• Journal of Yeungnam Medical Science
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• 제29권2호
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• pp.96-101
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• 2012

Allogeneic hematopoietic stem cell transplantation (HSCT) is considered the optimal curative treatment for acute myeloid leukemia (AML), but some patients develop bone marrow relapse due to remnant leukemia, and few patients develop extramedullary relapse without bone marrow relapse. Isolated extramedullary relapse (IMER) is defined as extramedullary relapse without bone marrow relapse. IMER has been reported in various sites, including the skin, soft tissue, and central nervous system(CNS). Isolated CNS relapse is relatively rare and is associated with poor prognosis due to the absence of an optimal treatment for it. Reported herein is a case involving an adult AML woman who suffered from isolated extramedullary relapse in the CNS after allogeneic HSCT. She was treated with intrathecal chemotherapy and whole-brain and spine radiotherapy, followed by systemic chemotherapy. She is currently well, with no evidence of leukemia recurrence for over six years.

• Parasites, Hosts and Diseases
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• 제50권4호
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• pp.353-355
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• 2012

We report here a case of inguinal sparganosis, initially regarded as myeloid sarcoma, diagnosed in a patient undergone allogeneic hematopoietic transplantation (HSCT). A 56-year-old male patient having myelodysplastic syndrome was treated with allogeneic HSCT after myeloablative conditioning regimen. At day 5 post-HSCT, the patient complained of a painless palpable mass on the left scrotum and inguinal area. Pelvic magnetic resonance imaging and computed tomography revealed suspected myeloid sarcoma. Gun-biopsy was performed, and the result revealed eosinophilic infiltrations without malignancy. Subsequent serologic IgG antibody test was positive for sparganum. Excisional biopsy as a therapeutic diagnosis was done, and the diagnosis of sparganosis was confirmed eventually. This is the first report of sparganosis after allogeneic HSCT mimicking myeloid sarcoma, giving a lesson that the physicians have to consider the possibility of sparganosis in this clinical situation and perform adequate diagnostic and therapeutic approaches.

• Clinical and Experimental Pediatrics
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• 제58권6호
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• pp.199-205
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• 2015

Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD). Given the recent progress in HSCT using URDs for childhood SAA, well-matched URDs became a realistic alternative for pediatric patients who have no suitable related donors and who are refractory to IST. However, it is quite challenging to treat patients with refractory SAA who lack suitable related or URDs. Even though haploidentical HSCT from genetically mismatched family members seemed to be an attractive procedure with the amazing benefit of readily available donors for most patients, early attempts were disappointing because of refractory graft-versus-host disease (GVHD) and excessively high transplant-related mortality. Recent advances with effective ex vivo depletion of T cells or unmanipulated in vivo regulation of T cells, better supportive care, and optimal conditioning regimens have significantly improved the outcome of haploidentical transplant. Besides considerable progress in the treatment of malignant diseases, recent emerging evidences for haploidentical HSCT in SAA has provided additional therapeutic options for patients with refractory diseases. Further improvements to decrease the rates of graft failure, GVHD, and infectious complications will facilitate the emergence of haploidentical HSCT as a front-line therapy for treating acquired SAA in children and adolescents who have no suitably matched donors.

• 한국융합학회논문지
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• 제8권4호
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• pp.49-57
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• 2017

본 연구는 소아암 환아의 조혈모세포이식 후 성장을 확인하고, 이에 영향을 미치는 요인들을 조사하고자 진행된 융합연구이다. 2009년 2월부터 3월까지 조혈모세포이식을 받은 소아암 환아 112명의 의무기록으로부터 키와 몸무게를 조사하였으며 자료 분석을 위해 혼합효과 모델을 사용하였다. 연구 결과 조혈모세포이식 후 대상자의 신장과 체중의 표준과의 평균표준편차값이 음의 값이었으며 동종이식 보다 자가이식의 경우 신장(p=0.0008)과 체중(p=0.0012)의 평균이 낮았다. 이식 후 신장에 영향을 주는 것으로 대상자의 이식 시 연령(p=0.0251)과 이식 형태(p=0.0020)가 확인되었으며. 동종이식 환아에서는 성장에 영향을 주는 것으로 스테로이드의 사용량이 확인되었다(p=0.0403). 대상자의 이식 후 체중은 이식 시 연령(p=0.0042), 이식형태(p=0.0035) 그리고 총정맥영양의 주입기간(p=0.0159)에 영향을 받는 것으로 나타났다. 본 연구 결과를 바탕으로 소아암 환아의 조혈모세포이식 후 성장이 잘 이루어질 수 있도록 성장저하의 고위험군을 식별할 수 있도록 하여야하며, 이러한 환아에게 적절한 간호중재가 수행되어야 할 것이다.

• Clinical and Experimental Pediatrics
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• 제55권3호
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• pp.93-99
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• 2012

Purpose: This study compared outcomes in children with acute leukemia who underwent transplantations with umbilical cord blood (UCB), bone marrow, or peripheral blood stem cells from a human leukocyte antigen (HLA)-matched related donor (MRD) or an unrelated donor (URD). Methods: This retrospective study included consecutive acute leukemia patients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) at Samsung Medical Center between 2005 and 2010. Patients received stem cells from MRD (n=33), URD (n=46), or UCB (n=41). Results: Neutrophil and platelet recovery were significantly longer after HSCT with UCB than with MRD or URD ($p$ <0.01 for both). In multivariate analysis using the MRD group as a reference, the URD group had a significantly higher risk of grade III to IV acute graft-versus-host disease (GVHD; relative risk [RR], 15.2; 95% confidence interval [CI], 1.2 to 186.2; $p$=0.03) and extensive chronic GVHD (RR, 6.9; 95% CI, 1.9 to 25.2; $p$ <0.01). For all 3 donor types, 5-year event-free survival (EFS) and overall survival were similar. Extensive chronic GVHD was associated with fewer relapses (RR, 0.1; 95% CI, 0.04 to 0.6; $p$ <0.01). Multivariate analysis showed that lower EFS was associated with advanced disease at transplantation (RR, 3.2; 95% CI, 1.3 to 7.8; $p$ <0.01) and total body irradiation (RR, 2.1; 95% CI, 1.0 to 4.3; $p$=0.04). Conclusion: Survival after UCB transplantation was similar to survival after MRD and URD transplantation. For patients lacking an HLA matched donor, the use of UCB is a suitable alternative.

• Journal of Hospice and Palliative Care
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• 제8권2호
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• pp.190-199
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• 2005

목적: 자가 및 동종 조혈모세포 이식종류에 따라, 이식단계별 불안과 우울 정도를 파악함으로써 이식종류와 이식단계에 따라 조혈모세포이식 환자의 정서적 지지에 적합한 간호중재 개발을 위한 기초 자료를 마련하고자 시도되었다. 방법: 서울 소재 C대학병원에 2002년 8월부터 2003년 8월까지 조혈모세포이식을 위해 입원한 환자 52명을 대상으로(자가 조혈모세포이식 환자 19명, 동종 조혈모세포이식 환자 33명)조혈모세포 이식단계별 즉 입원 시, 이식 전날, 퇴원 시 총 3회 대상자를 방문하여 상태불안과 우울 정도를 조사하였다. 조사된 자료는 SAS 프로그램을 이용하여 분석하였으며, 자가 조혈모세포이식과 동종 조혈모세포이식의 이식단계별 불안과 우울은 반복측정 분산분석으로 검정하였으며, Bonferroni 다중비교로 사후검정을 실시하였다. 이식단계별 불안과 우울에 영향을 미치는 주요변인은 Stepwise multiple regression으로 분석하였다. 결과: 자가 조혈모세포이식 환자의 불안은 입원 시 42.3점, 이식전날 45.0점, 퇴원 시 40.8점이었으며, 동종 조혈모세포이식 환자의 불안은 입원 시 48.4점, 이식전날 48.7점, 퇴원 시 47.1점으로 두 군간 유의한 차이가 있었다(F=4.15, P=0.047). 자가조혈모세포이식 환자의 이식 전날 우울이 39.0점으로 입원 시 33.0점보다 유의하게 높았으며(F=21.45, P=0.0004). 동종 조혈모세포이식 환자의 우울도 이식전날 40.3점으로 입원 시 34.9점보다 유의하게 높았다(F=20.99, P=0.0002). 자가 조혈모이식단계별 불안에 영향을 미치는 주요인은 우울이었으며, 특히 입원 시에는 입원 전 직업유무가 불안에 영향을 주는 요인이었다. 동종 조혈모세포이식 환자의 입원 시 불안에 영향을 미치는 주요 요인은 우울, 성별이었으며, 퇴원 시에는 재발경험과 성별이었다. 자가 조혈모세포이식과 동종 조혈모세포이식 모두에서 이식단계별 우울에 영향을 미치는 요인은 불안으로 분석되었다. 결론: 이식전반에 걸쳐 동종 조혈모세포이식 환자의 불안과 우울이 자가 조혈모세포이식 환자보다 높았으며, 이식단계별로는 이식전날 우울이 가장 높았다. 따라서 조혈모세포이식을 위해 입원한 환자에게는 이식전날 우울에 대한 정서적 지지가 필요하며 특히 동종조혈모세포 이식환자는 입원 시, 이식 전날, 퇴원 시 등 이식 단계별로 불안과 우울을 경감시킬 수 있는 간호중재 개발과 그 적용이 요구된다.

• IMMUNE NETWORK
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• 제3권2호
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• pp.150-155
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• 2003

Background: We investigated the effect of donor marrow T cell depletion, administration of FK506, cyclosporin A (CSA), and 3-deazaadenosine (DZA) on graft versus host disease (GVHD) after allogeneic murine hematopoietic stem cell transplantation (HSCT). Methods: We used 4 to 6 week old Balb/c ($H-2^d$, recipient), and C3H/He ($H-2^k$, donor) mice. Total body irradiated recipients received $1{\times}10^7$ bone marrow cells (BM) and $0.5{\times}10^7$ splenocytes of donor under FK506 (36 mg/kg/day), CSA (5 mg/kg/day, 20 mg/kg/day), and DZA (45 mg/kg/day), which were injected intraperitoneally from day 1 to day 14 daily and then three times a week for another 2 weeks. To prevent the GVHD, irradiated Balb/c mice were transplanted with $1{\times}10^7$ rotor-off (R/O) cells of donor BM. The severity of GVHD was assessed daily by clinical scoring method. Results: All experimental groups were well grafted after HSCT. Mice in experimental group showed higher GVHD score and more rapid progression of GVHD than the mice with R/O cells (R/O group) (p<0.01). There were relatively low GVHD scores and slow progressions in FK506 and low dose CSAgroups than high dose CSA group (p<0.01). The survival was better in FK506 group than low dose CSA group. All mice treated with CSA died within 12 days after HSCT. The GVHD score in DZA group was low and slow in comparison with control group (p<0.05), but severity and progression were similar with low dose CSA group (p=0.11). All mice without immunosuppressive treatment died within 8 days, but all survived in R/O group (p<0.01). Survival in low dose CSA group was longer than in control group (p<0.05), but in high dose CSA group, survival was similar to control group. The survival benefit in DZA group was similar with low dose CSA group. FK506 group has the best survival benefit than other groups (p<0.01), comparable with R/O group (p=0.18), although probability of survival was 60%. Conclusion: We developed lethal GVHD model after allogeneic murine HSCT. In this model, immunosuppressive agents showed survival benefits in prevention of GVHD. DZA showed similar survival benefits to low dose CSA. We propose that DZA can be used as a new immunosuppressive agent to prevent GVHD after allogeneic HSCT.

• Clinical and Experimental Pediatrics
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• 제56권1호
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• pp.26-31
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• 2013

Purpose: Lymphocyte subset recovery is an important factor that determines the success of hematopoietic stem cell transplantation (HSCT). Temporal differences in the recovery of lymphocyte subsets and the factors influencing this recovery are important variables that affect a patient's posttransplant immune reconstitution, and therefore require investigation. Methods: The time taken to achieve lymphocyte subset recovery and the factors influencing this recovery were investigated in 59 children who had undergone HSCT at the Department of Pediatrics, The Catholic University of Korea Seoul St. Mary's Hospital, and who had an uneventful follow-up period of at least 1 year. Analyses were carried out at 3 and 12 months post-transplant. An additional study was performed 1 month post-transplant to evaluate natural killer (NK) cell recovery. The impact of pre- and post-transplant variables, including diagnosis of Epstein-Barr virus (EBV) DNAemia posttransplant, on lymphocyte recovery was evaluated. Results: The lymphocyte subsets recovered in the following order: NK cells, cytotoxic T cells, B cells, and helper T cells. At 1 month post-transplant, acute graft-versus-host disease was found to contribute significantly to the delay of $CD16^+/56^+$ cell recovery. Younger patients showed delayed recovery of both $CD3^+/CD8^+$ and $CD19^+$ cells. EBV DNAemia had a deleterious impact on the recovery of both $CD3^+$ and $CD3^+/CD4^+$ lymphocytes at 1 year post-transplant. Conclusion: In our pediatric allogeneic HSCT cohort, helper T cells were the last subset to recover. Younger age and EBV DNAemia had a negative impact on the post-transplant recovery of T cells and B cells.

• Clinical and Experimental Pediatrics
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• 제49권2호
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• pp.173-180
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• 2006

목 적 : CMV 감염은 여전히 조혈모세포 이식 후 가장 중요한 감염 중 하나로 이환율과 사망률의 주요 원인이다. 조혈모세포 이식 후 CMV 감염 발생에 대한 위험인자의 분석 및 CMV pp65 항원혈증에 입각한 선제치료의 효과와 질환의 경과를 평가하고자 본 연구를 시행하였다. 방 법 : 1998년 10월부터 2003년 12월까지 가톨릭대학교 성모병원 소아과에서 이식을 시행받은 환아를 대상으로 하였다. pp66항원을 이용한 항원혈증검사를 토대로 혈연간 이식 환아의 경우 CMV 항원 양성세포가 5개 이상 발견된 경우, 비혈연간 이식 환아의 경우는 CMV 항원 양성세포가 하나라도 발견된 경우 ganciclovir 선제치료를 시작하였다. 결 과 : CMV 항원혈증은 대상 환아 213명 중 88명(41.3%)에서 관찰되었고, 각각 비혈연간 골수이식(62.5%), 비혈연간 제대혈이식(36.8%), HLA-일치 혈연간 이식(25.3%)이었다. 이식유형에 따른 CMV 항원혈증 발생확률은 비혈연간 골수이식($62.5{\pm}5.4%$)이 비혈연간 제대혈이식($36.8{\pm}7.8%$) 또는 HLA-일치 혈연간 이식($25.3{\pm}4.5%$)보다 통계적으로 유의하게 높았다. 단변량 분석에 의하면 비혈연간 이식, 이식 시 환자 연령(5세 이상), 이식 전 환자의 CMV-IgG, 전처치로 전신방사선조사의 사용 및 2도 이상의 급성 이식편대 숙주병의 발생이 CMV 항원혈증 발생의 위험인자이었다. 다변량분석에 의하면 비혈연간 이식, 이식전 환자의 CMV-IgG 양성상태 및 2도 이상의 급성 이식편대 숙주병의 발생이 독립적인 위험인자이었다. 이식환자 213명 중 7례(3.3%)에서 CMV 질환이 발생하였다(고항원혈증에서 6례 발생). 결 론 : 소아 조혈모세포 이식에 있어서 CMV 감염의 위험인자는 이식 전 환자의 CMV 혈청학적 상태, 조혈모세포 공급원, 급성 이식편대 숙주병이었으며, CMV 항원혈증에 입각한 ganciclovir 선제치료는 CMV 질환의 발생을 예방하는데 효과적이었다.

• Journal of Chest Surgery
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• 제50권5호
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• pp.382-385
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• 2017

A 47-year-old man with myasthenia gravis (MG) was admitted for a lung transplant. He had bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia. MG developed after stem cell transplantation. Bilateral sequential lung transplantations and a total thymectomy were performed. The patient underwent right diaphragmatic plication simultaneously due to preoperatively diagnosed right diaphragmatic paralysis. A tracheostomy was performed and bilevel positive airway pressure (BiPAP) was applied on postoperative days 8 and 9, respectively. The patient was transferred to the general ward on postoperative day 12, successfully weaned off BiPAP on postoperative day 18, and finally discharged on postoperative day 62.