• Title/Summary/Keyword: 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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    • v.17 no.3
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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
    • Korean Journal of Veterinary Research
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    • v.48 no.2
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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.

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

  • Kim, Jun-young;Joung, Jin-yong;Son, Chang-gue;Cho, Jung-hyo
    • The Journal of Internal Korean Medicine
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    • v.37 no.3
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    • pp.539-547
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    • 2016
  • Myelodysplastic syndrome (MDS) is a typical myeloid malignancy characterized by cell dysplasia in bone marrow. Currently, there is no therapeutic treatment for MDS. The only available therapies either relieve symptoms or prevent the development of acute myeloid leukemia (AML). This study aimed to report the effects of traditional Korean medicine (TKM) on MDS by presenting two case reports. The patient in Case 1 was diagnosed with primary MDS and regularly received acupuncture treatments and herbal medicine. In Case 2, a patient with ovarian cancer was diagnosed with secondary MDS, which resulted from an adverse reaction to chemotherapy. This patient took herbal medicine for four years and was hospitalized three times. In order to have their condition evaluated, both patients underwent regular blood tests. The patient in Case 1, who showed blood transfusion dependency, received only two blood transfusions after TKM treatment, and the person’s health condition was stable as of January 2016 without any signs of AML development. The patient in Case 2 also has stable health condition. TKM treatment effectively treated their MDS symptoms and improved their general health conditions without any adverse effects. It also prevented the rapid development of AML and maximized the effects of conservative therapy.

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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    • v.33 no.2
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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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    • v.31 no.3
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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.