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myelodysplasia syndrome transfer

Ting Zhou, Stephanie N Perez, Ziming Cheng, Marsha C Kinney, Madeleine E Lemieux, Linda M Scott, Vivienne I Rebel
Perturbations in CREB binding protein (CREBBP) are associated with hematopoietic malignancies, including myelodysplastic syndrome (MDS). Mice hemizygous for Crebbp develop myelodysplasia with proliferative features, reminiscent of human MDS/myeloproliferative neoplasm-unclassifiable (MDS/MPN-U), and a proportion goes on to develop acute myeloid leukemia (AML). We have also shown that the Crebbp+/- non-hematopoietic bone marrow microenvironment induces excessive myeloproliferation of wild-type cells. We now report that transplantation of unfractionated Crebbp+/- bone marrow into wild-type recipients resulted in either early-onset AML or late-onset MDS and MDS/MPN-U...
2016: PloS One
Xianghong Chen, Erika A Eksioglu, Junmin Zhou, Ling Zhang, Julie Djeu, Nicole Fortenbery, Pearlie Epling-Burnette, Sandra Van Bijnen, Harry Dolstra, John Cannon, Je-in Youn, Sarah S Donatelli, Dahui Qin, Theo De Witte, Jianguo Tao, Huaquan Wang, Pingyan Cheng, Dmitry I Gabrilovich, Alan List, Sheng Wei
Myelodysplastic syndromes (MDS) are age-dependent stem cell malignancies that share biological features of activated adaptive immune response and ineffective hematopoiesis. Here we report that myeloid-derived suppressor cells (MDSC), which are classically linked to immunosuppression, inflammation, and cancer, were markedly expanded in the bone marrow of MDS patients and played a pathogenetic role in the development of ineffective hematopoiesis. These clonally distinct MDSC overproduce hematopoietic suppressive cytokines and function as potent apoptotic effectors targeting autologous hematopoietic progenitors...
November 2013: Journal of Clinical Investigation
Adrian D Murphy, Peter A Williamson, Martin Vesely
We present the case of a 68-year old gentleman with previously diagnosed myelodysplastic syndrome with pancytopenia who presented with exophthalmia, rhinorrhea, and ophthalmoplegia. Nasal endoscopy revealed black necrotic lesions. He was diagnosed with rhino-orbital mucormycosis and commenced on intravenous antifungals. Despite this therapy he progressed to have total unilateral loss of visual acuity and cutaneous necrosis. He underwent emergency orbital exenteration and extensive cheek and sinus debridement...
March 2013: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
Junji Tokushige, Toshimitsu Ueki, Keijiro Sato, Yuko Fujikawa, Ikuo Shimizu, Daigo Akahane, Masahiko Sumi, Mayumi Ueno, Naoaki Ichikawa, Hikaru Kobayashi
A 56-year-old man was diagnosed with acute myeloid leukemia with myelodysplasia-related changes. Chromosomal analysis showed a complex karyotype. Complete remission could not be achieved even after several induction chemotherapy regimens, and the patient suffered from invasive pulmonary aspergillosis. He was transferred to our hospital and underwent reduced-intensity conditioning cord blood transplantation (RIC-CBT) in a non-remission state. The conditioning regimen involved fludarabine 125 mg/m2 combined with melphalan 140 mg/m2 and total body irradiation (4 Gy)...
May 2012: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Steven M Fruchtman, Robert M Petitt, Harriet S Gilbert, Garrick Fiddler, Andrew Lyne
Appropriate treatment for nonreactive thrombocytosis resulting from a myeloproliferative disorder (MPD) is surrounded by controversy. Although few doubt the association of thrombocytosis with increased risk for life-threatening events such as thrombosis or hemorrhage, or the association between clonal myeloproliferation and the progression to acute leukemia or myelofibrosis, controversy exists regarding the timing and nature of appropriate therapeutic intervention. Studies have shown that treatment with myelosuppressive agents such as chlorambucil, busulfan, radiophosphorus (32P), and hydroxyurea reduces the platelet count...
May 2005: Leukemia Research
Satkiran S Grewal, Jeffrey P Kahn, Margaret L MacMillan, Norma K C Ramsay, John E Wagner
The only proven cure for Fanconi anemia (FA)-associated bone marrow failure is successful allogeneic hematopoietic stem cell transplantation (HSCT). However, HSCT with donors other than HLA-identical siblings is associated with high morbidity and poor survival. Therefore, we used preimplantation genetic diagnosis (PGD) to select an embryo produced by in vitro fertilization (IVF) that was unaffected by FA and was HLA-identical to the proband. The patient was a 6-year-old girl with FA and myelodysplasia previously treated with oxymetholone and prednisone...
February 1, 2004: Blood
M Mita, T Ishibashi, T Shichishima, Y Maruyama
An unusual case of Werner's syndrome with myelodysplastic syndrome (MDS) in a 63-year-old male is reported. He was transferred to our hospital for evaluation of pancytopenia in August 1994. He had surgery for cataracts at age 47. On admission, his weight was 39 kg and his height was 148 cm. He looked pale, had thin limbs, a bird-like face, loss of hair, a hoarse voice, and atrophic skin with ulcers. A complete blood count showed: hemoglobin 8.7 g/dl; platelet count 1.5 x 10(4)/microliters; and white blood cell count 2,900/microliters with 3% blasts...
August 1996: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
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