of 18 patients (12%) for CC. Hepatic SOS was observed in 12 patients (8%). According to the modified Seattle criteria we identified ten patients with early-onset disease in the first 20 days after HSCT and two patients who developed hepatic SOS later on day +44 and day +83 after transplantation (late-onset SOS), respectively. If hepatic SOS was diagnosed all of our patients were treated with defibrotide as early as possible. Two patients (17%) developed severe hepatic SOS and died of multi-organ failure. The remaining ten patients with hepatic SOS (83%) could be successfully treated and survived. Patients with HPSE genotypes GG or AG of rs4693608 (G>A) had a significantly reduced incidence of hepatic SOS on day 100 after HSCT compared to patients with genotype AA (5% vs. 14%, p=0.038). In addition, incidence of hepatic SOS in patients with genotype CC or CT of rs4364254 (C>T) was significantly decreased in comparison to patients with genotype TT (2% vs. 15%, p=0.004). Interestingly, no patient with genotype CC developed hepatic SOS. Because both SNPs co-occur in vivo, we generated subsets: AA-TT, GG-CC and a group with remaining SNP combinations. We found significant differences between all three patient groups (p=0.035). Patients with AA-TT showed the highest incidence of hepatic SOS (17%), while hepatic SOS was not observed in patients with GG-CC (0%) and residual combinations were numerically in-between (5%). An impact caused by main patient and donor characteristics, established risk factors for hepatic SOS, and conditioning regimen could be excluded in multivariate analyses. Conclusions: This study provides the first evidence that HPSE polymorphisms are significant independent risk factors (p=0.030) for the development of hepatic SOS and should be evaluated in further trials. Disclosures No relevant conflicts of interest to declare.


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