• Journal of Chest Surgery
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• 제52권5호
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• pp.376-379
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• 2019

Concomitant Loeys-Dietz syndrome (LDS) and hematologic malignancies are exceptionally rare. This is the first report of a patient operated on for aortic root dilation who had been previously diagnosed with LDS and B-cell-lymphoma. After completion of chemotherapy and complete remission, an elective valve-sparing aortic root replacement (using the David-V method) was performed. Due to the positive family history, preoperative genetic counseling was conducted, and revealed LDS with a TGFBR1 (transforming growth factor beta receptor type I) mutation in 6 probands of the family, albeit in 1 of them posthumously. This missense mutation has been previously described in relation to aortic dissection, but a causative relationship to malignancy has so far neither been proposed nor proven.

• 임상간호연구
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• 제22권1호
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• pp.1-9
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• 2016

Purpose: This study was done to identify effects of oral care protocol on oral mucositis and oral care performance in hematologic malignancy patients receiving chemotherapy. Methods: The design of this study was a nonequivalent control group pretest-posttest design. Both groups were patients diagnosed with hematologic malignancies who were receiving chemotherapy-each group had 20 patients. In the experimental group, patients were given intensive education on oral care based on the oral care protocol, whereas in the controlled group, each patient was given an educational brochure. Before chemotherapy, and 3 days, 7 days, and 14 days after chemotherapy, oral mucositis status of two groups were assessed using the guide to physical assessment of the oral cavity. Oral care performance was examined before chemotherapy and 14 days later. Results: The experimental group with the oral care protocol showed a significant difference (F=18.15, p<.001) in the oral mucositis status, and also in oral care performance (t=-10.33, p<.001). Conclusion: Findings indicate that the application of the oral care protocol is an effective tool for lowering the occurrence of oral mucositis and enhancing oral care performance in hematologic malignancy patients receiving chemotherapy.

• Molecules and Cells
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• 제41권8호
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• pp.717-723
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• 2018

Chimeric antigen receptor (CAR) T-cell therapy, an emerging immunotherapy, has demonstrated promising clinical results in hematological malignancies including B-cell malignancies. However, accessibility to this transformative medicine is highly limited due to the complex process of manufacturing, limited options for target antigens, and insufficient anti-tumor responses against solid tumors. Advances in gene-editing technologies, such as the development of Zinc Finger Nucleases (ZFNs), Transcription Activator-Like Effector Nucleases (TALENs), and Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR/Cas9), have provided novel engineering strategies to address these limitations. Development of next-generation CAR-T cells using gene-editing technologies would enhance the therapeutic potential of CAR-T cell treatment for both hematologic and solid tumors. Here we summarize the unmet medical needs of current CAR-T cell therapies and gene-editing strategies to resolve these challenges as well as safety concerns of gene-edited CAR-T therapies.

• Advances in pediatric surgery
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• 제15권2호
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• pp.103-112
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• 2009

Catheter related and perianal problems are common surgical complications encountered during the treatment of pediatric malignancies. However acute surgical abdominal emergencies are rare. The aim of this study is to review acute surgical abdominal complications that occur during the treatment of childhood malignancies. Out of a total of 1,222 patients who were newly diagnosed with malignant disease, between January 2003 and May 2008, there were 10 patients who required surgery because of acute abdominal emergencies. Their medical records were reviewed retrospectively. Hematologic malignancies were present in 7 patients (4 leukemia, 2 lymphoma, 1 Langerhans cell histiocytosis) and solid tumors in 3 patients (1 adrenocortical carcinoma, 1 desmoplastic small round cell tumor, 1 rhabdomyosarcoma). Seven patients had intestinal obstruction, two had gastrointestinal perforation and one, typhlitis. Intestinal obstructions were treated with resection of the involved segment with (N=2) or without (N=3) enterostomy. Two patients had enterostomy alone when resection could not be performed. Intestinal perforation was treated with primary repair. Typhlitis of the ascending colon was treated with ileostomy. Right hemicolectomy was necessary the next day because of the rapidly progressing sepsis. Three patients are now alive on chemotherapy and one patient was lost to followed-up. Among six patients who died, five died of their original disease progression and one of uncontrolled sepsis after intestinal perforation. Although rare, acute surgical abdominal complications can occur in childhood malignancies. Rapid and accurate diagnosis and appropriate operation are required for effective treatment of the complications.

• 한국임상약학회:학술대회논문집
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• 한국임상약학회 2007년도 추계학술대회
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• pp.199-200
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• 2007

• Annals of Clinical Neurophysiology
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• 제25권2호
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• pp.84-92
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• 2023

Background: Clinical spectrum of immunoglobulin M (IgM) monoclonal gammopathy varies from IgM monoclonal gammopathy of unknown significance (IgM-MGUS) to hematological malignancies. We evaluated the clinical features, electrophysiological characteristics, and prognosis of patients with peripheral neuropathy associated with IgM monoclonal gammopathy (PN-IgM MG). Methods: We retrospectively evaluated 25 patients with PN-IgM MG. Peripheral neuropathy was classified as axonal, demyelinating, or undetermined, based on electrophysiological studies. We classified the enrolled patients into the IgM-MGUS and malignancy groups, and compared the clinical and electrophysiological features between the groups. Results: Fifteen patients had IgM-MGUS and 10 had hematologic malignancies (Waldenström's macroglobulinemia: two and B-cell non-Hodgkin's lymphoma: eight). In the electrophysiological evaluation, the nerve conduction study (NCS) criteria for demyelination were met in 86.7% of the IgM-MGUS group and 10.0% of the malignancy group. In particular, the distal latencies of the motor NCS in the IgM-MGUS group were significantly prolonged compared to those in the malignancy group (median, 9.1 ± 5.1 [IgM-MGUS], 4.2 ± 1.3 [malignancy], p = 0.003; ulnar, 5.4 ± 1.9 [IgM-MGUS], 2.9 ± 0.9 [malignancy], p = 0.001; fibular, 9.3 ± 5.1 [IgM-MGUS], 3.8 ± 0.3 [malignancy], p = 0.01; P-posterior tibial, 8.3 ± 5.4 [IgM-MGUS], 4.4 ± 1.0 [malignancy], p = 0.04). Overall treatment responses were significantly worse in the malignancy group than in the IgM-MGUS group (p = 0.004), and the modified Rankin Scale score at the last visit was higher in the malignancy group than in the IgM-MGUS group (2.0 ± 1.1 [IgM-MGUS], 4.2 ± 1.7 [malignancy], p = 0.001), although there was no significant difference at the initial assessment. Conclusions: The risk of hematological malignancy should be carefully assessed in patients with PN-IgM MG without electrophysiological demyelination features.

• Journal of Hospice and Palliative Care
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• 제25권1호
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• pp.12-24
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• 2022

Purpose: This descriptive study compared the perceptions, determinants, and needs of patients, family members, nurses, and physicians regarding life-sustaining treatment decisions for patients with hematologic malignancies in the hematology-oncology department of a tertiary hospital in Seoul, Korea. Methods: In total, 147 subjects were recruited, gave written consent, and provided data by completing a structured questionnaire. Data were analyzed using analysis of variance, the chi-square test, and the Fisher exact test. Results: Nurses (F=3.35) and physicians (F=3.57) showed significantly greater familiarity with the Act on Decisions on Life-Sustaining Treatment than patients (F=2.69) and family members (F=2.59); (F=19.58, P<0.001). Many respondents, including 19 (51.4%) family members, 16 (43.2%) physicians, and 11 (29.7%) nurses, agreed that the patient's opinion had the greatest effect when making life-sustaining treatment decisions. Twelve (33.3%) patients answered that mental, physical, and financial burdens were the most important factors in life-sustaining treatment decisions, and there was a significant difference among the four groups (P<0.001). Twenty-four patients (66.7%), 27 (73.0%) family members, and 21(56.8%) nurses answered that physicians were the most appropriate people to provide information regarding life-sustaining treatment decisions. Unexpectedly, 19 (51.4%) physicians answered that hospice nurse practitioners were the most appropriate people to talk to about life-sustaining treatment (P<0.001). Conclusion: It is of utmost importance that the patient and physician determine when life-sustaining treatment should be withdrawn, with the patient making the ultimate decision. Doctors and nurses have the responsibility to provide detailed information. The goal of end-of-life planning is to ensure patients' dignity and respect their values.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.566-571
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• 2003

목 적 : 백질뇌병증은 중추신경계 예방 요법을 시행하는 혈액종양 환자들에게는 심각한 합병증의 하나로, 이러한 백질뇌병증을 최소화하기 위해서는 조기 진단과 그에 따른 치료 방법의 변경이 중요하다 하겠다. 이 연구는 소아 혈액종양 환자에서 예방적 중추신경계 치료에 따른 백질뇌병증의 임상 양상 및 치료 방법 변경 후의 예후에 관해 조사하고, 백질뇌병증의 발생을 최소화하기 위한 정기적 두부 자기공명영상 촬영의 의의를 살펴보고자 시행되었다. 방 법 : 1995년 10월부터 2002년 5월까지 경북대학교병원 소아과에서 급성 림프구성 백혈병 및 B세포 림프종으로 진단받고 중추신경계 예방 치료 후 백질뇌병증이 발견된 16명의 환자를 대상으로 병력지와 MRI를 후향적으로 조사하였다. 결 과 : 급성 림프구성 백혈병 환자 15명과 B세포 림프종 환자 1명의 백질뇌병증 진단 시 연령은 중앙값 5년 3개월이었고, 남녀비는 3 : 1이었다. 항암치료 시작 후 백질뇌병증 진단까지 걸린 시간은 2개월에서 17개월이었다. 백질뇌병증 발생 전 투여한 척수강 내 MTX 투여 횟수는 2회에서 15회로 16명에서 모두 시행하였고, 두부 방사선 치료는 10명에서만 시행하였고 용량은 1,800 rads였다. 백질뇌병증 진단 전 경련, 성격 변화, 두통, 정신 이상, 구음 장애, 의식 변화 등의 정신신경학적 증상이 있었던 경우가 10명 있었으며 증상이 없었던 경우가 6명이었다. 백질뇌병증이 진단되었던 16명의 환자 중 4명은 사망하였으며 나머지 12명의 환아 중에서 현재 계속 항암치료 중인 경우가 8명, 항암치료를 종료한 환자가 3명, 그리고 보호자가 원하여 항암치료를 중지한 경우가 1명이었다. 백질뇌병증 진단 후 자기공명영상의 추적관찰을 시행한 경우가 12명, 시기상 아직 시행하지 못한 경우가 4명이었으며 추적관찰을 시행한 경우 중 자기공명영상에서 병변이 호전된 경우가 4명, 지속되는 경우가 6명, 악화된 경우가 2명이었다. 병변이 호전된 4명 중 2명은 병변이 거의 사라져 정상에 가까운 소견을 보였다. 결 론 : 중추신경계 예방 요법으로 사용하는 척수강 내 MTX 주입 요법과 두부 방사선 조사를 비롯하여 정맥으로 투여되는 MTX 또한 백질뇌병증을 유발하며, 이러한 백질뇌병증은 증상없이 발현되는 경우도 많아 병변의 조기발견을 위한 정기적인 자기공명영상의 촬영이 필요하며, 백질뇌병증의 발견 즉시 즉각적인 조치를 취하여 신경학적 기능의 회복을 가능하게 해야 할 것이라고 생각된다.

• IMMUNE NETWORK
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• 제13권5호
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• pp.222-226
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• 2013

Translocations involving chromosome 21q22 are frequently observed in hematologic malignancies including acute myeloid leukemia (AML), most of which have been known to be involved in malignant transformation through transcriptional dysregulation of Runt-related transcription factor 1 (RUNX1) target genes. Nineteen RUNX1 translocational partner genes, at least, have been identified, but not Homeobox A (HOXA) genes so far. We report a novel translocation of RUNX1 into the HOXA gene cluster in a 57-year-old female AML patient who had been diagnosed with myelofibrosis 39 months ahead. G-banding showed 46,XX,t(7;21)(p15;q22). The involvement of RUNX1 and HOXA genes was confirmed by fluorescence in situ hybridization.

• Tuberculosis and Respiratory Diseases
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• 제72권3호
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• pp.318-322
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• 2012

Sarcoidosis is an inflammatory disease involving multiple-organs with an unknown cause. The new onset of sarcoidosis associated with therapeutic agents has been observed in 3 clinical settings; tumor necrosis factor antagonists in autoimmune rheumatologic diseases, interferon alpha with or without ribavirin in patients with chronic hepatitis C or melanoma, and antineoplastic agent-associated sarcoidosis in patients with hematologic malignancies. Here, we report a female patient who developed sarcoidosis after capecitabine treatment as an adjuvant chemotherapy for sigmoid colon cancer. To our knowledge, this is the first report of a capecitabine-induced sarcoidosis.