• BLOOD RESEARCH
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• 제53권4호
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• pp.288-293
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• 2018

Background Although allogeneic hematopoietic cell transplantation (HCT) is the only curative treatment option for myelodysplastic syndrome (MDS), a substantial number of patients experience relapse. We reviewed the clinical outcomes of patients with MDS who relapsed after allogeneic HCT. Methods Thirty patients who experienced relapse or progression after allogeneic HCT for MDS between July 2000 and May 2016 were included in this retrospective analysis. Results The median time from HCT to relapse was 6.6 (range, 0.9-136.3) months. Donor lymphocyte infusions (DLIs) were administered to four patients: one achieved complete remission (CR) and survived disease free, while three did not respond to DLI and died. Hypomethylating agents were administered to seven patients: one who had stable disease continuously received decitabine, while six died without response to treatment. Six patients received AML-like intensive chemotherapy, and three achieved CR: two underwent second HCT and one DLI. One patient receiving second HCT survived without disease, but the other two relapsed and died. Three, four, and eight patients who did not respond to intensive chemotherapy, low-dose cytarabine, and best supportive care, respectively, died. One patient who underwent second HCT following cytogenetic relapse survived disease free. Median overall survival after relapse was 4.4 months, and relapse within 6 months after HCT was associated with shorter survival. Conclusion Outcomes of MDS patients relapsing after allogeneic HCT were disappointing. Some patients could be saved using DLI or second HCT.

• 대한영상의학회지
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• 제85권2호
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• pp.474-479
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• 2024

Mucormycosis는 Mucorales에 의해 유발되는 진균 감염으로, 다양한 장기를 침범할 수 있으며 그중에서도 폐점균증은 드물지만 특히 면역 저하 환자에서 생명을 위협하는 기회 감염이다. 폐점균증의 영상 소견은 다양하지만, 폐점액진균증 환자에서 점점 커지는 폐동맥색전증을 모방하는 경우는 드물게 보고되었다. 저자들은 골수이형성 증후군 환자에서 종괴와 폐색전증을 모방한 혈관 침습성 점막 진균증의 증례에 대해 보고하고자 한다.

• BLOOD RESEARCH
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• 제53권4호
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• pp.268-268
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• 2018

• 대한수의학회지
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• 제48권2호
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• pp.203-207
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• 2008

A 3-year-old spayed female Persian feline with non regenerative anemia showed persistent autoagglutination in EDTA anticoagulated blood. Primary immune-mediated hemolytic anemia (IMHA) was suspected and the underlying causes for IMHA were excluded by radiologic, sonographic, serologic and molecular studies. Cytologic examination of the bone marrow revealed that dysmyelopoiesis and dysplastic changes were prominent in the erythroid cells. These changes included asynchronous maturation of the nucleus and cytoplasm, binucleation, trinucleation, fragmented or lobulated nuclei and multilineages. Mild dysgranulopoiesis and dysmegakaryocytopoiesis were also detected including pseudo Pelger-Huet anomalies, giant band neutrophils, asynchronous maturation of the nucleus and cytoplasm in granulopoiesis and large hypolobulated forms as well as dwarf megakaryocytes in megakaryocytopoiesis. Myelodysplastic syndrome and congenital dysmyelopoiesis was ruled out by the low number of blast cells. Finally, secondary dysmyelopoiesis associated with IMHA was diagnosed and immunosuppressive treatment was successfully responsive.

• 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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• 제49권1호
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• pp.107-111
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• 2006

크론병은 매우 드문 질환이나 지난 10년간 발생률이 꾸준히 증가하고 있다. 저자들은 골수 이형성 증후군을 가진 환아에서 크론병이 발병한 2례를 소아에서는 최초로 보고하는 바이다. 첫번째 환아는 3세에 골수 이형성 증후군으로 진단받았고, 수 년간 지속된 반복적인 복통 및 설사, 혈변, 성장 부전이 있어 8세에 크론병으로 진단받았다. 대장 내시경 검사에서는 맹장에서 오름 결장에 걸쳐 조약돌상 점막과 표재성 궤양 및 염증성 삼출이 있었으며, 조직 소견은 궤양 사이에 정상 점막을 포함하고 있으면서 림프구 침윤을 보였다. Mesalazine과 deflazacort로 치료 후 증상은 호전을 보였다. 두 번째 환아는 9세에 골수 이형성증후군으로 진단받았으며, 13세에 반복되는 혈변과 복통, 구토, 발열로 크론병으로 진단받았다. 대장 내시경 검사에서 크고 깊은 경화성 궤양이 회맹판에서 맹장 쪽 주위에서 발견되었다. 이 외의 부위에는 병변이 없어 병변을 절제하였고, 조직은 경벽 염증과 림프구 집합을 동반한 궤양 소견을 보였다. 절제술 후 증상은 호전을 보였다.

• 대한한방내과학회지
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• 제37권3호
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• pp.539-547
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• 2016

본 증례에서는 한방치료를 받은 두 명의 골수이형성증후군 환자를 통하여 골수이형성증후군에 대한 한방치료의 효과를 보여주고자 하였다. 골수이형성증후군은 골수의 증식과 구성 세포들의 이형성, 비효율적인 조혈을 특징으로 하는 골수의 악성 종양이다. 현재까지 골수이형성증후군에 대한 완벽한 치료법은 없으며 골수이형성증후군의 증상을 경감시키거나 급성 골수성 백혈병으로의 진행을 막는 목적으로 치료가 이뤄지고 있다. 첫 번째 환자는 1차성 골수이형성증후군 환자로 2011년 1월부터 2016년 현재까지 꾸준히 침 치료 및 한약치료를 받고 있다. 두 번째 환자는 난소암 치료를 위한 항암화학요법의 부작용으로 생긴 2차성 골수이형성증후군 환자로 2010년 10월부터 4년간 꾸준한 한약 복용 및 3번의 입원치료를 받은 환자이다. 침 치료, 한약 치료와 함께 환자들의 상태를 평가하기 위해 정기적인 혈액검사가 이루어졌다. 골수이형성증후군 진단 후 두 달에 한 번씩 수혈을 받으며 수혈 의존 경향을 보이던 첫 번째 환자는 한방치료 후 단 두 번의 수혈밖에 받지 않았고 급성골수성백혈병으로 진행하지도 않았으며 건강한 몸 상태를 유지하고 있다. 두 번째 환자 역시 꾸준한 한방치료를 통해 비교적 좋은 삶의 질을 유지하며 기본 체력 또한 많이 상승되면서 골수 이식을 받을 정도까지 몸 상태가 개선되었다. 본 증례보고는 마땅한 치료법이 없는 골수이형성증후군 환자들에게 한방 치료가 특별한 부작용 없이 환자들이 호소하는 증상을 개선하고 기본적인 체력 증진에 도움이 되며 급성 골수성 백혈병으로의 진행을 예방하는 동시에 기존의 보존적 치료법의 효과를 극대화 시키는 효과가 있음을 보여주는 증례라고 하겠다.

• Asian Pacific Journal of Cancer Prevention
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• 제17권3호
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• pp.1519-1529
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• 2016

Background: Matrix metalloproteinase -2 (gelatinase-A, Mr 72,000 type IV collagenase, MMP-2) and -9 (gelatinase-B, Mr 92,000 type IV collagenase, MMP-9) are key molecules that play roles in tumor growth, invasion, tissue remodeling, metastasis and stem-cell regulation by digesting extracellular matrix barriers. MMP-2 and -9 are well known to impact on solid cancer susceptibility, whereas, in hematological malignancies, a paucity of data is available to resolve the function of these regulatory molecules in bone marrow mononuclear cells (BM-MNCs) and stromal cells of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Objectives: The present study aimed to investigate mRNA expression and gelatinase A and B secretion from BM-MNCs in vitro and genotypic associations of MMP-2 (-1306 C/T; rs243865), MMP-9 (-1562 C/T; rs3918242), tissue inhibitor of metalloproteinase -1 (TIMP-1) (372T/C; rs4898, Exon 5) and TIMP-2 (-418G/C; rs8179090) in MDS and AML. Results: The study covered cases of confirmed MDS (n=50), AML (n=32) and healthy controls (n=110). MMP-9 mRNA expression revealed 2 fold increased expression in MDS-RAEB II and 2.5 fold in AML M-4 (60-70% blasts). Secretion of gelatinase-B also revealed the MMP-9 mRNA expression and ELISA data also supported these data. We noted that those patients having more blast crises presented with more secretion of MMP-9 and its mRNA expression. In contrast MMP-9 (-1562 C/T) showed significant polymorphic associations in MDS (p<0.02) and AML (p<0.02). MMP-9 mRNA expression of C/T and T/T genotypes were 1.5 and 2.5 fold increased in MDS and AML respectively. In AML, MMP-2 C/T and T/T genotypes showed 2.0 fold mRNA expression. Only MMP-9 (-1306 C/T) showed significant 4 fold (p<0.001) increased risk with chemical and x-ray exposed MDS, while tobacco and cigarette smokers have 3 fold (p<0.04) risk in AML. Conclusions: In view of our results, MMP-9 revealed synergistic secretion and expression in blast crises of MDS and AML with 'gene' polymorphic effects and is significantly associated with increased risk with tobacco, cigarette and environmental exposure. Release and secretion of these enzymes may influence hematopoietic cell behavior and may be important in the clinical point of view. It may offer valuable tools for diagnosis and prognosis, as well as possible targets for the treatments.

• Asian Pacific Journal of Cancer Prevention
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• 제17권4호
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• pp.2341-2342
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• 2016

• Molecules and Cells
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• 제43권2호
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• pp.139-144
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• 2020

Somatic RUNX1 mutations are found in approximately 10% of patients with de novo acute myeloid leukemia (AML), but are more common in secondary forms of myelodysplastic syndrome (MDS) or AML. Particularly, this applies to MDS/AML developing from certain types of leukemia-prone inherited bone marrow failure syndromes. How these RUNX1 mutations contribute to the pathobiology of secondary MDS/AML is still unknown. This mini-review focusses on the role of RUNX1 mutations as the most common secondary leukemogenic hit in MDS/AML evolving from severe congenital neutropenia (SCN).