• 제목/요약/키워드: Primary -myelodysplastic syndrome

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Adult Primary Myelodysplastic Syndrome: Experience from a Tertiary Care Center in Pakistan

  • Sultan, Sadia;Irfan, Syed Mohammed
    • Asian Pacific Journal of Cancer Prevention
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    • 제17권3호
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    • pp.1535-1537
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    • 2016
  • Background: Primary myelodysplastic syndrome (MDS) is an acquired clonal disorder of myeloid progenitor cells, characterized by peripheral cytopenias in the presence of hypercellular marrow with dysplastic features. Our aim was to study the demographical and clinicopathological features of adult Pakistani patients with MDS at disease presentation. Materials and Methods: This single centre study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Data were retrieved from the patient archives. Results: Overall 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean age of $57.6{\pm}17.4years$ and median of 64 years. The male to female ratio was 1.7:1. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Examination was unremarkable in 42.2% of patients. Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20%. The commonest laboratory finding was anemia (hemoglobin < 10 g/dl in 41 (91.1%) patients. Out of these, 27 (60%) patients had normocytic anemia, followed by macrocytic (22.2%) and microcytic (8.8%). Conclusions: Primary MDS in Pakistani patients demonstrates a male preponderance. The proportion of anemic patients was high in our series with predominance of normocytic anemia. However, other clinico-hematological features appear comparable to published data.

Dysmyelopoiesis in a cat with immune-mediated hemolytic anemia

  • Yu, Do-Hyeon;Lee, Jong-Hyun;Yoon, Ji-Seon;Li, Ying-Hua;Lee, Mi-Jin;Park, Jinho
    • 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.

한방 치료를 통한 골수이형성증후군 치험례 2례 (Well-Managed Myelodysplastic Syndrome Patients Treated with Traditional Korean Medicine; Report of Two Cases)

  • 김준영;정진용;손창규;조정효
    • 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.

Hidden Hematologic Disease in Trauma Patients: A Report of Two Cases

  • Jang, Sung Woo;Jung, Pil Young
    • Journal of Trauma and Injury
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    • 제33권2호
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    • pp.112-118
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    • 2020
  • In trauma patients, coagulopathy and abnormal increases or decreases in cell counts are frequently observed, and are associated with high mortality and morbidity in the acute phase of trauma. Because major trauma is often life-threatening, and hematologic abnormalities are multi-factorial and transient, major blood loss is usually suspected to be the primary cause of these abnormalities, and much time and cost may be spent attempting to identify a focus of hemorrhage that might or might not actually exist. Persistent abnormalities in the complete blood count, however, require clinical suspicion of other hematologic diseases to minimize improper transfusions and to improve outcomes, including mortality. Physicians at trauma centers should be familiar with the clinical characteristics of hematologic diseases and should consider these diseases in trauma patients. In this report, we present cases of two hematologic disorders found in trauma patients: autoimmune hemolytic anemia induced by systemic lupus erythematosus and myelodysplastic syndrome.

Inhibition of DNMT3B and PI3K/AKT/mTOR and ERK Pathways as a Novel Mechanism of Volasertib on Hypomethylating Agent-Resistant Cells

  • Eun-Ji Choi;Bon-Kwan Koo;Eun-Hye Hur;Ju Hyun Moon;Ji Yun Kim;Han-Seung Park;Yunsuk Choi;Kyoo-Hyung Lee;Jung-Hee Lee;Eun Kyung Choi;Je-Hwan Lee
    • Biomolecules & Therapeutics
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    • 제31권3호
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    • pp.319-329
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    • 2023
  • Resistance to hypomethylating agents (HMAs) in myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) is a concerning problem. Polo-like kinase 1 (PLK1) is a key cell cycle modulator and is known to be associated with an activation of the PI3K pathway, which is related to the stabilization of DNA methyltransferase 1 (DNMT1), a target of HMAs. We investigated the effects of volasertib on HMA-resistant cell lines (MOLM/AZA-1 and MOLM/DEC-5) derived from MOLM-13, and bone marrow (BM) samples obtained from patients with MDS (BM blasts >5%) or AML evolved from MDS (MDS/AML). Volasertib effectively inhibited the proliferation of HMA-resistant cells with suppression of DNMTs and PI3K/AKT/mTOR and ERK pathways. Volasertib also showed significant inhibitory effects against primary BM cells from patients with MDS or MDS/AML, and the effects of volasertib inversely correlated with DNMT3B expression. The DNMT3B-overexpressed AML cells showed primary resistance to volasertib treatment. Our data suggest that volasertib has a potential role in overcoming HMA resistance in patients with MDS and MDS/AML by suppressing the expression of DNMT3 enzymes and PI3K/AKT/mTOR and ERK pathways. We also found that DNMT3B overexpression might be associated with resistance to volasertib.